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Written 2 hours ago

Bethany & I are home from the hospital now. Her labs have the hospital pathologist concerned about a bone marrow disorder ~ one that carries an average survival rate of less than 1 year for healthy children who undergo intensive treatment, which Bethany would not survive due to her other medical conditions. Without a bone marrow biopsy & lumbar puncture, we couldn't be sure of a diagnosis of the disorder in question (which is why I'm not even bothering to name it), but we are not pursuing a concrete diagnosis because it doesn't matter if she has it or not. When the pathologist told the pediatrician that she should contact heme-onc about Bethany, the ped told him, "She's pretty much end stage already." It shocked me to hear those words, but then again, it really didn't. We have known things were not going in the right direction for awhile. I just didn't realize that we'd arrive at this point so quickly. So, we aren't going to worry about the bone marrow issue. As the ped told me this afternoon, there is enough going on to bring us to the point we are at and the diagnosis of something else would not change one aspect of the decisions we are making. She said that knowing Bethany has a problem with her bone marrow on top of everything else only confirms that we are making loving, good choices for our little girl as we decide to stop all extraordinary measures of sustaining her life.

Please pray for safe travel for Bethany, Emily & me as we drive to Portland tomorrow morning. I will meet with the palliative care doctor & rheumatologist and then, once we are settled for the night, I will detail what is meant by "extraordinary measures" and what we anticipate will take place over the next few weeks.

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Written Jan 18, 2013 9:50pm

For the third time in five weeks, Bethany is sick enough to require hospitalization for treatment of congestive heart failure & flaring of her arthritis. Her hemoglobin is now 7.0 (the low end of normal is 11.4), she has no functioning immune system, her liver enzymes have continued to climb, her inflammation markers are WAY high and the Kineret is not working well enough to control her disease. Her

rheumatologist said we will need to add weekly IV steroids to the mix PLUS we've jacked up her oral steroids to 5 times what she was getting, so she's back on *very* high-dose oral steroids with the expectation being that we will hopefully be able to wean her down to a moderate dose, where she will stay (no more anticipation of getting her off of them). We will still be talking on Monday about adding cyclosporine, which is a major immune suppressing medication typically given to transplant patients to prevent rejection (ie: it would keep her with no functioning immune system, making her susceptible to every opportunistic infection that comes her way). She needs a blood transfusion, IV steroids & diuretics to pull off the fluid she is retaining. She'll now get 3 doses per day of one diuretic (Lasix) & 2 doses per day of a second one (Spironolactone).

Dr. S and I talked briefly today about whether it is time to start doing less. This is a topic that will be broached when I see Dr. K on Monday, too. I am hoping to be able to sit down with Dr. T (palliative care) during this trip north, too, since he is well-versed with situations involving ethics and both Dr. S & I believe his guidance is needed. Bethany's situation brings to mind the ethical dilemma of "Just because you CAN do something to a child, SHOULD you?" For a child with extremely poor quality of life who has multiple incurable conditions, two that her doctors are struggling with and that are not well-controlled, how far do we (her medical team & parents) go in trying to manage her diseases? Do we load her up with twice-daily oral steroids, weekly IV steroids, a shot of chemo once a week, daily immune-suppressing shots, another big-gun immunosuppressing drug, diuretics to pull off the fluid that keeps accumulating around her heart and lungs, & a couple other medications to treat the side effects of her primary meds in order to enable her to sit, mostly unmoving, for a couple of weeks before she needs to be hospitalized for a day or two to stabilize her before we start the cycle once more? We CAN continue to do that, but SHOULD we? I am *NOT* actually asking anyone's opinion and honestly, I would prefer that no one tell me what they think they would do or what you think I should do because this is something that those who fully know every aspect of the situation (Bethany's doctors and Charley & myself) will be deciding. It isn't something that people on the internet should be weighing in on, no matter how well-meaning, so please, please respect my request to not offer advice on this particular matter if you choose to comment on this journal entry. I am sharing all of this only to help you put yourself in my shoes and understand part of what we (Bethany's medical team & Charley & myself) are wrestling with.

At this point, I'm not even beginning to open the door on how this all FEELS. I can't. When I have tried to crack that door even a smidgeon at

different times during the day, I have been hit with a wave of nausea and deep sadness and I realized that I can't deal with the emotion yet. It's too much. So if I sound clinical and devoid of emotion, it's because I am deliberately trying to keep emotion out of this for now. When I know which direction we are heading and what our game plan for Bethany is going to be, I will begin processing how I feel about all of it. For now, for tonight, I am attempting to not fall apart because there is a part of me that is still hoping God is going to open the door to something... some treatment, some medication, some THING... that is going to offer us real hope for Bethany & her future. After Monday, I will face whatever reality is set before me. That's still a few days away, though, so until then, I'll try to ignore how much my heart hurts and pretend that I actually believe we could get a different outcome than that which it seems we are being prepared for.


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I'm sad. Bethany had a high fever last night & a low-grade fever this morning. She's getting red blotches on her skin that come & go. She's not sick with a virus (she has zero other signs/symptoms of illness). This is her arthritis trying to flare. I contacted her rheumatologist & we emailed back & forth last night/this morning. He wants to see us in clinic on Monday so we can discuss adding Cyclosporine or perhaps something else to the Kineret & Methotrexate & Prednisolone she's already getting. The worst part? He said to stop weaning Bethany off the steroid (Prednisolone) and to actually bump her back UP to a dose that is more effective. He wrote, "Hope that helps ... at least a little. So sorry ... truly."

This is not going in the right direction, you guys. She's not getting better in any sense of the word. Her calf muscles are so atrophied from non-use (as a result of her arthritis) that her AFO's ~ the pair we picked up just a few months ago since the pair she had had gotten way too big for her legs ~ are now wayyyyy big on her again. I don't think her calves are more than a few inches around anymore. And the worse she feels, the more introverted she becomes. It's just so SAD. Remind me, please, of how God has a plan He is working out... one that is going to prosper and not harm Bethany... because honestly, I am just

not seeing it. I know it's true even if I can't see it.... even if I don't feel it... but honestly, having two kids with life-limiting conditions is really, really, REALLY hard... and the days when a doctor reminds me that nothing we're trying is working very well make it tough to maintain hope. It makes me so sad for Bethany. Existing is not the same as living.... I wish I knew what God was doing here.

Written 2 hours ago

Bethany & I are home from the hospital now. Her labs have the hospital pathologist concerned about a bone marrow disorder ~ one that carries an average survival rate of less than 1 year for healthy children who undergo intensive treatment, which Bethany would not survive due to her other medical conditions. Without a bone marrow biopsy & lumbar puncture, we couldn't be sure of a diagnosis of the disorder in question (which is why I'm not even bothering to name it), but we are not pursuing a concrete diagnosis because it doesn't matter if she has it or not. When the pathologist told the pediatrician that she should contact heme-onc about Bethany, the ped told him, "She's pretty much end stage already." It shocked me to hear those words, but then again, it really didn't. We have known things were not going in the right direction for awhile. I just didn't realize that we'd arrive at this point so quickly. So, we aren't going to worry about the bone marrow issue. As the ped told me this afternoon, there is enough going on to bring us to the point we are at and the diagnosis of something else would not change one aspect of the decisions we are making. She said that knowing Bethany has a problem with her bone marrow on top of everything else only confirms that we are making loving, good choices for our little girl as we decide to stop all extraordinary measures of sustaining her life.

Please pray for safe travel for Bethany, Emily & me as we drive to Portland tomorrow morning. I will meet with the palliative care doctor & rheumatologist and then, once we are settled for the night, I will detail what is meant by "extraordinary measures" and what we anticipate will take place over the next few weeks.


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For the third time in five weeks, Bethany is sick enough to require hospitalization for treatment of congestive heart failure & flaring of her arthritis. Her hemoglobin is now 7.0 (the low end of normal is 11.4), she has no functioning immune system, her liver enzymes have continued to climb, her inflammation markers are WAY high and the Kineret is not working well enough to control her disease. Her rheumatologist said we will need to add weekly IV steroids to the mix PLUS we've jacked up her oral steroids to 5 times what she was getting, so she's back on *very* high-dose oral steroids with the expectation being that we will hopefully be able to wean her down to a moderate dose, where she will stay (no more anticipation of getting her off of them). We will still be talking on Monday about adding cyclosporine, which is a major immune suppressing medication typically given to transplant patients to prevent rejection (ie: it would keep her with no functioning immune system, making her susceptible to every opportunistic infection that comes her way). She needs a blood transfusion, IV steroids & diuretics to pull off the fluid she is retaining. She'll now get 3 doses per day of one diuretic (Lasix) & 2 doses per day of a second one (Spironolactone).

Dr. S and I talked briefly today about whether it is time to start doing less. This is a topic that will be broached when I see Dr. K on Monday, too. I am hoping to be able to sit down with Dr. T (palliative care) during this trip north, too, since he is well-versed with situations involving ethics and both Dr. S & I believe his guidance is needed. Bethany's situation brings to mind the ethical dilemma of "Just because you CAN do something to a child, SHOULD you?" For a child with extremely poor quality of life who has multiple incurable conditions, two that her doctors are struggling with and that are not well-controlled, how far do we (her medical team & parents) go in trying to manage her diseases? Do we load her up with twice-daily oral steroids, weekly IV steroids, a shot of chemo once a week, daily immune-suppressing shots, another big-gun immunosuppressing drug, diuretics to pull off the fluid that keeps accumulating around her heart and lungs, & a couple other medications to treat the side effects of her primary meds in order to enable her to sit, mostly unmoving, for a couple of weeks before she needs to be hospitalized for a day or two to stabilize her before we start the cycle once more? We CAN continue to do that, but SHOULD we? I am *NOT* actually asking anyone's opinion and honestly, I would prefer that no one tell me what they think they would do or what you think I should do because this is something that those who fully know every aspect of the situation (Bethany's doctors and Charley & myself) will be deciding. It isn't something that people on the internet should be weighing in on, no matter how well-meaning, so please, please respect my request to not offer advice on this particular matter if you choose to comment on this journal entry. I am

sharing all of this only to help you put yourself in my shoes and understand part of what we (Bethany's medical team & Charley & myself) are wrestling with.

At this point, I'm not even beginning to open the door on how this all FEELS. I can't. When I have tried to crack that door even a smidgeon at different times during the day, I have been hit with a wave of nausea and deep sadness and I realized that I can't deal with the emotion yet. It's too much. So if I sound clinical and devoid of emotion, it's because I am deliberately trying to keep emotion out of this for now. When I know which direction we are heading and what our game plan for Bethany is going to be, I will begin processing how I feel about all of it. For now, for tonight, I am attempting to not fall apart because there is a part of me that is still hoping God is going to open the door to something... some treatment, some medication, some THING... that is going to offer us real hope for Bethany & her future. After Monday, I will face whatever reality is set before me. That's still a few days away, though, so until then, I'll try to ignore how much my heart hurts and pretend that I actually believe we could get a different outcome than that which it seems we are being prepared for.


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I'm sad. Bethany had a high fever last night & a low-grade fever this morning. She's getting red blotches on her skin that come & go. She's not sick with a virus (she has zero other signs/symptoms of illness). This is her arthritis trying to flare. I contacted her rheumatologist & we emailed back & forth last night/this morning. He wants to see us in clinic on Monday so we can discuss adding Cyclosporine or perhaps something else to the Kineret & Methotrexate & Prednisolone she's already getting. The worst part? He said to stop weaning Bethany off the steroid (Prednisolone) and to actually bump her back UP to a dose that is more effective. He wrote, "Hope that helps ... at least a little. So sorry ... truly."

This is not going in the right direction, you guys. She's not getting better in any sense of the word. Her calf muscles are so atrophied from non-use (as a result of her arthritis) that her AFO's ~ the pair we

picked up just a few months ago since the pair she had had gotten way too big for her legs ~ are now wayyyyy big on her again. I don't think her calves are more than a few inches around anymore. And the worse she feels, the more introverted she becomes. It's just so SAD. Remind me, please, of how God has a plan He is working out... one that is going to prosper and not harm Bethany... because honestly, I am just not seeing it. I know it's true even if I can't see it.... even if I don't feel it... but honestly, having two kids with life-limiting conditions is really, really, REALLY hard... and the days when a doctor reminds me that nothing we're trying is working very well make it tough to maintain hope. It makes me so sad for Bethany. Existing is not the same as living.... I wish I knew what God was doing here.

Written Dec 30, 2012 10:52pm

Bethany's white cell count is down to 2,800. Her other labs are about the same as yesterday, except for her ferritin, which has sky-rocketed, and her red cell morphology is substantially more abnormal. The rheumatologist & heme-onc & infectious disease doc don't know if Bethany has some raging infection (despite nothing growing out in her blood or urine cultures, but heme-onc said things can look a lot different in an immune-suppressed person) or if this is disease progression of her arthritis and her weaning off Actemra and having to wait a week to get Kineret after having it for 3 days in the hospital (in Portland) allowed her s-JIA to "explode" with a huge flare. Some labs support infection while others point to arthritis, but many of them flat-out contradict each other (such as her ferritin going up but her LDH going down ~ they usually go in the same direction) and that has left the doctors unsure what is going on. As such, the only thing that has been changed today is the addition of morphine to help Bethany be more comfortable. The doctors are in agreement that pursuing "comfort measures" is appropriate, so they're doing that. More labs will be drawn in the morning and the hope is that we'll see her number of white cells moving up (along with her other cell lines). If that happens, it will point to disease progression of her arthritis being the culprit of this hospitalization (well, in addition to her heart failure, but cardiology says that is "stable" for now... not that it means everything is okay, because it's not, but at least it's not getting worse right now while this other... whatever... is going on). If her white cells drop even further, then heme-onc will take over with treatment for a bit to get her cell lines more stabilized. I don't know what that would entail. Dr. Kingsbury is hoping she can pull out of this on her own. If she can't, he can do pulse steroids again to artificially inflate her cell lines, but he's really hoping we won't need to.

Bethany looks about the same ~ no better, no worse. Her feeds were

re-started this afternoon to evaluate whether she starts having GI bleeding again (she hasn't gotten anything in her gut since being admitted). Thus far, she's tolerating the feeds without problem and no bleeding has been observed, which is good news.

To those who have suggested Mastocytosis as a diagnosis (in the guestbook) and asked whether we could just start treating Bethany for that in case that is what's wrong with her, the answers are no, she does not have that condition and no, we can't treat her for a condition she does not have. Treating her for mastocytosis would be as inappropriate as treating her for prostate cancer ~ if she doesn't have the condition, there's no reason to subject her to treatment for it. Also, as medically-fragile as Bethany is, no one is going to start her on any medication without believing it is absolutely necessary & will hopefully benefit her enough to make any risk involved worth taking. Thank you for wanting to help, though. That is very kind of you. <smile>


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Written Dec 30, 2012 2:37am

Bethany's labs tonight are very bad. Her white cell count dropped from 41,200 yesterday to 7,800 today. Her hemoglobin dropped from 11.8 to 7.9. Her hematocrit dropped from 35.9 to 24.0. Her liver enzymes are up, her kidney function is down, a sensitive test to assess for heart failure (a BNP) is elevated to the "moderate heart failure" range. Her CRP almost doubled in 24 hours. I had a serious conversation with the nurse and asked her to please call the doctor on-call (who, fortuitously, is the heme-onc & understands labs) and get her to call Bethany's rheumatologist. We need to know if she is flipping back into MAS or if this is the Kineret causing her cell lines to drop so significantly. The nurse said they are typing Bethany & will have blood held for a transfusion tomorrow morning and she will get it if her hemoglobin drops any further. Her echo from today looks "stable" as compared to the last one done at the local hospital, which means we still have no understanding of what is causing Bethany to be in heart failure.

I feel sick and shaky. Bethany has been grinding her teeth horribly all day long despite having tylenol with codeine, which means she is extremely uncomfortable. The doctor on-call said she doesn't feel

comfortable changing anything tonight, but said we could give smaller doses of T3 at more-frequent intervals. So instead of getting 7.5 mls every 6 hours, she'll get 5 mls every 4 hours. I doubt it'll make a significant difference, but hopefully I will be wrong.

The doctor talked with Dr. K in Portland and a bunch more labs were ordered for tomorrow morning, so we should have a better idea which way things are heading then.


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Hi. Sorry for the quiet, but there hasn't been much information to update. Bethany had her echo but I don't know any results from it. She's not needing oxygen while sitting up, so when she's awake, she is O2-free. The lasix & spironolactone are doing their job & Bethany's fluid status is better (she is breathing better again). There are some emotionally-tough things being discussed behind-the-scenes that I will share once I know what decisions have been made. Thank you, most sincerely, for your prayers. I can feel God's presence all around and am so thankful for it.


Bethany is back in the hospital. The black poop was, indeed, blood (confirmed by lab testing), but no one knows why she has a small GI bleed going on.

She had a fever of 102, a white count over 41,000, inflammation markers all sky-high, and when she was admitted, her oxygen saturation level was 68%, so she's back on oxygen. Over the course of 6 hours, she got increasingly puffy in her face, hands & feet and her breathing was labored in certain positions, which has cardiology believing she is back in congestive heart failure again.

She is being started on vancomycin & cefipime in case the GI problem is related to an infection. Blood cultures were taken & it'll be 48 hours before we know whether anything is growing out.

She has the same arrhythmia she displayed during her last hospitalization ~ the heart block caused by damage to her vagus nerve ~ and when she sleeps, she experiences frequent episodes of bradycardia. While Dr. S (ped) was talking with the cardiologist, Bethany desatted to 80% and we assumed she had pulled her cannula off, but we were surprised to realize that the cannula was still in place. The cardiologist's response was to quadruple her dose of lasix to get the fluid developing removed from her body as quickly as possible.

At this point, there is a 3-pronged approach: cardiology, rheumatology & infectious disease. Cardiology has her on a big dose of IV lasix and spironolactone. They want an echo done tomorrow so they can compare it to her echoes done 2 weeks ago. The chest x-ray done this afternoon showed a small amount of fluid around her lungs, but as the doctor reminded me, that is what it looked like just before she tanked last time, so that doesn't necessarily mean anything reassuring. Cardiology does not know why Bethany is experiencing heart failure since her mitral valve insufficiency is not severe. It doesn't make sense to them that she is having the heart problems that she is, but they acknowledge she IS in heart failure... they just can't explain WHY. Rheumatology is holding Bethany's dose of steroids where they're at (no more weaning at this time). She is still at a high dose twice a day, but we can't take her down anymore when her arthritis labs are starting to climb & look bad. We will continue giving her Kineret injections because it's our only chance of getting her s-JIA under control. No one is feeling very optimistic about that situation right now. Infectious disease doesn't know if she's got an infection yet, but with the high fever & white count through the roof (yes, steroids increase white count, but 41,000 is high, even for Bethany, and it was half that 2 weeks ago when she was on MORE steroids, so that is why there's concern) and the GI problem, they feel they must error on the side of caution. She'll stay on oxygen and the respiratory therapists will keep an eye on how she's doing in that department.

Once again, Bethany is very sick. Prayers for her would be great. I know this journal entry is lacking emotion, but it is 1:26am and I am just now getting a chance to get online & update what is going on. I have not sat down & tried to process any of this yet. When I do, I'll be able to express how I am feeling. Right now, I am too numb to focus on that part.


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Bethany continues to not feel well. Some of it is definitely behavioral, as we will watch her clap her hands together and be totally fine with it, but as soon as someone touches her hands to try and play with her, she scrunches up her face & fusses as though she's in pain. Whether that's a RAD behavior or an autistic thing, we don't know. It doesn't really matter, either. It's just frustrating because it makes it difficult to know what TRULY causes her discomfort and what is completely fake. I picked up a month's worth of tylenol with codeine today, so we can now give her T3 every 6 hours around the clock and that will definitely help with our peace of mind in knowing that she is not suffering.

Her Kineret finally arrived today, so I will begin her daily injections tomorrow. She is not going to be pleased with me at all, as the shots cause her a lot of pain. EMLA or LMX cream ~ numbing agents ~ don't do a thing to help. The pain is not associated with the needle piercing the skin, but rather, with the thick, viscous medication going into her body. Teenagers rate this injection as a 10 out of 10 on a pain scale and honestly, there isn't anything that anyone has found yet that helps make it less painful. I've read a LOT and we tried different things while Bethany was inpatient in Portland and got 3 days' worth of injections there. The rheumatologist said there really wasn't anything that was going to make a difference and I believe him since he's been prescribing this medication to kids for years.

Friday is not going to be Bethany's favorite day of the week. Each Friday, she will have to get labs drawn (1-3 needle sticks in her arms), a Methotrexate injection (1 needle stick in her thigh) & a Kineret injection (1 needle stick in her other thigh). There's no way around it, so... yeah... Fridays won't be fun days for her. If the medication helps, it will be worth it, but if not, I'll have a hard time not feeling like we tortured this child for the 2 month trial period. Unfortunately, there's no way to know if it is going to benefit Bethany outside of just doing it and seeing what happens.

I am interested in seeing what her labs look like tomorrow because for the past 2 days, her poop has been black in color and I'm not sure if it's old blood or a reaction to one of her new medications. If her hemoglobin/hematocrit are lower than they've been, I will call the ped to ask what she thinks. Honestly, I am really hoping it's just a side effect of her meds, but since it's happened for 2 days, it's probably worth mentioning, especially if the labs don't look great. She doesn't have any sign of fresh blood, so I'm hoping it's nothing to worry about.

We'll see how things look tomorrow.


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I know it's Christmas Day, but since both Charley & Adam are working today, our family will be celebrating Jesus' birthday tomorrow, instead.

This is a picture of Bethany taken this morning. This is a fair representation of her mood much of the time. I don't know what to do for her. She's miserable. She's on pain medication & steroids, which SHOULD be keeping her quite chilled, but she's totally unhappy. I know it's Christmas and I should only be writing happy-happy updates, but this little girl doesn't know the meaning of that word anymore and I don't know how to fix that.

What are we, her family, supposed to DO to help this child? IS there any help for her? If so, what is it? The two diuretics we have to give her twice a day to keep the fluid from accumulating around her heart & lungs dries her out & that, combined with her mouth-breathing, makes her lips chap & break apart & bleed. No amount of Vaseline or Chapstick or Carmex or Burt's Bees helps because she licks it off as soon as we put it on. I am having to wean her down on the steroids, which she hates because the only time she feels GOOD is when she's on massively-high doses of steroids (the 400+ mg/day IV dose) plus pain medication (fentanyl was her friend in the hospital). And you know, even when she felt GOOD, she STILL didn't DO a darn thing. She didn't move around on that hospital bed. She sat unmoving except for holding one of her two rattles, which she would shake against her face. Oh, and she would wave her hands & stick her tongue out & smile in her attachment-disordered way when a stranger entered her room. As

http://www.caringbridge.org/tres/images/photos/2/5/6/9/256912/l.hmYsPfaZlaDsguGZ.jpg

long as they stayed away from her, she was willing to schmooze & act happy with nurses, doctors, therapists, Child Life specialists or the chaplain. The smile disappeared quickly when a person touched her, though, and she would try to shove their hands away. When the physical therapist came in to work with her, the most movement she was able to get out of Bethany was two "butt scoots" in one hour. The PT was at a loss for what to do with her. She agreed that without a way to motivate a child, trying to make progress is exceptionally difficult.

*sigh*

I have never felt so totally hopeless about a child's situation as I do about where we are with Bethany. I am so sad about everything going on with her. We brought her home from Ukraine because we wanted her to have a happy life with a family who loved her. Instead, it feels like we brought her out of one hellish existence to thrust her into another one. People say that at least she knows she is loved and I think, "Does she?" Does the love we feel for her permeate through the attachment disorder, autism, pain from her arthritis & general misery caused by having so many medications coursing through her body? Considering she is the most content when she is left completely alone and does not have anyone trying to interact with her, it makes me wonder. Sometimes I think that at least in Ukraine, she didn't hurt at all. She suffered there, but she wasn't aware of it because they kept her drugged to the gills in the orphanage. We brought her home and now she suffers here, too, albeit in a different way. Yes, she is fed & gets her diaper changed regularly & is kept clean & warm, but she also hurts terribly & is misery personified. I understand that God wants every orphan to have a family and we KNOW that we were directed by Him to adopt Bethany, but I'd be lying if I said that we didn't sometimes wonder why He asked us to do this, especially when He knew what was going to happen to Joshua. Despite our misgivings, we do NOT regret adopting Bethany. If we had known about all of her problems before going to get her, we still would have adopted her because, as I said, we were 100% convinced she was the child God wanted us to bring home. I don't regret adopting Bethie. I am just incredibly, tremendously brokenhearted that her life is so awful and nothing we are trying is helping her in a significant manner. This reality is not at all what we envisioned when we were going through the adoption process and it's really sad for everyone involved, including my daughter.

THIS is why I wrote in my last journal entry that everyone involved in Bethany's care agrees it would be more merciful for her to die than to continue living. I had someone leave an unkind comment because they were appalled by that sentiment (among other things). The commenter

said that I'd NEVER say that about Joshua (since I so obviously favor him over Bethany), which is, of course, completely untrue since I've expressed many times that dying will be a gift for Joshua because it will free him from the suffering he endures on a daily basis. The huge difference between Bethany & Joshua's situations is that we are able to give Joshua a good quality of life despite his continued decline and we have not been able to achieve that for Bethany no matter how much we try. Joshua is predominantly happy; Bethany is predominantly unhappy. Joshua wants to interact with his family & giving him a metric ton of painkillers enables him to enjoy life to the fullest extent he can. Bethany wants everyone to leave her alone & giving her painkillers stops her from crying incessantly all day long, but does not give her any desire to explore her surroundings or interact with people and it doesn't make her happy. So yes, at this point in time, since we have not found a way to bring enjoyment to Bethany's life aside from moments here & there, it would be merciful for her to go to heaven, a place where she would be made whole & healthy & she could finally experience the peace & happiness that we have wanted for her ever since bringing her home. That doesn't mean we would be happy if she died. We wouldn't. We would be heartbroken. In some ways, it would be worse to lose Bethany than Joshua because we do have millions of happy memories with Joshua to think of and bring us some comfort (and on the flip side of that, we will be acutely aware of all we have lost & are missing out on once he's gone). We don't have that with Bethie and it would be really hard to think back over her life with us & remember how miserable she was and to know that nothing we did made a profound difference. It is already incredibly difficult to look at her every day & feel I am failing her because I can't make things better for her. If I gave anyone the impression that I wouldn't care if Bethany died, I am sorry. For the record, I would care. Very much so. She's my daughter & I love her. Yes, I get frustrated by her situation. Who wouldn't? It's not like anyone would wish this reality upon their child! My being able to recognize the truth that death would be better for Bethany than a long life here & stand in agreement with her doctors about that truth does not have any bearing on how sad I would be to lose her. I didn't cry when I signed Joshua's DNR paperwork. I fell apart (something that doesn't happen often because I don't generally cry in front of doctors) when I signed Bethany's. By some people's assessments, that would indicate I favor Bethany over Joshua, which is a ridiculous way to judge me (the accusation leveled against me is that I favor Joshua & am content to let Bethany suffer endlessly because I don't care about her at all). I love both of my medically-fragile kids. I ache for both of them, albeit in different ways because while their situations have some similarities, they are also vastly different. And if it has seemed that I'm content to let Bethany suffer endlessly, then really, all I can say is that anyone who thinks that needs to learn to

read with comprehension since my journal entries have been filled with my horror & anguish at the pain my child endures, so I'm unsure how they've come to the conclusion that I don't care.

I don't know what to do for Bethany. Her doctors don't know what to do. Just like her big brother, she has a mixture of conditions that don't play well together. Severe arthritis causes fluid to build up around her lungs, which are diseased & therefore forces her heart, which is not perfectly functional, to work harder, which leads to congestive heart failure. Severe autism & an attachment disorder means she has very little drive to connect or interact with the people who love her; instead, she schmoozes with strangers, giving smiles & hand waves to them while reserving her rolling eyes, glares & anger for her family (I suspect part of that is because she's mad that we're not making her feel better). Not wanting to move makes her joints stiffen. Forcing her to move makes her angry & then miserable. Steroids cause fluid retention. Diuretics pull the fluid off. Pain is chronic and severe. Opioids calm the pain but leave her tired & "dopey". Down syndrome comes with low tone & constipation is already a challenge because of that, so adding opioids makes constipation worse and requires more medication to manage. It's a never-ending, vicious cycle. Treating one symptom sets off a cascade of events because of her other conditions. It's a mess. There's no better word for it. And yeah, it's really not fair. I know life isn't about being fair, but sometimes it seems that one kid gets nailed with way too much. I have thought that about Joshua at times, but I think it about Bethany pretty much every day. Joshua is dying, but he's got quality of life as he goes. Bethany has multiple conditions that could lead to her dying very young and we can't seem to get a grip on any of them sufficiently enough to get her to a place where she can be content in her existence. It sucks for her. It sucks for our entire family. It's not fair to anyone, most especially, her. And because of that, I feel utterly hopeless about Bethany. Hopeless that things will ever get better. Hopeless that anything we try is actually going to make a difference for her. It would be wonderful if God has something coming down the pike that is going to change Bethany's life in a magnificent, positive way, but I no longer have any genuine hope that it will happen. Still, it'd be great if 2013 is a good year for her. I don't really see how it could get much worse.


Sorry for the lack of update yesterday. Once I was home, computer time was limited, especially after Joshua woke up. :) It is wonderful to be home!

Bethany is fine. She's exactly the same as before, except now she isn't in pain, which is obviously a huge relief.

With congestive heart failure added to her list of medical conditions, no one really knows how long she is going to live. We don't see her leaving us in the near future, but if the medications she's on lead to adverse side effects or she gets a bad infection, things could get dicey very quickly. We are keeping her DNR status the same because if she were to code, we would not want to resuscitate her since we all know the life we would be trying to bring her back to and it's not a life anyone would want for their child. It's really awful that everyone involved in Bethany's care agrees it would be more merciful for her to die than to continue living, but there's the reality.

We are hopeful that we'll see more smiles and more happiness from Bethany now that she's not in severe pain, but so far, she has been content to just sit and watch the world go by. Tomorrow, I will be resuming her standing therapy and I anticipate she will hate that as much as always, but hey, maybe she'll surprise us and be okay with it!

Thank you so much for the prayers and words of encouragement and support during Bethany's hospitalization. I am very thankful that God has given me a community of readers who care about me & my kids.


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Due to a major storm that closed the mountain passes between here and my home, Charley was unable to get up to Portland to pick up Bethany & me. We've spent the day making alternative plans.

B and I will be flying home tomorrow. We're staying overnight at the Ronald McDonald House and will take a cab to the airport tomorrow (the hospital gave us a carseat to use) and then fly home. There's been a lot of details to organize, like getting Bethany's meds and letters stating that yes, those are her prescribed medications so that we can get through security without hassles and getting a feeding pump delivered since ours is at home (remember, she was flown up here, so we have NOTHING ~ not even diapers or a coat!). It would have been a

million times easier to just stay one more night in the hospital and that is what we had thought was going to happen, but then they said they couldn't justify keeping her another night, so we had to go. As I write this at 6pm, we are still sitting in the hospital, Bethany still hasn't been de-accessed, I still haven't gotten the feeding pump or a letter to show the airlines, and my patience is wearing thin. Still, God provided the money for the plane ticket & many people in the hospital have been working to get things organized, so I shouldn't complain too much.

I'll update again after we are home tomorrow afternoon. Please pray that there are no snafu's, no problems, no delays, and that everything goes smoothly and we have a safe (short) flight home.

Thanks.


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Bethany is getting discharged from the hospital today. She'll go home on 2 diuretics to help keep fluid from accumulating around her lungs again, steroids (prednisolone), Kineret (daily shot) and will continue getting methotrexate & zantac. She'll be on feeds for 16 hours per day, just like before she went into the hospital.

Dr. K gave me an outline for how to wean Bethany from the steroids & then we'll evaluate how well Kineret is or is not working for her.

Bethany is... Bethany. She sits in the hospital bed & plays with one of two rattles and she smiles at people & waves at them as long as they stay a good distance away from her. She's adorable, but I really want more than a cute little rock for a daughter. I want more for her than to sit in one spot all day every day. Unfortunately, no one knows if that is possible and with her combination of conditions, we don't even know if she's going to have a future. I've had some hard talks with several doctors while we've been here and at this point, we're working for quality of life over quantity.

She's not hurting because steroids and tylenol with codeine (T3) work well together. Dr. T (palliative care) said that after Bethany is off

steroids, if we find the T3 isn't enough to keep her comfortable, he'll start her on a fentanyl patch. His goal is to make sure she does not suffer. I have the utmost confidence he'll take good care of Bethie because he is fantastic with Joshua and I'm thankful to have the palliative care team involved with Bethany now.

I am ready to go home. It will take a few days to get used to the new routine of medications, but there's always an adjustment period when big changes are made. I just want to hug my husband and hold my kids. This past week has been an emotional suckfest. While I have zero understanding of what God is doing or what His purposes are, I still wholeheartedly believe that as long as I am obedient to Him, whatever the cost, He will work out the details & I will make it through to the other side of this mountain range. That said, I really do wish He would give me some inkling of how He is piecing together all of these crazy details of our life because this is pretty much the hardest journey I've been on in my adult life.


Sorry for no update yesterday. I hate it when people have something serious going on and leave everyone who is praying & caring about the situation hanging, so I sincerely apologize for doing it, myself.

Yesterday was a hard day for me, emotionally-speaking, which translated into my not knowing what to write. I'll admit, after crying myself to sleep last night, I sit here today, still not feeling great, but for the sake of keeping up with what's going on with Bethany, here I am. I want to keep track of what goes on with Bethany just as much as I do for Joshua (heaven forbid anyone accuse me of not caring about her because she's adopted), even if it doesn't always come across that way.

Bethany had needed oxygen overnight (not last night, but night before that), so she had arm restraints on. As a result, she woke up very stiff in her upper body. When I removed her restraints after the nurse took off her nasal cannula, I gently rubbed her arms and bent her elbows, helping them to loosen up. This was painful for Bethany, so I asked the nurse if I could get a dose of pain medication before I continued with moving her joints. The nurse told me that all she had orders for was tylenol. Plain tylenol. Not fentanyl. Not even tylenol with codeine. I asked why Bethany didn't have AT LEAST T3 ordered since that is a home medication, but the nurse didn't know. She did not seem to understand why I would think my child needed an opioid medication & I didn't feel up to arguing or explaining, so I told her, "Sure, bring the

tylenol; thanks," with the plan to talk to the first doctor who came in. I knew I could get the situation fixed; it was just a bit frustrating to have to deal with it at all since I thought it had gotten all worked out while Bethany was still in PICU.

Later in the morning, I laid Bethany down to change her diaper. I walked away to put the diaper on the counter so the nurse could weigh it, washed my hands, then turned around to see Bethany struggling to get herself into a sitting position. For 15 minutes, I watched as she tried to sit herself up. All morning, Bethany had trouble getting herself from a lying-down position to a sitting position. No one knew why. My concern was whether she was losing another skill since she's already lost so many milestones she'd previously achieved (pulling to a stand, crawling, cruising, babbling, playing with a wide variety of toys in an appropriate manner, etc). Thankfully, by the afternoon, she was doing better. It was still taking her longer than usual to maneuver into a sitting position, but she could do it. I asked if anyone had any idea WHY she was struggling to do something that she's been capable of for years and no one had an answer. For some reason, it appears I am the only one who thinks it's a problem when a child loses milestones and regresses. One doctor told me there was no reason to evaluate for a degenerative process because "It won't change the outcome." Super. That sucked to hear.

She got her first shot of Kineret and yep, the reports of it being a 10 out of 10 on the pain scale seem to be correct. I'm a bit queasy at the idea of having to inflict this on her every day, especially since it carries some big risks for Bethany, but with it being the last medication we have available to try (Dr. K decided Enbrel would be useless to try because, as a TNF inhibitor, it wouldn't touch her systemic symptoms), we really don't have a choice.

She had two members of the CCA (Children's Cancer Association) music therapy team come in to sing for her and she LOVED it! I took a video ~ hope you enjoy it as much as we all did. :) The explanation on the video is for CCA ~ the team members asked if they could have a copy of the video with an explanation of who Bethany is and what her medical challenges are. https://www.youtube.com/watch?v=JMVRSVkjERc

I know everyone will say how happy Bethany looks, but as her rheumatologist reminded me, it's artificial and all due to steroids. Bethany loves steroids and when she's on the super-huge-mega doses she's been getting in the hospital, she's at her best. She can move her hands and bend her fingers and the swelling in her joints goes down and she looks GREAT and acts HAPPY. It's fantastic ~ really ~ and I

enjoy how adorable she is and the smiles and the respite from what is her usual reality. What I cannot forget because we've been here so many times before (and her rheumatologist does not forget, either) is that this is temporary. As soon as we begin weaning her off steroids, we will lose this happy Bethie. It always happens. She can't be on mega-high-dose steroids long-term. That would eventually destroy her body & kill her. So yes, please DO enjoy the smiley, happy little girl in that video (I am enjoying her for as long as the steroid rush lasts), but do not think that is the child we live with every day and please do not judge the things I say based on that video. Remember the video clips from the complexbethany.blogspot.com blog and know that *those* videos more-accurately depict what Bethany's life is like.

In the evening, the rheumatologist came by to tell me that since we started Kineret, there was something else I needed to know. Kineret can also cause pulmonary problems. I laughed in disbelief & said, "Oh right, because she needs MORE pulmonary problems!" He replied, "I know, but she has a serious disease & more than that, she has a severe form of a serious disease and unfortunately, to treat her disease means we have to use medications that can cause serious problems, including life-threatening risks. That's why we'll have to watch her very carefully."

Yep. That's where we're at now. Oh, and this is our last-ditch effort. If Kineret doesn't work, we'll have only steroids available to try to control this awful disease. And while yes, her little body LOVES steroids, keeping her on the high doses she would require to keep her disease controlled would lead to some really ugly things. There's a reason long-term steroid use can't work... please pray that Kineret works for Bethany. Really. I can't emphasize enough how badly we need for this to work. It'd be nice if it would work without killing her in the process, too.

And speaking of pulmonary problems, cardiology added a second diuretic to Bethany's regimen. The lasix wasn't enough and the dose they were using was lowering her potassium, so they couldn't go up on the dose; instead, they added another medication to dry her out so her lungs & heart don't get surrounded by fluid. She'll be discharged on both medications. Her arthritis will continue causing her to get fluid around her lungs and her lungs are permanently damaged, so they can't tolerate too much fluid around them before it negatively affects her heart, which makes congestive heart failure worse.

See why we need the Kineret to work? If we can't keep her arthritis under control, her lungs & heart will suffer & that could be fatal. If the medication affects her lungs in a bad way, that could be fatal. If the

medication flips her into MAS, that could be fatal. It's a good thing we have nothing to feel concerned about, right?

Today or tomorrow, Bethany will be seen by rehab, OT, PT, and (I think) a developmental pediatrician. She'll also see her other specialists (cardiology, rheumatology, palliative care). RT has already come by this morning. She's looking pretty good today, I think, as she sits in bed and plays with her rattle.

I'm physically rested, but emotionally very tired after yesterday. The realization that we could lose Bethany before we lose Joshua was shocking, and I think I surprised Dr. K when I told him (in the presence of the Child Life specialist, who is a friend of mine) that there are things worse than death, so if we can't get Bethany's condition under control in such a way that she can function & progress developmentally & enjoy life, then really, it would be merciful for her to go to heaven, where she could be pain-free & healed completely. The Child Life Specialist agreed. Dr. K didn't say anything.

If you think what I said sounds cold-hearted, let me assure you, it's the truth. Watching your child suffer EVERY. SINGLE. DAY and lose skills and have zero quality of life makes an impact on a mother's heart. I have watched Bethany hurt so severely it causes her to vomit but no one would do anything to help her & it took me 8 months of fighting to finally get a doctor to write a script for pain medication, but even with pain relief, she has multiple medical conditions that are working against each other & will shorten her life (how unfair is *that*?!?!). I have watched the light go out of her eyes as she retreats further & further into her little autistic world because there is so little in the real world that can entice her to want to interact for more than a few minutes at a time on her terms. I have watched Joshua writhe on the floor, holding his head & screaming because the pain was so intense, and there was absolutely NOTHING I could do because the largest doses of the multiple pain medications he was on did not touch his agony. So yeah, there are things worse than death. If you think otherwise, thank God for your sheltered, naive existence that makes it impossible for you to comprehend begging God to take your child to heaven so their suffering can end (while simultaneously sobbing because the thought of having to live without that child is a literal stabbing pain in your chest). I can't even begin to tell you how much I would like to live in that reality again.


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Bethany got moved out of PICU tonight (about 8:30pm). They took her off oxygen, but had to put her back on it after she couldn't maintain her saturation level above 85% while sleeping. Still, she's moving forward and improving. Dr. K (rheumatologist) came by again tonight and marveled at how great steroids are for Bethany. It's true; the girl LOOOOOVES being on high-dose steroids. It's too bad life can't be maintained on them long-term; otherwise, it would definitely be our medication of choice.

She'll start Kineret either tomorrow or Wednesday, her steroids will start being weaned (awww, poor girl won't like that) and she'll be switched from IV Lasix to oral since she will be going home on that medication.

Dr. T, the palliative care doctor, came by with 2 other docs (Kathleen, who is his colleague in the palliative care program, and Melissa, who will be his replacement after he leaves for his new job) and we had a nice, long chat about Bethany & Joshua. He suggested starting Bethany on a 12.5 mcg/hr fentanyl patch to keep her pain-free & happy. He said that having to change fentanyl patches for 2 kids would be easier than having to dose 2 kids with oxycodone every 4 hours. :) I'm not arguing with the man about that! I'm glad I won't have to worry about making sure Bethany doesn't get Joshua's dose of oxycodone by accident, which I could see happening if they were taking the same oral medication. No one will mess up fentanyl patches. They're sized differently and the 12.5 mcg patches are tiny. Joshua's 100 mcg & 75 mcg patches are huge by comparison. So yeah, I'm happier with the idea of Bethany being on a fentanyl patch. The other benefit is that I don't have to remember to dose her with pain medication every 4 hours ~ the patch gets changed every 3 days (or every 2.5 if you're Joshua). MUCH easier & better pain coverage for Bethie, too, which is a win-win for everyone involved!


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I have got LOTS of news about Bethany. Of primary importance is that she is now considered out of danger & is going to survive this hospitalization! Yea!

Of secondary importance is that we finally have an understanding of what happened to get her to such a scary place: congestive heart failure brought about by pleural effusions caused by her poorly-controlled systemic juvenile idiopathic arthritis. Dr. Kingsbury & the PICU doctors & I had a long discussion this morning to talk about the complexity of all of this. I'll try to summarize as simply as possible for those who are not familiar with medical stuff.

Okay... systemic arthritis affects the entire body, both outside & inside. It causes inflammation internally and can cause fluid to collect around the lungs when the arthritis is severe. This normally is not a big problem, as arthritis treatment controls the systemic symptoms of the condition and a person can usually compensate with a small amount of fluid around their lungs. In fact, Bethany has had a small collection of fluid at the base of her left lung for months and it was not causing any problem for her.

When the arthritis is NOT responding to treatment, the systemic symptoms can get worse but a person's labs won't necessarily show any big problem right away. That's what we saw with Bethany's labs ~ her inflammation markers have been low for months & we thought her systemic symptoms were being pretty well-managed even though we knew (from her joint stiffness & increasing pain) that her arthritis, itself, was not being controlled well at all.

Because of her s-JIA being poorly-controlled, she began to collect fluid around her lungs. And then, because she has a congenital heart defect that is "repaired" (the cardiologist put the quotation marks around that word as he explained this to me, saying that though her heart is repaired, it is not perfect & won't ever be), the increasing fluid around her lungs, coupled with her parenchymal lung disease (which I learned she's had for over a year, but it is now substantially worse) put an increasing workload on her heart, which didn't appreciate the strain. That is where we saw the heart block pop up ~ the Weinke-bach arrhythmia that was triggered by the injury her vagus nerve sustained during her heart surgery in August ~ and when Bethany began retaining fluid quickly, which was exacerbated by her body flipping into Macrophage Activation Syndrome when her bone marrow got too

stressed.

So, what we saw happen on Saturday while she was in the hospital at home was the increasing fluid retention caused by her being in congestive heart failure as a result of the increasing amount of fluid around her lungs, which was caused by her rheumatoid disease being poorly-controlled & thus flipping her into the life-threatening condition of MAS. She has a bunch of conditions that mixed together to create what one doctor called "the perfect storm".

Bethany is being weaned off the high-flow oxygen and is anticipated to be put back on just a nasal cannula later today. All antibiotics have been stopped. She is now on a small dose of lasix given twice a day because her weight is back to 12.7kg (down from 15kg when admitted ~ that's almost 5 pounds of excess fluid pulled off her lungs! No wonder the child was struggling to breathe!). She is continuing to receive steroids at a "generous" dose, but not the super-high dose she's been getting. Her labs look *a lot* better and all 3 cell lines are bouncing back, which is a sign that the MAS has been reversed. Dr. K (rheum) wants to start Bethany on Kineret while she's here so that he can see how she's going to do and so she's already here if her bone marrow completely revolts against the medication and tries to throw her back into MAS again.

She will still be put on palliative care and I will be meeting with Dr. T sometime today.

She will also have a consult with her GI to see if we can determine why she is not doing well with feedings. The attending doc said she appears to have a problem with motility and the dietician suggested having her on a different formula that is already mostly-broken-down & putting her on a motility medication (like Reglan or Erythromycin, which is what Joshua takes for his gut motility abnormality).

Her lung disease is permanent and there's no fix for that. The cardiologist said the million dollar question was whether her heart working harder caused the problems with her lungs (the fluid accumulation, which began because of her arthritis but got bad, very quickly, because of congestive heart failure) or whether her lungs being surrounded by fluid caused her heart to work harder. Both would lead to the same result and it's a "which came first, the chicken or the egg" question, but he doesn't know the answer and therefore, we don't know if this is going to happen again.

I asked about a long-term prognosis and was told by the PICU attending doc that he would come talk to me, privately, later today. Dr.

K (rheum) said that if we run out of treatment options & are left with only steroids to try to control Bethany's arthritis, it will be bad & she'd probably only have a few years to live. He quickly followed that up by saying that he has not lost hope yet and he hopes that we can keep her controlled with Kineret until the FDA approves a new medication that we can then try (that should happen sometime in 2013).

Sooooo.... there ya go! LOTS of information. Good news is that she's not in danger of dying in the immediate future as a result of this crisis. The not-so-good news is that she's got permanent damage to some organ systems and her prognosis isn't really a great one. Still, she looks a lot better and if she does well today, she should get moved out of PICU tomorrow and home by Christmas, which is incredible considering how critically-ill she was on Saturday. To go from "This may not end well," and "Have you given any thought to what you would want to do if she dies?" (that question was referring to burial, cremation, transporting her body down home to cremate or doing it up here, etc) to "She is doing well now. We should be able to get her out of here in time for Christmas," is exhilarating. I am so grateful for the fantastic medical care Bethany has received here and SO thankful for the hundreds of prayers lifted to God on my little girl's behalf. I praise God for giving wisdom to everyone who needed it. I am so happy today!!!!


Some good news: the Lasix is working to pull off a bunch of the 2kg in fluid that Bethany was distended with. She's down 800cc (almost 27 ounces) and as a result, she's breathing more easily now. Praising God for this GOOD development!

Please pray that Bethany will get some sleep tonight. For the past 2 days, she hasn't been able to sleep more than a short stretch before waking again. She sleeps in a sitting position since laying back is too uncomfortable for her. She needs sleep to heal and, at the risk of sounding selfish, I need sleep to continue functioning. I have gotten about 10 hours of sleep over the past 3 nights and I need to get some tonight. The only way that is happening is if Bethany sleeps, too.

I am going to go try to get her down for the night and then I plan to lay down, myself. If nothing significant happens overnight, I will update again tomorrow after meeting with the rheumatologist and palliative care physician. If something important happens tonight, I will update right away.

Thank you for your prayers and words of comfort and support and love

and caring. I am blessed to be surrounded by the family of God, even if it's not in a physical sense. Good night.


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Bethany is sleeping right now with the help of versed and fentanyl and a bed inclined 60 degrees to make breathing easier for her. She's getting face-mask CPAP for 10 minutes every 4 hours to open up the parts of her lungs that have sort of collapsed on themselves (not a true collapsed lung, but pockets ~ that's how the RT explained it to me), followed by deep suctioning of her lungs to suck out

as much gunk as they can. As you might expect, Bethany HATES these treatments. She is still on high-flow oxygen but desats into the 80's at times, anyway. Her heart rate is more steady now after a lot of arrhythmia & alarms going off for that.

I slept/dozed for 1.5 hours and will try to rest during the day if the opportunity presents itself.

While Bethany doesn't look like she is in awful condition, everyone is being very deliberate and sober in their conversations with me and making sure I understand how serious this is. Trust me; I get it. I am very accustomed to watching Joshua look good up until the moment he stops breathing. They said Bethany is in an equally-precarious position (not for the same reasons as Joshua, of course). The good thing about being up here is that pretty much everyone knows Joshua from his years of hospitalizations, so transitioning the rapport over to Bethany is easy. Everyone is being sweet & caring to my girl, which I appreciate.

I haven't met with any doctors yet, but I was assured they were being called in today versus waiting for tomorrow.


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We're here at the hospital (Randall Children's) in Portland. Bethany did all right during the trip up. She is now on high-flow oxygen, is getting Lasix because she's gone from 13kg yesterday to 15kg today, will be seeing cardiology and rheumatology and infectious disease in a few hours. The PICU intensivist (one of our favorites, thank you, God) said this may not end well. In addition to her bone marrow flipping out (the MAS) and her lungs being diseased (still don't know what s causing the problem there), her liver is steadily enlarging & she's having more problems breathing and she's got rales on the right side, all which are indicative of a cardiac problem. She's got both a diastolic and systolic murmur ~ one of those is new (she's had a murmur from a mitral valve leak, but Dr. B emphasized this is new and abnormal). She has a lot of organ systems struggling right now. They are holding off on the bronchioalveolar lavage because the concern is that if they put her on a vent (which they have to do to do the test), they wouldn't be able to get her off of it again. Dr. B said this will not be a straightforward thing because her severe immune suppression is going to muck up labs and seeing the full picture (ie: she has no fever, which might be because this is more cardiac than infection, but might be because her immune system is suppressed and can't mount a fever). They are getting palliative care involved, as well (same doctor that manages Joshua's care). They will keep her comfortable and out of pain ~ that is a huge priority and I am grateful for that. After the past 8 months of suffering this baby girl has endured, being kept pain-free is a huge blessing. This is scary serious and I feel like I'm going to throw up. I'm shaking as I try to type. Dr. B wants me to try to get some sleep since I've been awake since 6:30am yesterday (over 23 hours), so I'm going to try. Please keep praying (thank you to everyone who has been). I will update after I take a nap.


Bethany is in full-blown MAS & her breathing has worsened. Her white cells have dropped 10,000 since yesterday and are now at 5,800. Her platelets are 122,000. Her red cells are 2,570. I know this means nothing to a lot of you, but it's BAD. Her ferritin is over 1500. Her LDH is over 1000. She's being flown to Portland; may be put on a ventilator for the flight. Charley, Adam & Joshua are heading to the hospital to pick up Megan & drive the truck home ~ they're bringing Joshua so I can say "goodbye" to him since I won't be able to see him for a few days (or longer). My heart is being torn in two. Please pray. This is life-threatening and exceedingly frightening.


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Megan and I think Bethany is working harder than usual to breathe and her belly is, to us, obviously distended. The nurse she has today agreed that her stomach is protruding, but said that since Bethany has bowel sounds, it's nothing to worry about. In light of Dr. S telling me to be very vigilant and report anything different or abnormal since Bethany could tank with very little warning, I am not reassured by the nurse's assessment.

Here's the video I said I'd upload and share showing the weird breathing this morning: http://www.youtube.com/watch?v=C-vxyC9wGn4&feature=youtu.be

She is currently asleep and is doing it again. I am pretty sure it's not normal. Megan and I have never seen her do this, anyway. http://www.youtube.com/watch?v=osecPYFYAIg&feature=youtu.be


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Megan's tests came back normal, so the ER doc said that while there is nothing emergently wrong, he wants her to have an echo this next week to check for the heart issues that are associated with Marfan's. SO.... thank you, God, that nothing serious showed up and thank you that Megan is feeling okay now. Thank you to everyone for praying and caring!

In Bethany news, they tried taking her off oxygen and she was satting at 84-86% while she was sitting up (just like yesterday, and they didn't leave her off long enough to watch it drop further), so the respiratory

therapist put her back on oxygen, but turned it down by 0.25L to see how she'd do. Her sats are in the low 90's now with the oxygen going. RT said she is not ready to come off O2 just yet.

She's currently getting her infusion of IV steroids. She has gotten quite puffy overnight because she hadn't peed in almost 18 hours, but she has finally starting urinating and as long as she continues, the anticipation is that she'll get rid of the excess fluid. If she doesn't, they'll give her something to help. When she sleeps, she breathes so forcefully that her body jerks/jolts. I took a video of it & if I can get the hospital internet to cooperate, will upload it and share a link later. Her respiration rate remains in the 45-70 breaths per minute range, which is really fast, but no one has commented on it other than to come in and silence the alarms when they go off, so I am assuming that it's an expected thing at this point.

She is continuing to receive antibiotics and IV fluids. Aside from that, today is pretty much a "holding pattern" kind of day since it's the weekend and we're waiting to see if the blood cultures grow out anything that would give the Infectious Disease doctors in Portland a clue about what bug is causing the problem in Bethany's lungs.


In the ER with Megan. She's hooked up to a heart monitor, they're going to do an EKG & chest x-ray & draw blood. The doctor said he is recommending Megan have an echo & Holter monitor in the next week, which can be done on an outpatient basis unless, you know, I end up in Portland with Bethany.

Turns out my daughter has weekly episodes of almost passing out and is having arrhythmias and weird stuff with her eyes. She's learned to recognize the signs of what's coming and sits herself down before she actually passes out. She's never mentioned this to me before because, as she explained to the doctor & me, "We deal with big stuff every day. This seemed like a little thing and I never put the almost passing out with the weird heart rhythm." THIS is the fallout from living the life we do with severely medically-fragile kids ~~ the stuff that would get a normal person's attention barely registers as more than a blip, which is really quite scary when I am sitting here listening to a doctor talking about the possibility of Marfan syndrome or a heart problem in connection with my oldest daughter.

So... deep breath... we'll get these tests done and go from there. The doctor did say that if anything significantly abnormal shows up on the

EKG, he'll be ordering the echo to be done while we're here.

Praying for this to be nothing, but they told us already that 21 year olds don't pass out or almost pass out on a regular basis for no reason, so I guess I need to pray this is nothing serious.


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I want to clarify a few things in case I wasn't clear on my last update....

The reason Bethany's labs and whatnot are not showing that she's super-sick is because her immune system is SO suppressed that her body can't launch a response to the germs in her lungs. Those germs ~ bacterial or fungal, we don't know yet ~ are damaging her lung tissue, and her body is not doing anything to fight them off.

The steroids she has to have every day via IV are necessary to keep her from flipping into full-blown Macrophage Activation Syndrome, which she would have a difficult time surviving in her compromised state. The steroids will further suppress her immune system AND mask signs of illness, but we cannot NOT give them to her.

Her heart arrhythmia is being caused by stress to her vagus nerve, which is secondary to what is happening with her lungs. The problem is not primarily her heart. That is the ONLY "good news" that I got today about all of this.

Dr. S said Bethany has so many organ systems having major problems and everything is complicated and not really responding well to treatment.

Thank you for praying. I am thankful that we were at the hospital today when she tanked. If we'd been at home, she would have gone into respiratory failure (most likely) and we would not have known it was happening until she passed out from lack of oxygen. I'm thankful for Dr. K telling Dr. S about the weird heart rhythm he noticed and for asking her to please monitor it today while she was at the hospital.

Without the heart monitor on, we would not have known about the arrhythmia Bethany is experiencing. I am thankful for whatever God did in Dr. S's heart today because she was as nice to Bethany as she's EVER been to Joshua (which is saying something, since she loves Joshie) and she was extremely kind to me, as well.

I got a call from the nurse at 6:30am. Bethany was okay (though she did have a scary episode of her respiration rate dropping to 8 & her O2 and heart rate dropping, which had a bunch of people in here to deal with that), but she was calling to tell me that MEGAN had passed out and had what appeared to be a 30 second seizure. They'd checked her blood sugar and that was normal. Her vitals were okay , but they felt she needed to be checked out & she was refusing to go to the ER. I called Adam at work and told his boss there was a medical emergency and I needed Adam to please come home. When he got home, I drove to the hospital. Megan seems to be mostly okay. She is still feeling shaky and not fully "herself". We are going to get her fed and then I'm taking her to the ER to make sure there's nothing serious going on. Since she has had bouts of almost passing out in the past (she sits herself down when she senses it coming on) and Charley has a couple nieces who have epilepsy, I'm refusing to let my daughter blow this off. Especially since she is still feeling "off" and it's been almost 4 hours since the episode.

Please pray for Bethany, for Joshua, for Megan. I am full-blown sick (just a bad cold, but I'm not feeling great) and "exhausted" doesn't describe the level of fatigue I'm experiencing, but I can do all things through Christ who strengthens me. Please pray for my continued ability to do & be all my children need me to do & be. I'll update on Bethany & Megan when I have more information.

I don't know what the day holds, but I know that God is working out something here and no matter what, He remains good and He is in control of everything. I really have to hold on to that knowledge because honestly, my emotional side is having a very difficult time with all of this.


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Dr. S has been here for 2 hours. This is NOT pneumonia, but they do not yet know what it is. She has parenchymal lung disease and big patches of "yuck" in both lungs (right worse than left). The biggest concern & suspicion is that she has some bacterial or fungal infection that is sitting in her lungs and her body is not launching an immune response against it at all. She shows NO signs of illness. No fever, no nasal discharge, her labs do not show any sign of infection because her body is not registering there's a problem, but her need for oxygen + heart arrhythmia + chest x-ray prove there is something serious going on.

They've cultured blood and ran the viral panel, which Dr. S said won't come back showing anything because they know this isn't viral, and they're treating as though she's got some community acquired bug, but Dr. S said the infectious disease doctor is worried this could become a huge mess since Bethany's body isn't even trying to fight the bug at all.

She said to keep my bag packed because at the first sign that Bethany is not improving, Portland wants her so they can do a bronchoalveolar lavage (they stick a tube down her throat, into her lungs, pour fluid in there to break the gunk loose, suck it out and culture it to find out what it is) and further treatment.

The cardiologist does not think she will go into a fatal arrhythmia. He said she is having atrial ectopy caused by the damage done to her vagus nerve during her surgery. He said it is a very odd pattern called Weinke-bach.

Rheumatologist is concerned that Bethany could be trying to flip into MAS. Her labs today are worrisome for that. Only her ferritin level is keeping her from that diagnosis. She will be getting IV steroids every day to attempt to keep her from going full-blown into that mess.

I was encouraged to go home and get some rest, then return for the day tomorrow morning. There are a LOT of reasons Bethany would potentially get flown up to Portland. The docs up there are ready for her if she needs to come up. We are going to take this day by day, I was told.

I'm heading home to get Megan (leaving Emily here to be with Bethany) and then I'll drive back to the hospital, pick up Emily and drop off Megan, then come home for the night.

It goes without saying that Bethany needs continued prayer.


The ped's nurse called a few minutes after I sent an email asking if someone would please give me some idea what is going on with my daughter. She said Dr. S has been conferring with 3 specialists for the past 90 minutes and it will be a bit longer before she can talk to me. Right now, they know she has a bad pneumonia. She will stay in the hospital here (far as I know). The cardiologist has not yet seen the EKG strip and is waiting on it. Infectious disease is

involved and giving orders for medications. Rheumatology wants her on high-dose IV steroids. I asked, "So this isn't very serious and I can stop worrying?" Nurse K replied, "Um... no... it IS serious." I asked, "But we can treat it here and she'll be okay, right?" She answered, "We hope so. Dr. S will be talking to you as soon as she can. Hang in there."

Respiratory therapist has been in here a couple of times. Nurse has been in to run a full viral panel. I'm told Dr. S will be here eventually, as she is still talking with Portland doctors. In the meantime, Bethany is on full isolation in PICU, on 2 liters per minute of supplemental oxygen. They've drawn blood from both her port and a peripheral vein (they do it simultaneously) to culture. She's gotten started on 2 antibiotics and they're checking her for mycoplasm because there's a chance she's got tuberculosis in her lungs now (she was treated for it when we brought her home from Ukraine, but it's possible she's had some lying dormant that got activated by the Actemra). Mycoplasm can also cause pneumonia (a different strand of that bacteria, I think... I don't fully know yet, as there's lots of information being tossed out and I have zero experience with this kind of medical issue, so this is all new to me).

Bethany cycles between being calm and being very agitated/distraught. She shakes her head rapidly back and forth any time anyone (including me) gets near her. She is NOT happy. When she has arrhythmia, her breathing gets much more labored, her respiration rate goes very high and she gets really upset. Her nurse said it doesn't feel good at all to have that happen (understandably).

Again, I'll update more when I hear from Dr. S.


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Please pray!

I brought Bethany to the pediatrician's for her pre-Actemra appointment and Bethany was satting at 88%, so Dr. S wrote orders for her to be put on a pulse ox to monitor her oxygen level and a heart monitor to watch or arrhythmias (in light of Dr. K hearing the weird rhythm on Wednesday). We came to the hospital for her Actemra infusion and a solumedrol infusion (IV steroids) and her labs were not good enough to allow her to receive Actemra. She did get the steroids, though. The scary part is that she was desatting and her oxygen level dropped to 69% (and stayed there) while she was laying down (this was happening from the minute we walked in the door & got her hooked up. It had NOTHING to do with the steroid infusion). We sat her up and it stayed between 78 and 84%. She was put on oxygen at 2 liters per minute to get her saturation levels back to normal.

Her heart began having arrhythmias and bradycardia (irregular heart rate and slow heart rate), so she was taken for a STAT chest x-ray. The pediatrician called the cardiologist and rheumatologist in Portland. The cardiologist said she was to be admitted locally (for now). When Bethany showed increased difficulty breathing (she was desatting to 84% even with supplemental oxygen) and even more irregularities in her heart rate, a STAT EKG was ordered and sent up to the cardiologist and Bethany was moved to PICU.

I am in PICU now with her. I still have not heard from Dr. S at all. I have no idea what is going on or if she will be flown to Portland. There is talk amongst the nurses of heart failure or fluid build-up around her heart. That is just talk right now since no one has heard anything official yet.

I don't do well in the "not knowing" phase. Please pray for answers to come quickly and for this to not be as serious as it seems at this time.

I'll update when I know something more.


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Thank you for your prayers ~ they worked! As is typical for Bethany, however, it wasn't a slam-dunk victory.

The good news is that we came home with a prescription for pain medication. Dr. K had said when he first walked into the room that he was NOT going to give us a script because he does not put systemics on narcotics and he is not comfortable managing narcotics for a child long-distance (except for one kid he treats who is terminal), but after listening to Bethany wail in pain for 15 minutes, non-stop, he looked at me and said, "Okay, this is ridiculous. I'm going to do what I just said I wasn't and give you a script for narcotics." That was totally a God thing. I didn't have to argue with him or convince him of anything; I just sat there and let Dr. K experience a small taste of what life with Bethany has become and he didn't block it out. I praise God that He moved on Dr. K's heart and the doctor we had today cared and agreed that it is inhumane to not do something to relieve Bethany's suffering.

Dr. K said Bethany's arthritis is very severe. Her joints look very bad. Her labs look "quasi-MAS" (ie: sort of like a person who is getting ready to flip into Macrophage Activation Syndrome, which is a life-threatening condition: http://emedicine.medscape.com/article/1380671-overview). He said that as we approach the 2 year anniversary of her diagnosis (in January), her disease is still not under control & she is getting worse over time, not better. I asked if she was going to die from this disease and he answered that systemics (those with systemic-JIA) have a higher mortality rate, yes. That isn't the same as saying he thinks Bethany is going to die from her disease, but I understood that the point was it could happen.

Actemra is no longer doing much to help Bethany. It *is* somewhat controlling her systemic symptoms, but not nearly well enough to justify continuing to use it, especially since Bethany is receiving a HUGE dose already. The only other aisle-1 inhibitor (I *think* that's what it's termed; I'm not looking at my sheet right now, but it's the same category of medication that Actemra is in and the only category that specifically treats Bethany's condition) available to treat systemic-JIA is Kineret, the uber-painful daily shot. We discussed this medication at length, weighing the pros & cons as they pertain to Bethany. The biggest concern is that Kineret is being linked with causing MAS in some severe systemics, which has led to their deaths. This is not written into any literature at this point. Dr. K said that I won't find a black box warning that Kineret can cause MAS in systemics, but he

attended a national rheumatological convention & the doctors there discussed things and found the similarities. As such, it would be a very real risk to give Bethany that medication.

We are in a very bad place with Bethany. We can't do nothing, for obvious reasons. Actemra is not working well enough. That leaves Kineret or Enbrel (which is a biologic, comes with its own set of yucky side effects, and only treats arthritis, NOT the systemic symptoms). The FDA will be approving a drug to treat systemic-JIA in hopefully about 6 months ~ canikunamb (not a new drug, but new for this disease) ~ but the results coming in that were shared at the national convention show this medication isn't having any better results at controlling both the systemic & arthritis symptoms of s-JIA. Also, the side effects are worse than Actemra. Dr. K told me about this and said he was disappointed, as he (and the other rheumatologists around the nation) had been hoping this drug would be a really good one.

So, this is the plan as of today:

1. Start Bethany on an 8 week trial of Kineret. I will give her the daily shot. We'll watch her vigilantly for signs/symptoms of MAS and watch her labs carefully. If she develops MAS, life-flight to Portland, where she would be treated with HUGE doses of steroids and Etoposide (chemo) by hematology-oncology. Dr. K said that this medication comes with a significant risk, but after sitting silently, staring at Bethany & pondering the situation for several minutes, he confirmed that this is the route he would take if it was his daughter. As he said, we have to try SOMETHING.

2. Twice weekly infusions of Methylprednisolone ~ IV steroids. This is an attempt to knock down her arthritis to help bring it under control. It may not help at all, but it's worth trying.

3. Continue weekly methotrexate.

4. IF Kineret does not help after 2 months, we will switch her to Enbrel + anticipate flares of systemic symptoms, which would be treated with high-dose oral steroids. This would not be a good long-term option, as continuous steroid use would cause many problems within Bethany's body.

5. Give narcotics around the clock, every 6 hours. Dr. T, Joshua's palliative care doctor, will oversee Bethany's pain management program & direct Dr. S as to dosages & medications. Dr. K wrote a script for a month of tylenol with codeine, saying that he knows it won't be strong enough, but it will at least take the edge off while Dr. T

& Dr. S get things figured out.

Oh, and as an aside, Dr. K was listening to Bethany's heart and was shocked/puzzled/concerned by the rhythm he heard, which he said he'd never heard in all his life. It was a dum... pause... dum-dum-dum (3 fast staccato beats in a row)... pause... dum... pause... dum-dum-dum. He had both Adam & me listen to it and then said that if Dr. S hears this same abnormal rhythm on Friday, he highly recommends we get an EKG & have the strip faxed up to Bethie's cardiologist. Because... you know... this child needs one more thing to go wrong in her little body, especially an organ that I thought was finally repaired. *sigh* Hopefully, it was a random thing & she'll sound fine at Friday's appointment.


I haven't said anything here lately, not because nothing has been going on with Bethany, but because I suspect what I would write would incense anyone who has a modicum of compassion and I haven't wanted to raise anyone's blood pressure by sharing what's happening here.

I've debated whether I should write anything before I take Bethany to Portland to see her rheumatologist this upcoming Wednesday or if I should wait until after her appointment, but have decided I really would appreciate knowing people are praying for the situation because truly, I need God to intervene, to move on Dr. K's heart, and to make something happen. It's just not going to happen otherwise.

So, okay, enough cryptic stuff. What's going on, right? Well, it's late (12:47am), so instead of trying to write everything out and explain it, I will share a link that will speak for itself. This is a link to a blog that I started many months ago so that Dr. K could see what we were seeing with Bethany. He found it helpful when she was having flares but wasn't scheduled to come up to see him any time soon.

http://complexbethany.blogspot.com/

Read the most-recent post. Watch the videos. Then understand that Dr. K is ADAMANTLY opposed to giving narcotic pain medication to a child. He doesn't care that she could always be weaned off of it at a later date if it became unnecessary. I brought up the idea of pain medication in June & got shot down. This time, I used videos to make a compelling argument that narcotic pain is helpful for Bethany. Dr. S,

the pediatrician, saw the videos on Friday, November 30th when I had Bethany in the office for her pre-Actemra check-up. She agreed that pain medication would be appropriate for Bethany on a daily basis, but would not prescribe anything, saying instead that she wanted to defer to Dr. K on the matter. She asked me to send the videos to him and told me she would call him to discuss the situation. I sent Dr. K an email with the link to the blog at 9:38am that day (11/30), asking him to please take 5 minutes to read what I'd written & watch the 4 very short video clips. I specifically said that I would like to know his thoughts about this situation prior to bringing Bethany up for her next appointment with him on December 12th. To date, I have not received a response of any sort.

This past Friday, December 7th, I had to go to the pediatrician's to pick up prescriptions for Joshua. While there, Dr. S spoke briefly with me about both Joshua & Bethany. I told her I had not yet heard from Dr. K and it had been a week since I emailed him the videos. She did not seem surprised to hear that and asked me what I thought about that. I answered that I suspected he felt backed into a corner because I know he is strongly against giving narcotics to children and she nodded and agreed with me, saying, "Yes, he is." She then told me, "Well, let's see what he says when you're up there."

In my opinion, since Dr. S is aware that Dr. K opposes narcotic pain medication but she agrees it is necessary and medically appropriate for Bethany (which is what she said to me), she could write the prescription and allow me to avoid having to fight Dr. K over it. That's what she did for Joshua, after all. She recognized that he was suffering with chronic, severe pain and she told me something along the lines of, "I can't do surgery, but I can help him not hurt." I wrote about it in a journal entry on Joshua's caringbridge page when that conversation took place, several years ago. She did not wait to see if Dr. W (Joshua's neurosurgeon & primary doctor in Portland) agreed with her decision. She didn't wait to get permission from Dr. W. She just wrote the prescription and got my baby boy out of pain. So why won't she do it for Bethany, you ask? I have no answer. I don't know. It is a question I ponder every single day.

So where does that leave us? Waiting for the appointment on Wednesday morning with Dr. K. Waiting to hear what he has to say. I am going to politely request both answers and action from him. If he has not taken 5 minutes to watch the videos and read what I wrote, I will show him the videos while I am there. I need to understand why he will not help Bethany by prescribing pain medication for her. She has been suffering since the middle of April. At that time, he increased her Actemra dose to 400mg. Nothing has been changed with regards to

her arthritis treatment since then, despite watching Bethany spiral downhill with regards to pain increasing & physical capabilities decreasing. At different times, Dr. K has brought up the idea of trying something new, but other than doing some joint injections in August to keep her from flaring before she had her heart surgery, nothing else has been implemented. That bone scan he suggested we do never happened (and not because I didn't want to do it). No one seems to be interested in determining WHY Bethany is in such pain every day. I will be asking Dr. K specifically to explain the reason for this. If he believes Bethany's pain is caused by her arthritis, then I want to know why he has not made any changes to her treatment in the past 8 months. It's not like she's getting any better on the regimen she's receiving! Yes, Actemra controls the systemic symptoms of her arthritis. She is not flaring. She is staying off steroids. That's wonderful. But Actemra doesn't do a thing to control her pain that, if caused by arthritis, should be addressed by her rheumatologist. If Dr. K does not believe the pain is caused by arthritis, then the next logical question would be, "So why has no one ordered any kind of test to determine what IS causing her such severe, chronic pain?" One would think it inhumane to let a small child suffer for two-thirds of a year. I have begun to wonder, if Bethany was neurotypical like Joshua was when he was first diagnosed with severe, chronic pain, would we be in this situation or would she have already been tested, diagnosed & then prescribed appropriate medication to alleviate her pain, just as what happened with my son? Joshua was younger than Bethany when he first began taking narcotic pain medication on a daily basis (he started with Tylenol with Codeine, then moved to Lortab and then to stronger medications when his condition substantially worsened) and at the time he began that medication, no one knew his condition was terminal, so the attitude was not, "Well, this won't have any long-term consequences." The attitude was, "We need to help this child to not hurt all day, every day. We need to do what we can to give him a decent quality of life." That is what I want for Bethany, too.

I realize I might sound unkind toward Bethany's doctors and I wish there was a way to explain what is happening and express my concerns without being uncharitable toward them. I am trying VERY hard to give them the benefit of the doubt, to think that MAYBE there is something I am missing here... maybe there is a really good reason we can't put Bethany on pain medication... but until one of her doctors takes the time to explain that really good reason to me, I am left drawing my own conclusions. I do not believe either Dr. K or Dr. S is uncaring. I don't believe they are bad doctors (actually, I believe they're both very good). I do question why neither one seems to be interested in getting Bethany out of pain when it is patently obvious she hurts so badly it interferes with her ability to function. A child who

spends her days trying to NOT move at all and vomits because the pain she experiences is so severe absolutely needs help. I can see it. My friends can see it. I am betting that anyone who watches the videos in that link I am sharing can see it. I don't understand why her doctors can't see it. Wait... to be more accurate, I don't understand how her pediatrician can see it but be comfortable doing nothing about it and I don't understand why her rheumatologist, assuming he took the time to watch the videos, would not immediately agree that something needs to be done. I am hoping that when I take Bethany to see him on Wednesday, he will walk into the room and be in complete agreement that yes, this is awful and yes, it has gone on long enough. If he thinks this is arthritis-related, I hope and pray he will have a definitive plan of action to alleviate my child's pain. If he does not think it's arthritis-related, I hope and pray he will have already spoken with the doctor that he believes Bethany needs to see in order to get treated appropriately and a plan of action will be ready to go.

This has gone on long enough. If I have to go higher up on the chain of command to get someone to help Bethany, I will do it because NO human being should be left to hurt the way she is when there are medications available to keep them comfortable. Heck, everyone says how awful Ukrainian orphanages are ~ and I'm not arguing it; they ARE terrible ~ but at least they drugged Bethany and kept her pain-free while she lived there. The fact that no one here in America will help her achieve a pain-free status when it is completely possible to do so makes me sick. My family & I are doing what we can, but without the help of a physician, what we can do is very limited and does not give Bethany any real measure of relief.

So... please pray for Dr. K to be humane. For him to be caring. For him to do the right thing for Bethany. She NEEDS someone besides her family to want to help her. I really don't think I can express just how desperate we are to find an end to the daily, never-ending pain that Bethany endures and to help her feel better.


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Written Nov 22, 2012 3:18pm

We've had a few crazy days with Bethany. Last Friday, Bethany had her port accessed for her Actemra infusion, which was a nonevent. On Sunday, a red circle had developed over her port. It was identical to the red area that formed a couple months ago & landed Bethany in the hospital for IV antibiotics. I drew a circle around the reddened area and went on with our day. On Monday, Charley & I took Bethany to her cardiologist appointment, which went well. She's got mild pulmonary hypertension again (it had been gone after surgery) & some leakage of her mitral valve, but overall she looks good & doesn't have to go back for 6 months, which is great. After the cardiologist appointment was over, we saw a pediatrician (not Dr. S since she does not work on Monday's) & that led to a trip to the hospital to get labs drawn & then we were told Bethany would need to be admitted for IV antibiotics & I was advised to cancel Bethany's appointments in Portland, so I called Dr. K & canceled the appointment with him but I didn't call the physiatrist because I didn't have his number programmed in my cell phone. THEN the ped called Dr. S to ask her what antibiotic to put Bethany on & Dr. S said we did NOT have to admit Bethie, but could give her oral antibiotics, instead, and to NOT cancel the appointment with Dr. K because she wanted him to look at the infected area. SOOOOOO... I called Dr. K's office a second time and asked if I could get Bethany's appointment slot again & was glad I had not canceled with physiatry yet. Then we went to get Bethany's script & found that the pharmacy couldn't get it in until Tuesday afternoon, but I had to leave for Portland on Tuesday morning, so I had to call the ped's office & get them to fax a script to a different pharmacy so we could pick the meds up right away. Meanwhile, there was a big storm coming in & driving north was supposed to be filled with lots of rain & wind, so I was not looking forward to the next day's drive at all. The bottom line for Bethany as of Monday afternoon was that she had a cellulitis/possible port infection again & would be on oral Clindamycin for a few weeks and we were told if oral meds didn't control the infection or if the cellulitis spread, she would need to be admitted for IV meds. I was instructed to report if she developed a fever, but other than that, we'd go on with life.

Tuesday came with high wind gusts throughout the Willamette Valley, LOTS of rain & flooding, so Charley called Dr. K and Dr. J to cancel Bethany's appointments because while the weather was probably okay for people with normal vision to drive in, I have some vision loss in my left eye (it's why I try to not drive on unlit streets at night ~ I can't see well enough), and with trucks throwing up water spray and rain coming down, my vision goes to pretty much nothing & I can't see anything at all in front of me, which is scary as heck when I'm going 65mph down an interstate. It would not have been safe for me to attempt to drive to Portland, so the appointments got canceled. Charley is going to get

December 12th off work so he can come to the next appointment and that way, it won't matter what the weather does because he can drive in anything. If he could have gotten Wednesday off, he would have driven Bethany & me to the appointments, but with Thursday being Thanksgiving, there was no way he could get a day off, so we just rescheduled.

Tuesday afternoon, Bethany developed a fever, so I emailed Dr. S's nurse to let her know. We engaged in a few phone conversations, with her asking questions & relaying my answers to Dr. S, and at 5:45pm, she called to tell me I had to bring Bethany to the hospital to get admitted. Her concern was the possibility of sepsis if Bethany had an infection that got into her bloodstream. I asked if we could finish dinner since we'd just sat down to eat and was told, "No, you need to bring her in now." Megan packed a bag while I got Bethany ready and then we headed out to the local hospital. I had to drive on the freeway in the rain in the dark, but God gave me eyes to see & I really do have the stretch of road between our house & the hospital pretty much memorized since I drive it so often (our ped's office is across the street from the hospital), so that helped.

Bethany had blood drawn from her port & a peripheral vein to culture, labs were drawn, an IV was placed in her head & IV Clindamycin was started. The ped came in to talk and look at the red area, which had not grown in size from the day before. I felt like it was total overkill to have her in the hospital since we'd done this just a couple months ago and no one even knew if this was a true infection or what, but I understood the doctor wanting to be safe versus sorry since Bethany's immune status leaves her more susceptible to getting seriously ill very quickly. I stayed until 10pm, then drove home. Megan stayed with Bethany at the hospital since I need to be with Joshua at night. I asked her to call me with the lab results once they came in, and when Megs called at 11:30pm and I heard that Bethany's lab values were essentially the same as they'd been the day before, I felt confident that this was not anything serious and I fully anticipated getting Bethany discharged the next morning.

On Wednesday morning, as I was driving to the hospital, Megan called while Dr. S was in Bethany's room so that Dr. S could talk to me. She agreed to discharge Bethany because B was not becoming septic and there was nothing being done at the hospital that we couldn't do at home. Megan had told Dr. S that with Joshua not doing well, she would REALLY like to be at home, & Dr. S told me she agreed completely with that. We agreed that Bethany would continue on oral Clindamycin & topical steroid cream at home and Dr. S took a picture of the red area & sent it up to Dr. K for him to see. Dr. S told me that as long as

Bethany doesn't get very ill, we can keep her at home to treat this. I thanked her, hung up, continued driving to the hospital and picked up my girls, then came home.

Today, the red area is a bit smaller and Bethany has no fever. I personally am not convinced she has an infection of any sort since her labs don't support that conclusion, but I don't know why accessing her port occasionally causes the red circle to appear, so I can't offer an alternative diagnosis. It doesn't happen every time we access her, so there's no rhyme or reason to it. I'm just relieved that it isn't serious and that we don't have to keep her in the hospital. Life is much easier to manage when it does not include a hospitalized child in the mix.


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Written Nov 21, 2012 12:55am

A lot has been going on, but I only have a few minutes right now, so I'll give the most important piece of information and try to get back here tomorrow to write a full update. Bethany is in the hospital with a fever & red area over her port again. IV antibiotics were started tonight (she began oral antibiotics yesterday but with the development of a fever, had to be admitted). At this time, I don't know if she has a port infection or cellulitis or what. No one knows exactly what we're dealing with yet.

The trip to Portland was canceled, so I won't have the opportunity to talk to her rheumatologist face-to-face for another 3 weeks. That's disappointing, but God's timing is perfect, so we'll get up there when it's meant to be.

Sorry for dropping a tidbit without full details, but like I said, I don't have the time to write a lot right now. I will try to get a full update written tomorrow if circumstances with Bethany & Joshua permit.

Written Nov 14, 2012 11:52pm

Yesterday, Bethany turned 5 years old. It's hard to believe my little girl is technically a school-aged kiddo now. It makes me incredibly, can't-even-put-it-into-words sad that she has regressed in her development and is now

incapable of doing the things she was doing at the beginning of the year. With circumstances in my family being the way they are, I am not able to run Bethany to hither & yon, looking for a doctor to help her, but I have decided that when I take her to see the rheumatologist next week, I am going to talk seriously with Dr. K about what we can do to get Bethany's pain under control. The way things are going are completely unacceptable and *something* has to change. At this point in time, my little girl has almost zero quality of life and there is precious little that we can do to alleviate her misery. Nothing we try gives her relief and it's both heartbreaking and frustrating.

I want pain medication prescribed for Bethany. Dr. K says my daughter's arthritis is severe and he tells me the Actemra is controlling her systemic symptoms, but not the actual arthritis portion of her disease. When I suggested putting Bethie on narcotics to eliminate her pain, the rheumatologist said he understood my reasoning, but he couldn't support my idea because if Bethany was still suffering, we needed to identify why & find a solution for the problem rather than masking it. That made sense to me, but unfortunately, absolutely nothing different has been tried for months, so it seems that either the doctors don't believe what I am telling them about Bethany & what they see for themselves every time she is in their presence or they just don't have any idea what to do. So, since nothing we have been doing for the past 7 months has helped Bethie feel better and the only other medication option we have at this time is Kineret, which is a daily shot that is ranked a 10 out of 10 on the pain scale by teenagers who have to endure them (according to Dr. K) and the rheumatologist is not jazzed about putting Bethany on that med, I don't really see any logical reason why he should refuse to prescribe a medication that will make her not hurt anymore. Of course, that doesn't mean he'll do it, but I'm going to ask him to explain his reasoning if he denies my request. I'm truly tired of living like this with Bethany. She deserves better and to the best of my ability, I'm going to try to get her what she needs. I may not be able to go full "mama bear" on the medical establishment right this minute and honestly, I hope I won't have to, but I am determined to do what I can. Waiting isn't doing anything for my sweet Beppy (how Joshua pronounces her name now, which I think is adorable) and if doctors can't get her arthritis under control, they ought to be willing to make her comfortable. I am 100% certain none of them would make *their* child endure chronic pain.

What my family & I want is for Bethany to be HAPPY. If she never learns to eat, play, walk or talk, we won't mind as long as she is happy. If we can't ever get her out of her autistic world, it's okay if she is happy there. If all of her interactions with us have to be on her terms for the rest of her life, we're okay with it if she is happy. We want our little girl back. We want the Bethie

who smiles when we approach her, who claps back when we clap at her, who waves & babbles her own approximation of "hi hi hi". We want the Bethie who is able to show interest in the world around her and who likes being a part of our family. We brought that Bethany home from Ukraine in mid-October, 2010. We lost her for the first year after her arthritis diagnosis while she was on steroids & miserable. We got her back in mid-February, 2012 after beginning Actemra and after just two short months, we lost her again. So now I'm on a mission to get "Happy Beppy" back. We know she's in there and when she isn't overwhelmed with hurting, we get to enjoy the sweet little girl who we love so very much. Now I just have to convince her rheumatologist to give prescription pain medication a try; either that, or he needs to cough up a new idea and actually implement it. The "wait & see" attitude that I've been indulging from Bethany's specialists can't fly anymore. It isn't fair to my daughter and I need to make them understand that she IS capable of so much more than what they think.

Wish me luck. Say a prayer. Join me in hoping for a change for the better for Bethany. She deserves to not hurt anymore & I am desperately hoping I can finally get her doctor to agree with me about that.


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Written Oct 31, 2012 6:17pm

Bethany is sick. She has a virus that the other kids have had, bronchitis, thrush (7 weeks of antibiotics finally got the best of her despite our giving probiotics, as well) & fluid behind one eardrum. She's back on Augmentin & Nystatin and we're giving her 1 ounce of fluid every 30 minutes in an attempt to keep her out of the hospital. Her pediatrician was ready to admit her today, but agreed to let us try to keep her hydrated enough to stay out since we've got Halloween tonight & we're doing a combination 21st birthday (Megan) + Thanksgiving + mini-Christmas celebration on Sunday, so we REALLY don't want anyone in the hospital & missing out. Since Bethany has lost 2.2 pounds since October 2nd & is throwing up even the small 4 ounce feeds we've been trying to give her, we're now doing the ultra-tiny 1 ounce every 30 minutes, instead. I asked for a feeding pump to be ordered for her so I can hook her up to a machine and program it to give the formula (or pedialyte) super-slowly all day long. Poor kid feels like garbage, even worse than usual, which is quite sad to see.

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She gets Actemra this Friday, but aside from controlling her swelling & fevers, it isn't touching her actual arthritis, so while I'm glad she gets it & is able to stay off steroids, I'm not thrilled with this medication overall. I'd rather try something else and see if we can get her arthritis to stop causing her so much pain in addition to controlling her systemic symptoms. Of course, changing meds will require a trip to Portland and the past two appointments have needed to be cancelled due to illness (she was supposed to see Dr. K today), so who knows when we'll be able to get something different started. Also, I don't know if Dr. K will still want to change things up. I'm hoping he will, though. It'd be nice to try SOMETHING to see if we can change things for the better.

I need to go finish kids' costumes, so I apologize for the drive-by posting. :) We generally don't "do" Halloween, but Joshua was excited by the idea of going around to get some candy and we're all about making memories with him these days, so this year, we're participating. Bethany will stay home and go to bed at her usual time since she's too sick to go out.


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So many times over this last week, I've opened up Bethany's journal, intending to update on how she was doing, and each time, words have eluded me. What could I say that I haven't said a dozen times already? The phrase "same sh*t, different day" has come to mind multiple times, but I doubted that would suffice as a journal entry.

Really, though, I don't know what to say. Bethany finished her 7th week of antibiotics and the redness over her port is finally almost gone. The incision is finally healed, too. She had her Actemra infusion this past Friday and that went smoothly. She's had some periods of wanting to be held & cuddled and she's been more interactive with our family again. That's all the good news.

On the flip side, she's still got awful joint pain, especially her back. She shrieks & wails & screams in pain when we have to move her in the morning or when we get her dressed. She is vomiting even more than usual & we still have no understanding of why or how to stop it. It's not an allergy to her formula, nor it is caused by being fed too fast. Her labs don't indicate massive inflammation or anything else that would cause throwing up. It's just a

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mystery. Still.

Six months of unsolved symptoms & unresolved joint pain has worn my family down. I've got doctors thinking I'm a hysterical mother. I've stretched the limits of my doctor-parent relationships with several specialists, a couple who I have spent years building a relationship of trust & mutual respect. No one knows what else to look for. *I* don't know what else to look for. I was told that I can get a referral to any specialist I want Bethany to see, but I don't have any real idea who would be appropriate to take her to and, truth be told, our family's circumstances right now are such that I can't go up to Portland over & over & over, taking Bethany to see doctor after doctor for who knows what. If I could take her to one doctor who would be able to diagnose her the day he saw her, I'd do it, but that isn't realistic in the slightest. So, for now, my family & I will continue taking care of Bethany to the best of our ability & we will continue getting her Actemra infusions & weekly labs & eventually she is either going to start getting better or she's going to get bad enough that whatever is causing her symptoms will become obvious & then one of her doctors will know what to do. Until then, no one has any new ideas and, as such, we're stuck in "wait & see" land, which is a lousy place to be.


Bethany is on day 5 of oral Clindamycin after having received 4 days of the same antibiotic via IV in the hospital. The red patch over her port is lighter in color, but is still hanging around, leading me to think that maybe the antibiotic we're using to treat this infection isn't the ideal one. After 9 days, you'd think the redness would be gone completely. Well, at least, that's what *I* would think. :) I guess if the infection blows up again after we stop the antibiotics in 2 more days, we'll know why.

She is throwing up more often than she's been. This is, what... 3 weeks of vomiting? 4? I have lost count. In the past few days, she has puked at least half of every feed. It doesn't matter how slowly we feed her, either. Yes, I'll be calling the ped's office tomorrow to let them know what's going on.

We've started her back with some gentle PT, which she absolutely HATES. She cries when anyone touches her and forces her (no matter how gently) to move her arms or legs. Still, it's important that she move because otherwise, her joints will get very stiff due to her arthritis. We think she's gotten into a vicious cycle of not moving because she hurts, which leads to her getting more stiff, which then causes her more pain when she moves. Since she absolutely refuses to

move herself, preferring to sit or lay in one spot all day long, we are having multiple sessions per day where her shoulders, elbows, wrists, fingers, hips, knees, ankles, toes & back are very gently moved through their full range of motion. She cries through the entire process every single time, but the second she stops being touched, she stops wailing & sits, glaring, at us. Her behavior causes us to believe that she's not crying because she hurts, but because she is angry that we are touching her and making her do something she doesn't want to do. That is the joy of sensory processing disorder plus severe autism combining with arthritis. It's extremely unpleasant.

Something is wrong with Bethany, something not yet identified, and as a result of this, she is angry A LOT and because she is non-verbal, what we get is crying and furious screaming when things are not going the way she wants. We are making her spend time in her stander each day ~ it's a piece of equipment that keeps Bethany in an upright position so she is forced to bear weight on her legs without collapsing herself to keep it from happening (which is what she does if we try to stand her up anywhere else) ~ and she gets so mad she tries to pull her own hair and bite her hands, and she screams angrily the entire time she is standing up. We aren't hurting her by making her stand. It is an important part of her PT that she stand because weight-bearing will help offset osteoporosis (she's prone to that because of her steroid use) and aids in keeping the GI tract functioning properly (ie: helps with constipation, which is a common problem amongst people with Down syndrome due to their low muscle tone).

We see a behavioral component coming into play more now. Bethany has made the connection between throwing a fit & being left alone, so now, if she wants to be left alone (which is pretty much all the time), she makes herself as unpleasant to be around as possible. A behavioral therapist explained to Charley & me that when an autistic child is learning something, they will connect the very last action they did with the acquisition of what they wanted. So, for example, if a child wants a cookie & starts crying for it, if a parent gives in & hands the child a cookie, the autistic child will connect crying with getting the cookie and the next time they want a cookie, they'll start crying for it because that is what "worked" the last time they wanted one. If their crying doesn't get them the cookie, they'll KEEP crying & either the parent will give in and give them a cookie (which tells the kid that if they cry long enough, they'll get the cookie) or they'll wait out the kid & refuse to give the cookie & the kid will eventually stop crying, at which point the parent can give the cookie and then the child connects being quiet with getting what they want (and so the next time they want a cookie, they won't think they have to cry to get it because that's how the autistic mind works). Soooo.... Bethany has learned that if she cries,

people will stop touching her, which is exactly what she wants, so she begins crying the second she is touched & stops the second a person's hand is withdrawn. Unfortunately, we can't NOT touch her throughout the day ~ even if we wanted to give in to her demand (which we don't, as that isn't exactly a healthy desire for any child to have), we'd have to touch her to change her diaper & feed her ~ and we are weary of tolerating her fit-throwing just because someone dared to touch her, so we are now working on modifying her behavior, which is a tough job with a child who is severely autistic and has Down syndrome. We move her limbs and play pat-a-cake and stroke her hair despite her growls & screams & attempts to slap or bite us. We tell her that she won't get what she wants by screaming and then we don't give in. That means that if she is having a fit because we are holding her, we keep holding her while calmly telling her over & over that as soon as she sits quietly, we will put her down. Once she quiets herself, we praise her ~ "Good sitting quietly, Bethie!" ~ and then we set her down and put a toy near her. This is behavior modification & it works, but for a child with severe cognitive impairment, for whom learning is very slow, in addition to autism, convincing her to let go of her inappropriate behaviors in favor of new, appropriate, ones is going to be a long process. Fortunately (or unfortunately, if you're looking at this from Bethany's perspective), we have an 11 year old who was severely autistic (he's now "mild-to-moderate" on the spectrum), so we've learned how to implement behavior modification techniques and the benefits of using them, so we know there is a light at the end of the yucky-behavior tunnel. It's going to take a long time, though. Bethany has a degree of stubbornness that is unlike anything I've experienced with any of my other kids. People joke that the stubborn gene is located on the 21st chromosome and kids with Down syndrome have that gene in triplicate (trisomy 21 ~ Down syndrome ~ is caused by having 3 copies of the 21st chromosome). I'm vouching that yep, it's very, very true!

I'm unsure what direction things are heading for Bethany with regards to her health concerns. Actemra helps insofar as keeping her from having a major arthritis flare, but it doesn't seem to be doing much for lessening the pain and stiffness in her joints. I don't know if or when her rheumatologist might want to change medications, but I anticipate bringing that up for discussion at Bethany's next appointment with him. She still needs to have her spinal cord detethered & she needs to get her adenoids out & tubes put into her ears. I've been hesitant to schedule any of those procedures yet, though, since she had such trouble healing after her heart surgery. Her port is *still* not fully healed and it's been 6.5 weeks since it was placed! I'm thinking I'll wait to see the rheumatologist and ask him if he thinks it's safe to start making appointments for the other surgeries Bethany needs. I'd like to get all of those done.

I'd like to be able to write that Bethany is happy & doing well, but sadly, that is not yet the reality. I suppose I should be optimistic and say I believe she'll get there, but in all honesty, I can't even imagine a day when Bethany will be smiling & interactive in the way she used to be. Megan & I were looking at pictures from the first two months after Bethany joined our family ~ those two months before she got so incredibly sick & wound up in the hospital for 6 of the next 8 weeks ~ and we were both so sad as we saw the differences in Bethany between then & now. If there was any way to get her back to the little girl she was before January, 2011, I'd do it. Unfortunately, I don't know if it's possible because we can't seem to get this stinking s-JIA completely under control & the light that used to be in her pretty brown eyes is gone. It's heartbreaking. We want so much more for her than the way things currently are.


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Written Oct 10, 2012 1:30am

Bethany was discharged from the hospital today. The redness over her port has decreased a little bit ~ enough to make her pediatrician comfortable with sending her home on oral Clindamycin ~ and that made everyone in my family happy to get our little girl home again.

She is not feeling well, is still vomiting some of her feeds, and is basically the same as she's been for so long, but at least the infection is clearing up. Dr. S said we need to be happy for the little things that we CAN fix right now, so there ya have it. Something to be happy about.

It was a hard day today, with reminders that no one knows what is wrong with Bethany or where to go from here. I have a plan for what I can (and will) do if things have not improved or been diagnosed by the time Joshua dies, but there really is no way I can implement my plan until the time comes that I can be gone from my house for an extended period of time and that isn't going to happen as long as Joshua is alive. There are no words to describe what it feels like to know you can't take proper care of one child because the other is at the end stage of his terminal illness, so you can't be away from him (nor do you want to

be). It's easily one of the most difficult situations I've encountered in my life. Eventually, Bethany will get answers for her symptoms. For now, however, we have to wait. Such is the reality of life sometimes. I just keep reminding myself that God has a plan, He is in control, and nothing that is happening on a daily basis is a surprise to Him. I also keep asking Him if maybe He'd like to let me in on a detail here or there so everything doesn't keep smacking me upside the head. Happy surprises, I like. The kind I've been getting as of late, though, with Bethany's wound dehiscence & a port infection? Not so much. I know it's not a picnic for her, either. People keep saying Bethie deserves to catch a break. Here's hoping God is listening & agrees!


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So maybe Bethany isn't responding to the antibiotic as well as we'd thought yesterday. Today, the redness hasn't changed at all. It is still filling the inked outline that I showed in the picture 3 days ago.

She has thrown up repeatedly today.

She has not moved in her crib. Literally. She has sat in one spot except for when she was picked up and held, which she tolerated for only a few minutes at a time.

The Actemra has "worn off", meaning she is growing increasingly irritable again.

Her day nurse had concerns that I am hoping she passes on to the doctors. I asked her to, but don't know if she made a chart note or not.

I requested that labs be drawn to evaluate for infection since we still don't know exactly what that redness is ~ the assumption is infection, but Bethany's labs on Friday didn't support the diagnosis of an infection and her blood culture hasn't grown out anything. The nurse called the doctor (not a pediatrician we know ~ I met him for the first time yesterday) and he ordered the labs, but said to do them tomorrow morning rather than today because he didn't want to have to try to

interpret the results. He said it would be easier to have Dr. S look at the results since she knows what Bethany's previous labs have looked like. I dubbed that doctor "Dr. Lazy" because ALL of Bethany's previous labs are available to that guy ~ all he has to do is look on the computer! Talk about ridiculous!!! Anyway, she'll get the labs drawn in the morning & I'll see if anything specific shows up. I'm hoping for SOMETHING to be obvious because I was told Bethany can't get discharged until the redness recedes and at the rate we're going, with nothing changing, it's looking like she could be inpatient for awhile if they don't figure out what that redness IS and what is causing it and what they want to do about it. If it weren't for the fact that I would have to figure out what to do with Joshua (which would be uber-challenging), I would almost wish they'd transfer Bethany up to Portland because at least there, Infectious Disease and other specialists could see her and they might be able to get to the bottom of the mystery that is my daughter.


Bethany is responding well to the antibiotic. The redness that had developed yesterday has decreased and is now back to where it was on Thursday night.

Other than that, nothing much has changed. She vomited her first feed of the day, has had a low-grade fever, is okay as long as she's not being messed with. So, in other words, she's at status quo.

I wanted to address something that keeps coming up in the guestbook comments. Several people have suggested I take Bethany to Mayo Clinic or St. Jude's or one of the other elite treatment centers in the US. That's a nice idea, but an impossible one. Aside from the fact that my family cannot afford to take Bethany across the country for an indeterminate amount of time, I can't be away from Joshua for an extended period of time since he is at what his physicians consider the end-stage of his condition. And contrary to what some may think, my being unable to travel with Bethany doesn't mean I love Joshua more than her or that I don't care about Bethany because she's adopted (both are accusations I've had leveled at me). It means that the circumstances of my family's life right now preclude our being able to travel outside our state for medical treatment. There are a LOT of families in the US who would like to be able to take their kids to one of the high-ranking medical facilities but are unable to. That in no way makes them bad parents or negligent or uncaring. It also means I don't suck for not taking Bethany.

Also, getting an unbiased second opinion is not always as easy as

calling a doctor and making an appointment. Oftentimes, you can't get an appointment without a doctor's referral, which is also not always easy to obtain, but even when you CAN get your doctor to agree to send you someplace else, that doesn't mean you're necessarily going to get an unbiased opinion. When you ask your doctor to refer your child to another doctor & they send a referral, they have the opportunity to say anything they want. If your doctor is giving the referral to appease you, then guess what? She can write something to the effect of, "Mother is concerned about XYZ. I have explained that her child's symptoms/labs/test results (whatever) are not indicative of XYZ. Please evaluate." What that means is "This mom is worried for no reason. I told her as much but she doesn't believe me. Please back me up. Thanks." If you think I'm nuts for believing that kind of thing happens, that's fine. I know what I know. Bottom line is, I can't just waltz into a heme-onc's office or call up a cancer treatment hospital and say, "Hey, something is wrong with my kid and I want to know if you'll do a bone marrow biopsy to check for leukemia or some other disorder of her bone marrow." It just doesn't work that way, though it's nice to imagine that it could.

I am doing what I can for Bethany. I spend hours every week on the phone & in email conversation with doctors and I don't think anyone outside my family & close circle of friends truly understands how anguished I am at not being able to get answers for Bethie and how much it eats me up inside that I have a child who is having medical problems that I can't get figured out. It took me 3 years of working with the medical establishment (and sometimes fighting against it) to get answers for Joshua & I will do what it takes to help Bethany, as well. Please don't assume that I'm not doing what I am physically capable of doing for my daughter. It's easy to think you'd do things so much better or that you'd "just" do X or Y or Z if it were YOUR child in the same situation as Bethany, but honestly, unless you also have 2 chronically-ill, medically-fragile kids, 1 who is dying, plus 6 other kids who need your attention every day, you really can't say with any authority what you would do in an identical situation.

*big sigh*

Anyway, if what I've written offends you, I'm sorry, as that is not my intention. I'm just pretty much at the end of my rope as far as tolerating snarky, insinuating or outright-nasty comments.


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The red spot over Bethany's port has increased by an inch in all directions. Dr. S has started her on IV Clindamycin.

Her kidney ultrasound showed a mild dilatation of the portion of the kidney that collects urine. Dr. S said it's nothing to be worried about.

Her pelvic x-ray showed a mild hip dysplasia on the right side, which is weird since her previous hip x-rays did not mention a dysplasia. Again, I was told it's nothing to worry about because we can't do anything about it.

Her spine x-rays were read as normal, but my friend (who is a chiropractor & very familiar with reading x-rays) looks at the films & immediately pointed out to the nurse who was showing us the x-ray a suspicious area on Bethany's L4 vertebra. I mentioned it to Dr. S and she said that she wasn't going to overrule a radiologist. I looked her square in the eye and reminded her that the radiologist had told us Joshua had osteosarcoma and obviously they were wrong about THAT, so why could she not believe they might have overlooked something on Bethany's film? She told me that it was a different radiologist this time. I just stared at her, so she added that this is why she is sending the films up to Portland for the pediatric radiologists to take a look. That satisfied me and I told her so. It may be nothing (the L4 looking different than her other vertebrae), but if it's something, I don't want it being overlooked.

Her labs looked okay in some areas & bad in other areas ~ typical. Her ferritin & LDH have jumped up quite a bit since Tuesday, but her inflammation markers are really low, so we're sure we're not looking at an arthritis problem.

It's good that she didn't show up with a bony tumor or a bone infection or kidney stones or any kind of mass there. Those are things we can check off the list of possible causes for her pain. It doesn't tell us what IS the problem, though, so I will continue pushing to get that figured out.


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Both Bethany & Joshua are admitted to our local hospital. Bethany is having x-rays of her back & sacroiliac joint (right side), IV antibiotics for the cellulitis over her port (the red spot), an ultrasound of her kidneys to check for hydronephrosis and/or stones as the cause of her pain, and her Actemra infusion. Joshua is getting fluids & pain meds & anti-nausea meds. He's repeatedly desatting to 72-75% and Dr. S said that if this progresses to where it appears to be his brain stem versus a viral illness, we will have to have a family consult to discuss how to manage him (ie: do we "overcomfort" with medication or not). I am stressed and scared and really wishing this weren't happening, but praying that Joshua will recover and start feeling better soon AND that Bethany's tests will give us some definitive answers.


This red area appeared over Bethany's port tonight. I don't know for sure what it is, but I think it might be a sign of infection, which would be odd since she is still on antibiotics. I outlined it so we can assess in the morning if it's changed in size (larger or smaller) and when I flip the picture upside down, it looks like her port incision (which is nicely scabbed over now) is having a thought bubble like a cartoon.

I wonder if it's saying, "Let's see the doctors pretend they don't see THIS!"

Maybe it's saying, "I won't be ignored!"

Or maybe it's saying, "Really? How sick does this kid have to get before someone is going to do something about it?"

http://www.caringbridge.org/tres/images/photos/2/5/6/9/256912/l.CNjcLWdbLCntrPIX.jpg

In all seriousness, Bethany is supposed to have Actemra tomorrow, so she'll see Dr. S before the infusion & we'll find out what is going on. If it's an infection, the question will be whether we get to stay at home and treat it locally or if they will want her in Portland. I know that removal of ports is not always necessary with an infection, but I suspect that if this is, in fact, an infection, the consensus is going to be that she's already had too many problems with this central line & they'll want to pull it. Again, we'll see.

There should be a song with lyrics that go "If it's not one thing, it's another." That song would be on a continual loop around here!

And though some might scoff at this, I will admit that I am hoping God is doing something and the red spot showing up out of the blue tonight is a sign that He is going to move mountains to get Bethany the care she needs & deserves. Last time I'll say it for tonight ~ we'll see!


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Heme-onc is refusing to do the bone marrow biopsy. I received an email from Bethany's rheumatologist saying he spoke with the heme-onc and she said she could not make a case for the procedure based upon current clinical information. He said I could pursue options in my local community or maybe schedule an appointment with the heme-onc.

What the heck????

Two doctors tell me verbally that they wonder if Bethany has a malignancy & that they think it's time to re-do a bone marrow biopsy. One doctor tells me that we should do it if for no other reason than to give us peace of mind. He even went so far as to tell me that he would do the biopsy himself if he could. The other doctor retracted her verbal statement about wondering about a malignancy, but wrote that while she doesn't think Bethany has a malignancy, it's something that doctors wonder about when they have a child who has a lot of odd

things going on. Then heme-onc says no and we're back where we started.

I wrote back to Dr. K an email saying that since Dr. G is certain there is nothing wrong with Bethany's bone marrow, I guess we can just check all disorders of the bone marrow of the list. Then I asked if the x-rays he's ordered don't show anything, will we still be doing a bone scan, and if we do and that doesn't show anything, what will be the next step? Jack her up on more Actemra or switching medications and hope for the best?

Dr. G better pray to GOD that Bethany does not actually have leukemia or ANY condition that would cause abnormal bone marrow & bone pain because if she does & we eventually get it diagnosed, I WILL pursue a lawsuit against her and every other doctor who is dropping the ball and refusing to provide appropriate care for my child.

I have been patient. I have tried to follow the proper chain of command in working with doctors. I have tried talking to doctors about Bethany's symptoms in an attempt to get someone to want to help figure out what is going on with her. This is not a matter of me being a parent who is looking for a problem that doesn't exist. Every doctor who examines Bethany & watches her wince & grimace in pain with movement and sees her misery agrees that yes, there is definitely something wrong. Then they leave it there. Dr. S tried at first to help figure out what was going on (back in May), but after the path she pursued led back to a rheumatology answer, she decided she wasn't going to do anything more because she didn't want to go down "blind alleys". She told me she could do more tests, but they'd just show abnormalities and then we'd get worried but it would all just be part of her s-JIA, so it was better to not do any tests and to let Dr. K lead us. So I looked to the rheumatologist, who, to his credit, DOES care about Bethany (at least he gives the impression that he does, which is a lot more than some other doctors do), but he doesn't have any answers, either. He's honest in admitting that, so I am not angry with him. I respect a doctor who can say, "Dang, I don't know! This kid is complicated," and is still willing to look into trying to find answers. I had THOUGHT we were finally going to get moving in that direction after Dr. K called me last Sunday and am now frustrated that nope, Dr. G doesn't want to do anything.

I'm amazed that the heme-onc can definitively decide Bethany has nothing abnormal with her bone marrow from just a phone conversation with Dr. K & nothing else. Impressive, eh? Like I said before, she'd better hope she's right. 100% correct. I'm not a sue-

happy person, but honestly, this strikes me as completely negligent. Maybe Dr. G is correct and a bone marrow biopsy would be barking up the wrong tree, but it's not an unreasonable test to request in light of what is going on with Bethany.

I feel like I am slowly going crazy. Why is it so dang difficult to get doctors to want to DO something ~ think about a kid's symptoms and what might be causing them, then order a test or two in an attempt to get a definitive diagnosis so the kid can be treated & then feel better? I'm so sorry this isn't a straightforward, clear-cut, easy thing! It's not like Bethany asked for this to happen to her and I certainly wasn't looking for it, either! If Dr. K was telling me that he thought this was all related to Bethany's arthritis, I could accept it (so long as we were treating the symptoms to keep Bethany comfortable), but that isn't what he is saying, so I can't understand why the ped is so reluctant to do anything that might lead to a diagnosis.

Is this a case of doctors not wanting to do something for a profoundly-disabled child? I understand she's complicated, but is that really a reason for doing NOTHING? Sometimes I wonder if their goal is to frustrate me to the point that I throw up my hands and scream, "Fine! I give up!" Sure would make their lives easier if I did, I'm sure.

I must be missing something because none of this makes sense to me.

Maybe I should pack up Joshua & Bethany and all of the email correspondence between myself & the doctors and go to the hospital and request a meeting with someone in hospital administration. Maybe I could get Bethany the help she needs if I took proof that doctors are willing to say there's something wrong but unwilling to do anything to determine what that "something" is to the head of the hospital and said someone is going to address my daughter's health needs in an appropriate manner or I was not leaving. Or maybe I should just contact the media. I'm guessing a story about a major hospital that won't provide appropriate treatment for a profoundly disabled child whose brother is dying might tug on some heartstrings and push the hospital to apply some pressure to the doctors they employ. Problem is, I don't WANT to do either of those things! I don't want to burn bridges. I don't want to have to be a total raging beyotch. I just want a doctor to want to help my kid and to refuse to give up trying until s/he finds an answer!

I watched one mother raise major hell in order to get her daughter the care she needed the last time Joshua was inpatient (November, 2011). The child got admitted to PICU even though she didn't have any medical need to BE in PICU just so the hospital could guarantee the

child would have immediate care if she needed it. They were falling over themselves to take care of that kid because they knew her mother was ready to take legal action against the hospital if they were negligent ~ at one point, Joshua's nurse left in the middle of giving Joshua a dose of pain medication because the hospital administration had handed down orders that that particular child was NOT to be kept waiting for any reason & her nurse had buzzed for more help. At the time, I thought the mom was out of line and being unnecessarily demanding. Now, though, I get it. I don't think medical personnel think about the stress they inflict on parents when they dismiss our legitimate concerns or, worse, agree that yep, there's definitely something wrong, but don't take any steps to identify the cause and then treat the problem. Maybe that mom felt there was nothing else she could do other than raise hell and threaten a lawsuit in order to get her daughter help. Maybe that's the step I need to take, as well. It's not like doing things "the right way" is getting Bethany any closer to feeling better.

I guess I know what I'll be spending concentrated time talking to God about.


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I can't keep doing this fighting doctors thing. I got an email from our ped that basically contradicted everything she'd told me at yesterday's appointment. I had thought that she, Dr. K & I were finally on the same page regarding Bethany. Apparently not. And I know I need to care... and I DO... but I am so completely worn out from fighting these battles, especially with a doctor who I thought would be the last person I'd ever have to convince that I have an understanding of my child & what she needs. Nothing I try makes a difference for Bethany and I don't know what to do anymore.

God needs to make a way where there seems to be no way. That about sums it up.


Bethany had her port accessed and blood drawn to do a culture so we can evaluate whether she has a line infection. Blood was also drawn (off her port) for labs, and then we headed across the street to the pediatrician's office for an appointment.

Her labs look about the same, which is better than finding out they're a lot worse. Her disposition is the same ~ crying when touched or moved around, but quiet and sometimes even smiling if we interact without touching her. Dr. S examined Bethany and was relieved to see the port incision scabbed over (FINALLY!), her chest incision closed up, and her left toe starting to look better. We're going to keep her on Septra for another week just to ensure that toe keeps healing. We chatted about how miserable Bethany is and what might be causing that. Dr. S said she wondered if we should do another bone marrow biopsy and maybe a bone scan or a CT of her abdomen to evaluate for malignancy. She actually did not say the word "malignancy" since Adam, Sarah & Joshua were also in the room; instead, she wrote it on the paper that covers the exam table as she said, "If this isn't a rheumatological problem, it could very well be this," (writing "malignancy" as she said "this"). All I said in response was, "All right." Really, what else could I say? It's not like I'd be surprised if Bethie had leukemia. We've been hearing about her wonky bone marrow for almost two years now and I've been warned repeatedly, by several doctors, that her bone marrow could "turn" at any time. She's been taking medication that increases a person's risk of developing leukemia and she has a chromosome abnormality that makes her 15 to 20 times more likely to have leukemia during her lifetime. Oh, and I had three heme-oncs tell me in May/June that we could be partially treating leukemia but we wouldn't know until we got her off steroids long enough to let it 'show its face'. So, really, as awful as getting a diagnosis of leukemia for Bethany would be (and yeah, it would be devastating because with her complex medical issues, I don't know if she'd survive cancer), it wouldn't be surprising. It would just be really, really sad. Maybe we'll get lucky, though, and all of the testing we're going to do will show a condition that is easily treatable & not scary at all. I think it's only fair that Bethany should get something that falls into those categories for once.


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Written Sep 30, 2012 6:24pm

A few days ago, I emailed Dr. K & laid out my concerns, thoughts, impressions, etc with regards to Bethany & the lack of action being taken to figure out what is wrong with her. He emailed back, asking for my phone number so we could have a real conversation. He called a few hours ago (yep, on a Sunday, while he's on vacation). We had a *really* good discussion & the plan now is to get some x-rays of Bethany's back, hips & other joints to see if anything is awry there and to ask Dr. G (heme-onc) to do a bone marrow aspiration & biopsy to evaluate for malignancy/leukemia. If both of those come back with normal results, then Dr. K will order a full-body bone scan to evaluate for bone inflammation, bone lesions or anything else that could possibly explain what is causing Bethany's ongoing, chronic pain and whacked-out labs. If all of those tests reveal no answers, he said we will sit down to discuss where to go from there.

He wants Bethany to see physiatry (rehab) ~ her appointment is Nov. 21st ~ and thinks that if nothing else, they can help us determine what Bethany is physically capable of achieving and maybe she would qualify for their intensive inpatient rehab program, which is 6 weeks of daily therapy for many hours each day. We can't do that right now, but Dr. K believes that if we can get her underlying condition figured out & treated appropriately, putting Bethany in an intensive inpatient rehab program would help her to develop skills & become a lot more functional than she currently is (he said she is at less than a 12 month old level for development). It made me happy to know he's thinking long-term for Bethany & how to help her overall development.

I am *so* relieved he is on the same page as me & is ready/willing to order tests so we can start trying to get Bethany figured out. He agreed with my statement that even if the tests don't tell us what IS wrong, they'll help us check off things that AREN'T wrong and that, in & of itself, will narrow the field of possibilities and should help us in our quest to get to a diagnosis. It's amazing what a shot of HOPE can do for a person's spirits. I could cry with the relief I feel that finally, someone has agreed to do something to help Bethany. I feel like I've been pushing & prodding & bugging doctors for so long... to finally hear that not only does someone agree with me that we're missing something important (all of Bethany's doctors have said they know something is being missed), but they agree that yeah, we need to get this figured out, is a huge huge HUGE blessing.


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I didn't hear anything from the pediatrician today, so I guess that means nothing grew in Bethany's cultures (blood or wound). And I guess THAT means God answered our prayers because there was pus in her port wound, which should have meant an infection. I'm very relieved & happy that it doesn't look like an infection is responsible for causing Bethany's misery.

Of course, that leaves the question of what IS responsible, but at this point in time, the doctors aren't trying to figure that out. They're content to say, "Oh good, she doesn't have an infection!" and leave it at that, conveniently ignoring the fact that she is absolutely miserable and has been slowly getting worse since mid-April. By focusing on only 4 lab values that are related to her arthritis (that are normal), they can conveniently not think about what her elevated wbc, elevated ferritin, elevated LDH, big 'left shift' (18% bands, 72% segs ~ her bone marrow is kicking out a LOT of immature white blood cells, which typically occurs during serious infections or blood disorders like leukemia), toxic granulation and Dohle bodies might be pointing to.

I don't believe Bethany has bad doctors. I know they are actually very good, very intelligent, doctors. I do believe they are being myopic in their focus right now by looking only at the port wound/injured toe as the potential causes for the symptoms we are currently seeing. I'm not sure WHY this is happening and I'm not going to speculate on a public journal. Suffice to say it's disappointing and my prayers are now centered around asking God to clearly & definitively show what is wrong with Bethany so that we can begin treating her appropriately & help her to feel better.

Thank you for praying against the infection. It really is amazing to me that the green/yellow gunk disappeared so suddenly and her cultures have not grown out any bad bugs. Adding Septra to the Keflex made a difference, but I am convinced the prayers of so many did, as well, and I want you to know that I appreciate the time you spent talking to God on behalf of my little girl.


We have a 24 hour reprieve. Bethany's white count is down a little from yesterday (but Actemra suppresses that value, so we don't know if it's *really* lower or not). Dr. S cultured the gunk in Bethany's port wound, but after I had cleaned it out this morning, there really wasn't a lot there (which is good ~ maybe the Septra + Keflex are working). Dr. S said she is super worried and that I should keep a bag packed because if anything grows in her blood or wound cultures, Bethany will be airlifted to Portland, but for today, we get to stay home, and there is *some* hope that the double antibiotics will kick any infection to the curb (assuming her port wound isn't infected with anything more than a superficial bug) & we can avoid a trip north. Adam told her, "The power of prayer at work!" and Dr. S agreed with him and recommended keeping it up.

So... please keep praying! Thank you!


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The plan is to add oral Septra to cover potential MRSA infection and evaluate how Bethany is doing tomorrow. If her toe, chest incision or port incision look any worse or if she develops a fever or otherwise worsens, she will see Dr. S tomorrow, more labs will be drawn & she'll get admitted to the hospital for IV antibiotics.

Considering that I just took her temperature and she has a low-grade fever and green slime in her port incision and yellow slime in the little hole in her chest incision, I am guessing she'll be admitted tomorrow. Then the question is whether we'll be able to treat her locally or if she'll have to go to Portland. I'm hoping for local, but will pack a bag for Portland just in case.

And if you think I am feeling frayed and on edge, you pegged it perfectly.


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Quick update: Bethany continues to be miserable when interacted with. No more fevers. Her chest incision from her heart surgery has a tiny hole at the bottom that opened up overnight. I hope & pray it won't grow bigger. Her port incision has not opened up any wider and remains 3-4mm open with the steri-strips holding the ends of the incision somewhat together. I found out via her pediatrician that her ferritin levels shot up last week & stayed there this week. Her white cell count is very high. Other labs are off. The overall picture is concerning.

Dr. S and Dr. K (rheumatologist) are emailing back and forth to hash out a plan of action. Last I read, the idea of hospital admittance & IV antibiotics was tossed out. Dr. S is waiting to hear from the general surgeon and/or cardiologist to determine if they think the port should come out. I'm waiting to hear what I need to do and when, while still holding out hope that we can treat this on an outpatient basis. The idea of having to take Bethany to Portland and/or put her in the hospital right now when Joshua is also not doing well creates stress. I can't be in two places at the same time and I feel like I NEED to be with both of my fragile kids.

I know God's timing is perfect and I know His plans are good, but I honestly can't see it with my human eyes at all right now.


Last night, I decided the reason I hadn't heard from the pediatrician *must* have been because she did not truly understand the extent of the dehiscence (split). I emailed her, attaching the same photo I'm sharing here, to show her just how much more the wound had opened and to express my concern about possible infection & the fact that with the sutures being pulled into the incision, it was going to be extremely difficult to remove them if we waited. I suggested removing the stitches, then asking the vascular surgeon who had placed the sutures to re-stitch the incision closed, using a lot more stitches this time.

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The phone rang this morning & it was the weekend nurse passing a message from Dr. S, asking me to please check my email right away. The email was Dr. S saying she was on-call this weekend (thank you, God) and wanted to know a few more things about the port & wound and as soon as she heard back from me, she would call Dr. P, the surgeon who helped us last week when her incision first split open. I wrote back and about 40 minutes later, my phone rang again and this time it was Dr. S telling me that she'd spoken at length with Dr. P, who felt it was time he bows out of this situation since Bethany is not his patient, he doesn't typically work with children, and he really was not comfortable continuing to do anything with a wound that was experiencing healing problems. He said that if Bethany WERE his patient, he would remove the port because the risk of infection was just too high not to, but since the cardiothoracic surgeon had placed the port, it was his call to make on whether it got removed. Dr. S and I chatted about our options and ultimately agreed that I would bring Bethany in right away to get the sutures removed & steri-strips (with adhesive) placed to try and hold the incision partially together as a stop-gap solution for today.

Ten minutes later, I had Bethie loaded in the truck & we were heading south to the ped's office. By the time I had my girlie in the doctor's office, she was crying hard in obvious pain, but neither Dr. S nor I could figure out what was hurting her. It wasn't the incision. She grimaced and cried harder when Dr. S was touching her toe, which is now red and swollen (but not hot to the touch) on the top surface of her toe from the nail bed to over the knuckle. Dr. S saw Bethany yesterday before her Actemra infusion and remembered that Bethie's toe did NOT look red at all 24 hours previously. That made her quite concerned. She also cried when her upper right abdomen was pressed against, but her liver did not feel enlarged. Bethany continued to cry so hard she eventually made her nose bleed. Every time we had to touch her or move her position, it brought fresh wails followed by a torrent of tears & heaving of her chest as she tried to catch her breath. Unfortunately, Dr. Sibley & I had no choice but to touch Bethany while we worked together to get the stitches out. We had to remove the scabs to get to two of the sutures & those two had to have the skin pulled a bit in order to get the knots unstuck from the skin they'd gotten buried in. It was pretty awful. The stitch on the far left was pulled almost entirely into the incision. The two little black threads you can see sticking out in the picture (on the left) had almost completely disappeared, so Dr. S had to go from inside the wound to clip the suture & remove it. Dr. S applied 1/2" wide steri-strips and used adhesive to help them stick so she could apply tension to the strips. By doing this, she was able to get the wound edges about 2-3mm apart.

She wrote another prescription for Keflex (third one, so this will make 30 days on this antibiotic), as well.

The plan is to see how the wound and Bethany do over the next 48 hours. I will email Dr. S on Monday with a full report and we'll go from there. It is anticipated that I will have to take Bethany to Portland to have surgery to remove the port & get the wound stitched together from the inside-out, but we're hoping to get through the weekend first.

Bethany was okay in the truck on the way home (she fell asleep), but after being carried into the house, she was back to crying again if she was touched. She felt warm, so I took her temperature, which was 100.2 with tylenol on board. Whether she was being interacted with or left alone, she was obviously in pain and miserable, grimacing & taking shuddering breaths as she laid on her side & tried to not move a muscle. Eventually, after she'd been fed, I cradled her gently in my arms and carefully carried her to her room, where I laid her in bed. I hoped she would sleep, but it took her awhile to drift off.

I can't shake the feeling that something is *really* wrong. For her to have a fever the day after getting Actemra is unheard of around here. For her to be in such pain, especially the day after an infusion, doesn't typically happen, either. She is the most miserable I've seen her in months, which is saying something. She was pretty warm when I went in to check on her, but since she was asleep, I didn't want to wake her to see what her temperature was. If she wakes up tonight, I will check it, but if she can sleep, I want to let her.

I'm really worried about Bethany. I don't know if this is an infection or possible sepsis brewing or if the "something" that everyone has been saying (for the past 5 months) would eventually show its face is finally starting to do just that. All I do know is that she's a little girl who is hurting terribly and no one (including my family) has any idea why. My prayer at this time is that God would shine a light on the cause of Bethany's symptoms and guide the doctors to answers because we *need* to get this figured out and treated appropriately. Not in another week or month, but *now*.


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*big sigh*

Bethany's port incision has partially split open again despite the stitches attempting to hold it together. There are three tiny dots along the top edge of the wound where the stitches were, but have been pulled into the skin. Along the bottom of the incision, you can see the nylon ends of the stitches, but the knots are now buried under the skin & on one of the stitches, all you can see are two little pieces of nylon that look like bug antenna sticking out from the hole where the knot of that stitch has been buried under skin. There are 3 little scabs where the skin tore as the stitches were pulled (the one on the far right is almost torn into the incision). The tissue is trying to come apart, which strikes me as worrisome.

I had called our pediatrician from the hospital (Bethany was getting her Actemra infusion when the new split occurred, but the two events are not related) to let her know about the dehiscence & to ask if we should get the same surgeon who placed the stitches 8 days ago to come remove them and re-stitch the incision, using a lot more than 3 stitches. Unfortunately, the ped never called me back or came by the hospital to check out what was going on. I can't tell you how thrilled I am (NOT) to be going into the weekend with a child whose white count is already above 32,000 & who now has an open wound leading to her port catheter (which goes into a major blood vessel to her heart) and who we just gave a honkin' dose of immunosuppressive medication via infusion & a small dose of a different immunosuppressive medication via injection. As one nurse told me, if Bethany gets some bacteria that isn't killed off by Keflex (the broad-spectrum antibiotic she's getting twice a day), we will be facing a huge mess (sepsis, need for port to be removed, IV antibiotics, hospitalization on TOP of her poor healing problem). All I can do is pray that doesn't happen, but I honestly can't say I would be surprised if it does.


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I took Bethany to Portland today for follow-up after her heart surgery and to see her rheumatologist. Nine hours of driving and three hours at the hospital made for a long day.

Cardiology update: Cardiologist, cardiothoracic surgeon & P.A. all saw Bethany today. They were nervous about Bethany's exceptionally-slow healing. Said we need to wean her off the steroids over the next few days & wait another week to take the stitches out of her port incision, but if it opens again, we should get it stitched back up. She'll stay on antibiotics for the duration since it's keeping infection at bay thus far (I'm pretty sure the copious amount of prayer being offered up on her behalf is playing a part in the lack of infection, as well). Her echo showed only a little bit of fluid around her heart now, but in the space where there had been fluid, there's now thickening. I don't know the significance of that and when I asked, Dr. K & Dr. I sort of hemmed & hawed & launched into how they'd like to see her sooner than they usually do (in 2 months versus 6), so where would it be most convenient for me to bring her ~ up to Portland or to the outreach clinic when they come closer to our town (I chose outreach clinic, of course)? So I don't know what, if anything, the "thickening" is significant of/for, but I'm going to assume it's nothing because I don't want to have one more thing to be concerned about.

She has had multiple tiny spots open up in her chest incision & leak fluid. Her echo today left the cardiologist saying he *thinks* the fluid she's leaking is coming from the tissue under the incision versus fluid around her heart. There's nothing we can do about it except apply pressure gauze and wait for it to heal over. If there was a large section of her chest incision opened up, they would stitch it closed, but with the openings being just small holes, we'll apply pressure through dressings and wait.

Rheumatology update: Dr. K isn't sure if Actemra is playing a part in the ultra-slow healing, but we agreed that we can't stop giving her Actemra because if we held her infusions, her arthritis would flare spectacularly & then we would be forced to put her on high-dose steroids again PLUS give IV pulse steroids and that would DEFINITELY affect her healing in the negative direction. Until she's healed & has had her tethered cord surgery & adenoids removed & tubes put in her ears, Dr. K doesn't want to change what we're doing with Bethany with regards to her medication, but we discussed how we are heading in the direction that something will have to change because even with the mega-dose of Actemra that Bethany gets, she only gets 4-5 good days before it wears off and then she gets increasingly cranky & painful until her next infusion.

Her port incision: It's open above the stitches, but the stitches are holding the internal tissue together, which is what is most important. No one has any idea/guesstimate for how long it will take for either of Bethany's incisions to fully heal. This lack of proper healing is what everyone was concerned would happen when we had to do Bethany's surgery so soon after weaning her off steroids, so no one is surprised that she is not healing well, but since we couldn't wait to do surgery, there's really no one to blame or be frustrated with. It's just her little body having trouble doing what it needs to do.


Bethany's port incision split open today. I'd taken her & 3 siblings out for an orthodontist appointment for one child, an orthotist appointment to pick up new AFOs for Bethany, and a handful of other errands that needed to be done. After the orthodontist appointment, we headed down the street to the orthotist's office. While there, I noticed that Bethany's shirt was bloody in the area where her port is, so I peeked under her shirt to see what was up. I was shocked to see a half-inch by one & one-half inch gaping wound where her closed incision had been! I could see at least one-half inch into the wound. Fat & muscle were clearly visible. Though I felt a bit shaky, I calmly asked the orthotist if he had any bandaids I could put over the wound on my way to the pediatrician's, where I knew I was headed next. He donned gloves & applied 3 steri-strips, then quickly put Bethie's new AFOs & shoes on her feet, had me sign a paper stating yep, I'd picked up the braces, told me he would call the ped's office to let them know we were on our way, and I flew out the door.

At the ped's office, we were ushered immediately into a room. Dr. M (different ped than our primary care doc, but Dr. S doesn't work on Thursdays) looked at the gaping wound, then went to his office to call Dr. I, the cardiovascular surgeon who'd placed the port. It turned out Dr. I was in surgery, so Dr. M tried to contact Bethany's cardiologist to ask what they wanted him to do. The concern was the risk of infection. The incision opened directly to a major blood vessel with a port catheter going into it, so bacteria that entered the wound could easily get into Bethany's blood stream, causing sepsis. With her reduced capacity to heal & fight off infection due to her immune system being

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compromised from steroids, methotrexate & Actemra, a serious infection could be *really* bad.

After Bethany & I had been sitting in the ped's office for 2 hours, waiting for the cardiologists to call back, Dr. M got fed up and began making phone calls to local surgeons to see if SOMEONE would be willing to help. Finally, he found a vascular surgeon over at the Emergency Department of the hospital finishing up a procedure who said he'd be willing to look at Bethany. He didn't make any promises to do anything, but the fact that he was willing to see her was better than anyone else had offered, so I loaded Bethie up and headed across the street to the hospital (a local friend had come to the ped's office to pick up my other kids so I didn't have to keep them waiting in the potentially-germ-filled doctor's office or hospital ED, where they could potentially pick up something & take it home to their fragile brother). Dr. M called the hospital to let them know we were coming.

At the ED, Bethany was immediately taken to triage, then to a room. Dr. P came in within minutes, looked at where the incision had split apart, and told me there were a few options, but he thought the best choice was to stitch the wound closed. Before he did anything, however, he told me he was going to call his wife, who is a pediatric hematology-oncologist. He said he was betting she had some experience with port incisions that popped open & would know what to do. Ultimately, it was decided to stitch the incision closed again, so after EMLA was applied (numbing cream) & local anesthetic injected, sutures were placed to pull the edges of the wound together. Bethany was a trooper during the entire process, never shedding a tear or uttering a whimper. She is one tough little princess!

Unfortunately, within minutes of placing the last suture, just after Betadine had been applied, the skin around the stitches began to tear. Dr. P told me he suspected the skin would degrade around the sutures (ie: stitches won't hold because the skin will tear), but he didn't feel like there was anything more he could do personally, so he covered the stitched incision with xerofoam dressing & a gauze pad topped off with a tegaderm patch, then sent us back to the ped's office to see what Dr. M wanted to do next.

When we got back to Dr. M's, we were again brought right to a room (they never allow Bethany to stay in the waiting room due to her immune compromised status) and Dr. M came in pretty quickly. He prescribed Keflex for Bethany once more, but at twice the dose she'd been getting for the week-long course she just finished on Tuesday (after her chest incision opened slightly while we were in Portland). Just before we headed out, the phone rang & it was Dr. C, one of the

cardiologist's, finally calling back. He added a prescription for silver sulfadiazine, a topical cream that inhibits the growth of a wide array of bacteria & yeast on the skin.

Both Dr. P at the hospital & Dr. M at the ped's office expressed concern about infection. The odds are in favor of an infection developing and Dr. P said that if that happens, Bethany will have to get the port removed while the sepsis is treated and then they'll put a new port in. He looked carefully at the wound (prior to stitching it closed) and told me, "You'll be heading back to Portland for this." Dr. M said he hoped that the stitches would hold. He gave me a pack of steri-strips to add more support to the skin and help hold the incision closed as he said, "I hope this will buy her a couple of days." He knows my family has 3 birthdays on Saturday (Adam, David & Sarah) that I'd like to be home to celebrate. He then added, "They are going to need to see this." I was sent home with instructions to *carefully* watch the chest incision for any sign of widening & to bring Bethany in at the first sign that it might be happening. I'm also supposed to watch for signs of infection.

I will be praying against infection setting in. I will be praying we can keep the incision closed & that it will heal. I will be praying her chest incision doesn't open. I will be praying the muscle fibers of her heart hold together securely. We still have 9 more days of steroids to treat the post-pericardiotomy syndrome. I wish we could stop those since it's making everything worse.

Bethany is doing okay. She's not really any different than she's been for months. It's very sad and discouraging and everyone in my family wishes we could find a way to help Bethany feel better. The happiest I've seen her since mid-April was when she was a couple days post-op, still getting regular doses of morphine & thus pain-free. It was so wonderful seeing my happy girl again, but it was much too brief. I am hoping the future will be happier for my Bethiebean. I don't know how we're going to achieve that goal, but I won't stop trying to get her there.


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Written Sep 8, 2012 2:23am

Hi. We just got home. Too much happened in the past 18 hours for me to even begin to try to write it all out, but I'll give a synopsis.

Bethany woke with a temperature of 102 degrees. Two hours after receiving tylenol with codeine & ibuprofen, her temperature was still 99.8 degrees. As soon as the meds wore off, her fever went up again and came down to just 100.0 degrees 90 minutes after dosing her once more. She saw the cardiologist, cardiology PA & rheumatologist today as attempts were made to determine the cause of her fever & other symptoms. Bottom line? It's strongly suspected that Bethany has postpericardiotomy syndrome, which is inflammation around her heart & lungs and high levels of antiheart antibodies. It's an autoimmune response of her body to the heart surgery, where her body is "attacking" her heart post-operatively. As such, she is now on high dose steroids for the next few weeks to bring this inflammatory process under control. She is also remaining on antibiotics because if what she has is not postpericardiotomy syndrome, then the next guess for her symptoms would be a bacterial infection of some sort, which would lead to an attempt to determine the source & extent of the infection (localized versus bacteremia). I was told we also needed to consider the possibility of a line infection. I replied that if Bethany has an infection of her central line just a week after getting it placed, I will put my head through a wall from banging my head so hard against it. Dr. K nodded in agreement, but reiterated that we had to keep it in mind as a consideration.

In addition to the heart issue, Bethany's labs are highly suspicious for the beginning of an arthritis flare. I asked how that would be possible in light of her having just gotten Actemra yesterday and Dr. K (rheumatologist) explained that with Bethany having had 2 units of blood transfused during her surgery, some of the antibodies built up against the antigen that causes her arthritis could have been lost (when she lost the blood that contained Actemra antibodies). As such, it could allow her arthritis to flare up, especially if she's got this other infection/inflammation process going on.

I was told we could go home, but I am to keep a fever journal & watch carefully for signs that her chest and/or port incision is/are opening and/or not healing well. The steroids will affect her healing, I was told, and there is enough concern about it that Dr. K tried to tell the cardiologist that he could not put Bethany on 'em, but the cardiologist insisted it needed to be done.

Oh, and remember the red spot I mentioned that was on her toe the day after surgery? No one knew where it had come from & no one was concerned? Well, now her entire toe is red & she has a black streaked

area at the tip. The cardiologist looked at it & dismissed it as an ingrown toenail despite the fact that it is most definitely NOT caused by a toenail digging into her skin. The rheumatologist looked at it again (he'd seen it when B was inpatient & on Wednesday in his office) and was *very* concerned. He had his nurse come in to look & he asked me to take pictures & email them each day so he can keep track of how it's looking. He told me he is worried there is some necrosis of tissue and that it could get worse. He is emailing our pediatrician to give her a heads-up about everything going on and to ask her to keep an eye on Bethany's foot. So... pray that her toe will heal without any problems. That's the last thing this child needs!

Soooooo.... that's where we're at. Home. With a sick little girl who is back on steroids, which we will pray won't negatively affect her healing. I am shaking my head at the complications that have emerged. After Bethany had done so well the first 5 days after surgery, I thought it would be smooth sailing. It is very sad & disappointing that things aren't turning out that way.


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Quick update:

~ Saw cardiology P.A, who changed the compression dressing over the draining incision and told me B would have an echo tomorrow instead of today. If the echo shows a lot of fluid, they'll re-admit her to the hospital, take her to the OR & put in a new drain for a few days. If the echo doesn't show an excessive amount of fluid, they'll let us go home with lots of compression dressings to soak up the drainage. The fluid draining isn't a problem, in and of itself. It's inconvenient, of course, but not a big deal. Infection is the biggest threat. We're praying the echo doesn't show any big pockets of fluid. Honestly, we just want to go home!

~ Bethany had her Actemra infusion today. It took just 90 minutes to do her labs & run the medication into her central line, which was a happy surprise. It typically takes 5-6 hours for our local hospital to get the same process done.

~ She has been a lot more grouchy today. I don't know if she's in more pain or what. We're giving her tylenol with codeine on a regular basis, which is a step backward since she had been needing only plain tylenol alternating with ibuprofen.

Other than that, I don't have much to report on Bethany until after her echo tomorrow afternoon.


By the time I got to the hospital with Bethany this morning (a 5 minute walk), the front of her shirt was completely saturated and I could have wrung the fluid from it. No exaggeration.

I took her straight to the cardiologist's office and we were taken to a room pretty quickly since Bethany was wailing & nothing I tried would console her (she'd already had tylenol with codeine & it wasn't helping at all). About 5 minutes later, the door opened and a nurse peeked in to tell me that the doctor was off-campus and would not be returning for at least a half-hour. A glance at my watch told me we could not wait around, so I asked if I could take Bethany to her rheumatology appointment and then return.

Dr. K and I discussed treatment options for getting Bethany's arthritis under control. We bounced around the idea of giving her daily IV Kineret or a combination of 2 oral meds (please don't ask me the names because I can't remember). Ultimately, we agreed we aren't going to change anything until after she's fully healed from her surgery. Then Dr. K reminded me that I need to contact Dr. C, the ENT, to get Bethany's adenoids removed & tubes put in her ears. It's not a big deal, but it needs to be done.

After the rheumatology appointment, I headed back downstairs to the cardiologist's office. Bethany was still fussy & obviously uncomfortable. The P.A. came in and confirmed that the drainage is coming through her incision, which looks infected, and said that he would like us to stay in town for another 2 days so that they can do an echo tomorrow and Friday to see what is happening with the fluid around Bethany's heart. He asked me to bring her back tomorrow at 10am and then said that after the appointment on Friday, they'll decide where they want to go from there. He prescribed Keflex, handed me a container full of gauze pads & a roll of tape to keep Bethany's incision covered, and told me he'd see us tomorrow at 10.

I could cry because I had thought we could leave today and now we're

stuck here for at least 2 more days. I know God is sovereign in all of this, but since I'm not, I am totally disappointed.


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Written Sep 5, 2012 12:34am

A few hours after being discharged from the hospital, the front of Bethany's shirt was soaked with bloody fluid. Adam & I cleaned her up and we both thought the fluid was coming from the holes where the drains had been pulled out this morning. I took Bethany back up to the hospital to get her checked out because while I know some drainage was to be expected, this was WAY beyond "some". The cardiology nurse told me that yes, there was more drainage than they expect to see, but it would probably be okay. She said that if the drainage continued into tomorrow at the same volume, I should bring her back in. She, too, had thought the drainage was coming only from the holes from where the chest tubes were.

We were wrong. The fluid draining from Bethany's chest is NOT coming from where the drains were removed. While yes, a tiny bit of fluid is draining out of those holes, the large amount is coming from an 1.5 inch opening in the lower part of her chest incision. It's not gaping wide open, but it is definitely split and we can dry off the area and then watch as it starts leaking fluid again. She oozed an area larger than my hand onto her shirt in the space of less than 2 hours. I called the cardiologist (thankfully, it was HER cardiologist versus one of the partners) and he said that yes, wound dehiscence (having the suture line split open) was a known risk of doing this surgery on Bethany since we knew going in she would not heal well. He said to use gauze to soak up the fluid and to take her to the cardiology clinic tomorrow to have the incision looked at. He said that since her white cell count was up, if the incision looks red or inflamed tomorrow, they'll have to put Bethany back on Keflex. He didn't address how we will treat the dihiscence; instead, he told me I would be instructed tomorrow in clinic about what to do.

He then told me he was glad he had me on the phone because he had run into Dr. W while doing evening rounds and she had asked how

Bethany was doing. He had told her that from a cardiac perspective, she was pretty good & she'd been discharged today. Then he told Dr. W about Bethany's slow heart rate & they discussed how anesthesia would slow her heart rate even more & how that would make everyone nervous & how spinal cord surgery can increase the vasovagal response and that, too, could be problematic since it would slow an already-too-slow heart rate. As such, he concluded, Dr. W wants to postpone Bethany's cord detethering surgery. I'm supposed to go by her office tomorrow to talk to her about it.

When I take Bethany to her rheumatology appointment tomorrow, I am going to ask the nurse to call Dr. S's nurse to inquire if it would be possible to arrange Bethany's Actemra infusion to be done on Friday in Medford. If so, the kids & I will drive home tomorrow. If we have to do the Actemra infusion on Friday up here, then I will see if we can get it done in the morning so that we can drive home in the afternoon. Worst-case scenario would be having to do the Actemra infusion up here in the afternoon, in which case we would drive home on Saturday morning.

PLEASE pray that Bethany will HEAL!!! This splitting open of part of her incision makes me worry about the state of her internal, dissolving stitches. We absolutely NEED her to heal sufficiently well enough to hold her heart tissue together once those sutures dissolve.


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Post-op day #5:

Bethany is getting discharged at 2pm! Woo hoo!!!

She had an echo, which showed some remaining fluid around her heart, but she'll be on Lasix for another week to help get rid of the last of it (fluid). She also had an EKG, which showed she still has a very slow heart rate due to sinus node dysfunction. Dr. King said the only treatment would be a pacemaker, but they don't want to do it at her age/size, so at this point, it's something they'll watch, but we won't do anything about right now. We may never need to put in a pacemaker,

but if it becomes necessary, at least it's an easy procedure to do so.

We won't be going home yet. Tomorrow, Bethany sees her rheumatologist. Friday, she has her Actemra infusion up here. And Monday, she has surgery to release her tethered spinal cord. But for a few days, we won't have to be up here at the hospital, which is a big YEA!!!!!


Post-op day 3 and Bethany is sitting, smiling & doing well. She's still draining a bunch from around her heart, but the fluid around her lungs is finally beginning to dry up. Her pain is well-controlled with tylenol and ibuprofen.

It's great to see her smiling and feeling better. I'm amazed at how quickly she's recovering! I know it's typical, but I am not at all accustomed to having a child do things the way they're supposed to after a surgery, so Bethany's recovery from this surgery strikes me as nothing short of amazing. :)


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Bethany is having a better day today. She is sitting herself up for short bursts of time and we're seeing smiles again, which is great!

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Her heart rate is pretty low & her cardiologist told me today there may be some sinus node damage as a result of the surgery, but at this point, they wouldn't do anything about it. He also said it might improve on its own in the next few days, so he'd be keeping an eye on it.

Her white cell count is above 36,000 but she doesn't show any sign of infection, so the cardiologist is hoping it's a sign of her arthritis and not anything more sinister. Since Bethany has no fever and isn't acting sick, it's unlikely she has an infection brewing. Still, she's getting antibiotics just in case.

Other than that, there isn't much to report. She sleeps a lot, is resting pretty comfortably, has only needed blow-by oxygen when the fluid around her lungs builds up, and overall is improving in her mood & ability to move around as the day goes on. All good news!

Oh, and the added benefit of lasix ~ the diuretic she's on post-operatively ~ is that it's pulling off all of the excess fluid she's been retaining because of her steroids. She's down 0.6 kg (1.32 lbs) in the past 24 hours!


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Written Aug 31, 2012 4:04pm

Cutting & pasting from Facebook due to time constraint:

Posted about an hour ago:

Sorry for lack of update... Bethany is having an okay day, but she's fighting against going to sleep, is very itchy (her nurse is trying to get permission to give B some benedryl), is NOT happy about not being able to pull at all of the tubes/wires and she keeps trying to sit up and can't understand why we won't let her. I *did* get to hold her for a few minutes while the nurse changed her bed, but she was pretty uncomfortable with that.

She has petechiae on her thighs, which, when I asked about it, was

met with a surprised, "I don't know..." as the nurse looked closely at her legs & tried to determine what might have caused it. I mentioned that Bethany has funky bone marrow, which led to a phone call to the doctor, which led to labs being drawn to check her coags & platelets to see what's going on. Waiting to hear the results. (update ~ labs looked fine. No explanation for the petechiae, but no one is getting worked up about it, either, so I'm not worrying about it).

Posted just recently:

Bethany is going to be moved out of PICU this afternoon. She's gotten her arterial line removed & the pacer wires, while still in her chest, have been removed from the pacemaker itself. Her chest tubes are still draining a lot, so she won't get one removed as anticipated, but they did take the tubes off the drain machine & attached bulbs to them, instead. All in all, she's doing really well & everyone is pleased with her progress.


Bethie is off the ventilator and NOT happy when she wakes up enough to realize her wrists are restrained and she's laying on her back (not her preferred position) and she's covered in tubes & wires that are touching her. For a severely autistic little girl who has massive sensory issues, this is more torturous than anything surgery could have dished out. Please pray for her to rest peacefully tonight and tomorrow. She got more chloral hydrate to help sedate her.

I am now with Joshua at the RMH & Adam is in PICU with Bethany. We'll switch back in the morning.

Good night to all and thank you once again for the prayer support today. I felt comforted & peaceful, which was really wonderful in light of the circumstances!


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The cardiologist just came by & said Bethany is breathing over the vent well enough to be extubated! In a few minutes, the RT and nurse will come in to remove the endotrach tube. Thank you, God!!!!! We'd been told by Dr. I that Bethany would be on the vent until tomorrow afternoon/evening, but here she is, getting extubated just 3 hours after surgery!!! GOD IS AWESOME!!! Omigosh, I'm so excited that she's doing so well!!!


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Bethie is resting comfortably. She's got a bunch of IVs, an arterial line, a central line in her neck, two chest tubes draining fluid from around her heart, two chest tubes placed to drain fluid from around her lungs if needed, a temporary external pacemaker, EKG leads and a urinary catheter snaking out from her little body, but it's all there to take good care of her. Oh, she's on a ventilator for now, too.

She's doing really well. The respiratory therapist was just in to lower her vent settings because Miss Bethany isn't initiating any breaths on her own and they'd like her to, so by lowering the settings, her CO2 level will rise, which will trigger her to breathe.

Charley had to leave to go back home since he works tomorrow. It was nice to have him here during the surgery & to not have to wait alone. I'm really happy that he got to see Bethany right after and see for

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himself that she looks good and is doing well.

Thank you for all the prayers today! My family is praising God for carrying Bethany through this surgery so beautifully and we are thanking Him for the skilled surgeon & medical staff that He provided to take care of Bethany.


Dr. I said the surgery went really well. He repaired the small VSD, the large ASD, the 3 valves & sutured the mitral valve to make it smaller. He said Bethany has 2 superior vena cavas (5% of population does) and they put her port into the right one. The left one was small, so he didn't touch it.

Best of all? Her pulmonary hypertension was gone when he got done with the surgery!!! Her pulmonary pressure was normal and he thinks it will stay that way! Woo hoo!!!

Okay, I get to go see my girl!


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Surgery update #4: Surgery is done! The team will be bringing Bethany upstairs soon and then it'll be about an hour before we will get to see her. Dr. I will be in to talk with Charley & me soon.

YEA!!!


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Surgery update #3: They've got about another hour and then they'll bring Bethany straight from the operating table to the PICU (the entire team comes with her ~ surgeon, anesthesiologist, nurses, etc) and it'll take another hour to get her all situated in PICU. Next update will be when they are done & ready to bring her up. Woo hoo!


Surgery update #2: Bethany is stable, things are going well, next update in an hour. :)

I know, that's not very informative, but that's all we were told.


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Surgery update #1: 2 hours in. Lines are all placed. Her chest is open & she has just been put on by-pass. Next update will be in 2 hours. Projected time remaining ~ about 6 hours.

Also, Charley got here just as Tami, the nurse case manager, was giving me the update on Bethany, so now I'm not waiting alone. :)


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Written Aug 30, 2012 10:55am

Bethany is in the operating room & I am sitting on the 5th floor in the ICU cardiac waiting area. The anesthesiologist told me it would be 7-8 hours, so today will be a long day. It's a beautiful day, though, and there is a wall of floor-to-ceiling windows in this room, so I can look out at the world going by. I know that no matter what happens, God is running the show & I feel peaceful and relaxed.

Charley is on his way & should be here in another hour or so.

Bethany was in great spirits this morning & gave me a kiss before the nurse & anesthesiologist took her away. It's always easier when the kiddo doesn't know what's coming & therefore isn't fearful or anxious.

I'll update again after I hear how things are going. Thanks for praying & sending good thoughts for my sweet girl!

Written Aug 30, 2012 12:25am

Today was an awful day from an emotional point of view. There was no good news for either Joshua or Bethany and lots of really hard stuff. To top it off, I wound up in urgent care getting diagnosed with a UTI. Really, it was the icing on the cake today.

Bethany's surgery tomorrow is at 8:30am and will take at least 4 hours.

Her tethered cord surgery will be at noon on September 10th.

Thanks for praying for Bethany's surgery tomorrow. I will be with her at the hospital at 6:30am (Adam will stay with Joshua at the RMH so he can sleep). Charley will be driving up in the morning (he got out of work late & was too wiped out to drive safely) & will have to return home on Friday so he can work. I'll post updates as I get them from the cardiac case manager. I've been told the first update will be 2 hours into the surgery since the first couple hours will be spent getting all of the lines/equipment into place (peripheral IVs, arterial line, central line in her jugular, potentially a line in her femoral artery, intubated & placed on ventilator, etc). After that, I was told updates would come more frequently.

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I don't want to sound melodramatic, but I am feeling concerned & would greatly appreciate prayer for not only Bethany, but also Joshua.

His oxygen saturation, which should be in the mid to high 90% range, is going down into the 70's when he is upright & talking during the day & into the low 80's when he is sleeping. I am positive this is contributing to his head pain, which is getting harder to control despite our giving meds every 2 hours. He asks me to gently rub the back of his neck because it hurts. The tubing that runs along the top right side of his head & down underneath the skin along his neck into his abdominal cavity to drain excess spinal fluid from the shunt in his head seems to be stuck in scar tissue, so any time the tubing gets stretched (like when he turns his head to the left) or if someone touches it when washing his hair or rubbing his neck, it causes him pain. We are giving him oxygen more often during the day to help his O2 levels get back to normal more quickly after each apnea episode.

I'm worried about Joshua. I'm nervous about how he will do being away from home for potentially 2 weeks. Stress makes things harder for him & being away from home will stress him. I desperately want to be able to keep him happy & relaxed as much as possible and I don't want him to "tank" while we're up there.

I'm apprehensive about this upcoming surgery for Bethany, which I was told today is being done not because it's good timing, but because her heart is bad enough we can't wait any longer to repair it. It's actually not the ideal time because she's only been off oral steroids for 18 days & she got steroids injected just 6 days ago.

I'm nervous about my appointment with Dr. T tomorrow since it's going to be a tough conversation.

I'm nervous about the pre-op appointment with Dr. I tomorrow since he will be discussing all of the concerns the surgical team has with regards to the repairs they will be doing to Bethany's heart. He will also be the final word on whether or not Dr. W can do Bethany's spinal

cord detethering surgery on September 10th (Dr. K said it was fine, but wanted to pass it by Dr. I, too).

I'm nervous about the pre-op lab draw because if the Actemra Bethany received 4 days ago (which technically should not have been given so close to surgery, but we couldn't risk not giving it to her & having her flare) has made her neutropenic (white cell count too low) or if her platelets (cells that stop bleeding) are too low, they won't be able to do surgery at all.

So... yeah... prayers and/or words of support, encouragement & comfort would be really appreciated. I've said before that it's difficult having 2 chronically-ill, medically-fragile kids and at times like this, it's REALLY difficult. Not just physically, but emotionally. Add in being away from my other kids & husband and... well... it's just a good recipe for feeling overwhelmed & really, really sad.


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Bethany's open-heart surgery will be next Thursday, August 30th, at 8:30am. We'll go to Portland on Wednesday for her pre-op appointments with the cardiothoracic surgeon and labs/chest x-ray, then check in at 7am the next morning for the surgery. If all goes as expected, she'll be inpatient for 5-7 days.

Dr. W, our neurosurgeon, might do Bethany's spinal cord detethering surgery on September 10th, when Bethany is sufficiently healed from her heart surgery to enable her to tolerate the back surgery and before we leave the hospital. Everyone agrees that it's best to get as many surgeries done while Bethany is able to tolerate them since we don't know how long we'll be able to hold her off of steroids. Considering how severe her arthritis is despite being on a 30 mg/kg dose of Actemra, it's anticipated that Bethany will be back on steroids again eventually.

The cardiothoracic surgeon will also place Bethany's central line (her port), thus saving her an extra trip to the OR.

I'm glad the surgery will finally be done & am hopeful Bethany will heal well and have no complications. I will have Joshua up in Portland, too, and will be meeting with his palliative care doctor and getting things with his medications & some other issues hashed

out during the time Bethany is inpatient, so prayers for both kids would be really appreciated!

Tomorrow is Actemra day, which will hopefully go without a hitch. I also hope her labs will look good & that they'll stay looking good so nothing messes up the surgery next week!


I'm up in Portland with Bethany, Megan & Joshua. We came up yesterday & will leave tomorrow. We've had a very busy 48 hours.

Bethany is puking some of her feeds, but no, she's not sick ~ it's 3 days before her next Actemra infusion, so she's cranky & pukey & rashy & puffy. At least her body can't get a full-on flare going, but still, she is not a happy camper. We get about 10 decent days after an Actemra infusion and then things get lousy until her next infusion. Dr. K said we may be able to move the infusions to every 10 days. He's going to check if insurance will cover 3 infusions per month rather than 2.

She saw the geneticist, who said he can't really DO anything to help Bethany until she's had some of the big things that are "wrong" with her taken care of. As usual, the need for heart surgery is standing in the way. He said there is no physiological reason why Bethany should not be walking & he doesn't know why she can't. He also said, very bluntly, "Right now, her biggest threat to staying alive is a cold. If she gets sick, she's toast. That will be what kills her." Gosh, Dr. A, thanks! He wasn't being unkind or a jerk ~ he said it in a very concerned tone of voice, as though it was genuinely worrying him. But still, it was BLUNT and both Megan & I sort of coughed like, "Did you just say that?!?" when we heard the words come out of his mouth.

The medical supply company who ordered Bethany's wheelchair for us met me at the hospital to deliver the chair. It is freaking adorable. It's fuschia, tiny & I can lift the thing into my van with just one hand! Bethany likes it, especially when we spin her. The DME company forgot to order the tall push handles, so it's not super-comfortable to push Bethany right now, but the new handles will come in soon (the guy apologized & said he'd order them tomorrow) & then they'll get put on, so no biggie. It's great to finally have it for her.

Today, Bethany had her MRV study (series of 3 MRIs of both upper extremities & her chest) to assess where a central line might be

placed. During the study, she had to be intubated after some breathing issues, but she was stabilized quickly & did fine. After the study was complete, Dr. K (rheumatologist) came to the MRI center to inject Bethany's joints with cortisone. He removed fluid from both knees and injected both knees, wrists, the front of both ankles & the back of one ankle. With the two extra pokes from IV placement, Bethany has a bunch of bruises & needle marks tonight (11 total).

This afternoon, Bethany saw her cardiologist (another Dr. K... I'll refer to him as Dr. K2 in this post to differentiate from Dr. K the rheumatologist). He did an exam, an echo, then came in to discuss the results. In a nutshell, Bethany's pulmonary hypertension (PH) has gotten bad. She has stenosis of her tricuspid valve, causing high pressure. Her atrial septal defect has gotten large, her ventricular septal defect is still small, her mitral valve leakage is moderate-to-severe & her aortic valve leakage is mild-to-moderate. He said he thinks she should have surgery in the next 2-3 weeks, but he needs to present her case to the surgical team tomorrow to determine if they want her to have a cardiac catheterization to get more information about the severity of her PH prior to surgery. Dr. K said he thinks they can repair her heart & get her PH to be okay with just surgery, but cautioned that the cardiothoracic surgeon might disagree with that assessment. He did not elaborate about what we may have to do after surgery if her PH is not better. I *think* they treat residual PH with medication, but I honestly don't know for sure.

When she has surgery, she'll be in the hospital for 5-7 days. Once we know the surgery date, I will talk to Dr. W (neurosurgeon) to see if she wants to schedule Bethany's tethered cord surgery to be done at the end of her heart hospitalization. Everyone (doctors) wants to get as many surgeries done as possible while they can be done.

Dr. K2 also said that he would talk with the surgical team about placing a central line for Bethany (her port) during her heart surgery so they can spare her an extra time of being under anesthesia. That would be convenient, most definitely.

Dr. K2 said the team is slightly nervous about Bethany, but he understands they need to do this surgery now, while she is off steroids, since it isn't going to last. He said I will hear from his scheduler either tomorrow or Friday to let me know a date for surgery or the cardiac catheterization, if that is what needs to be done first.

I feel worn out. I know it's great that we're finally going to get Bethany's surgeries done, but the reason everyone wants to get the

"big" stuff done is so that we can hopefully figure out what the underlying stuff is that is going on. As Dr. A said yesterday, until the big stuff gets taken care of, it will be impossible to figure out what the littler stuff is. And littler doesn't mean insignificant (he made a point of saying that)... it just means the big stuff has been masking it.

I feel relieved that the heart surgery is finally going to get done. I am really hopeful that she'll have more energy & feel better with her heart repaired.

I feel trepidation, hoping that we aren't going to be putting her through all of this for nothing.

Mostly, I'm just sad for Bethany that she has so many health challenges. Sometimes it's really overwhelming & exhausting, for her & for me (and the rest of the family). My sweet little girl didn't deserve the hand she was dealt, that's for sure, and honestly, I can't see where God is in this. I am reminded of a song whose lyrics include "When you don't understand, when you don't see His plan, when you can't trace His hand, trust His heart." I do trust God & I believe He works things out according to a plan that is ultimately for our good. I don't SEE it right now, but that doesn't mean nothing is happening and I know that. The day is coming when God will wipe all tears from our eyes & we will understand exactly what He was doing all along. I hold onto that promise on days like today where nothing else makes any sense.


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Bethany will be off oral prednisolone as of tomorrow. Just as always, per her usual history, she began vomiting, spiking fevers, experiencing joint swelling & rash and irritability on Sunday. Her body really does not like being off steroids and while the increased dose of Actemra seemed to help, it also seems to wear off after a week & then we're left with an unhappy little girl for a week until we can get her another infusion.

I drove Bethany to Portland today after just 2.5 hours of sleep last night because Joshua had a tough night, so my level of tired after 9 hours of driving today has hit critical level & I will be enlisting my adult kids to take care of Joshua tonight so I can get some much-needed sleep. Anyway, Bethany saw her rheumatologist, who is on the same page as me regarding not wanting to put B back on oral steroids unless we absolutely cannot avoid it. We're going to just deal with Bethany being symptomatic until she has her next Actemra infusion on Friday and then we'll cross our fingers, hope & pray that it knocks the arthritis down & chills it out again. On the 22nd, Bethany will have MRVs to assess where a central line can be placed ~ it's a series of MRIs done one after the other (the MRI center has booked out 6 hours for her), under sedation, and after she's done but before she's woken up, Dr. K will come down to the MRI clinic & inject Bethany's joints with cortisone to hopefully give her some temporary relief & buy her more time off oral steroids while we wait for cardiology to give the "okay" to do her heart surgery.

After Bethany's appointment with rheumatology, we headed downstairs to talk to the cardiology surgery scheduler. I explained that I'd been instructed to let the cardiologist (another Dr. K) know when Bethany was off steroids so we could schedule her surgery. The woman I spoke with was snooty & rude, and after giving me erroneous information that I politely corrected her on, she told me in a voice dripping with disdain, "It says here that we're to wait 6 months after she's off steroids before doing surgery." WHAT??? I asked if that is what Dr. L had written in his chart note. She told me it was. I informed her that Dr. L is not Bethany's cardiologist & had only seen Bethany because Dr. K had gone home sick the day we'd driven up from southern Oregon to see him and as such, I am going to defer to what Dr. K said, which was that we needed to wait 2 months, not 6. She got really snippy with me and I kind of sort of really snapped at her & told her that I know Dr. K has done surgeries on kids who have been on low-dose steroids and THEY didn't have to be off for 6 months, so I wanted to know WHY we've gone from being told we had to wait 2 months post-steroids to 6 months! She said she would have to talk to Dr. K and I told her to go ahead and to make sure she asks him WHY it will take 6 months for MY child to have surgery when it doesn't take 6 months for other kids and to tell him that Bethany's mom wants to know his reasoning (because, you know, if it's because she is multiply handicapped & therefore he doesn't think she's worth the effort, I will pretty much make a huge stink with the hospital over it)!

The receptionist called while I was driving home, sweet as pie, telling me that Dr. King wants to see Bethany so he can schedule her surgery

and she would call me tomorrow with an appointment date & time.

I guess being tired & cranky sometimes can pay off because you lose the desire to make people happy when they are playing STUPID games with your kid's health! The fact is, we don't have time to wait 6 months to do Bethany's heart surgery. She is losing muscle mass in her calves (just got casted for new AFOs because of it) & experiences lower back pain & is having bladder and bowel changes because of her tethered cord and syrinx & nerve damage can be permanent if the cord is not released in a timely manner, but we can't get that surgery done until her heart is okay. Her adenoids need to come out, she needs tubes in her ears to hopefully improve her hearing (she's got some hearing loss in her left ear), and ya know, I don't really CARE that she is an almost-5 year old who looks like an 18 month old and acts like a 10 month old ~~ she's my daughter and is deserves the same level of medical care that her brother has received!

*sigh*

The Actemra is causing Bethany's white cells to drop & her liver enzymes to creep up. Both of those trends could eventually cause her to not be able to receive her infusion. That would be a mess because it's the Actemra that is keeping her arthritis from blowing up again. If she becomes neutropenic or her liver gets too 'angry', I'm not quite sure what step we'll take to get things back under control again. Megan asked Dr. K what will happen if we can't use Actemra and he shook his head, told her "Oh, don't say that!" and then added that it would be a big, big problem. So... yeah... we're hoping it doesn't come to that. She has a bunch of other lab values that are completely whacked out, but as Dr. K said today, she fluctuates wildly from one extreme to the other, so it's hard to know what to worry about and what is okay for *her*. As such, we have to follow trends & that means when we see some potentially-worrisome lab results, we have to wait for a few more lab draws (she gets one each week) to determine if things are truly going in the wrong direction. I am not going to bring up the potentially-worrisome things unless/until it becomes apparent that it's something we're going to have to deal with. I think that is probably the best way to avoid confusion & needlessly worrying anyone. :)

As always, prayer can only help. My little girl definitely needs it.


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Written Jul 25, 2012 10:09pm

Saw the rheumatologist today. Dr. K agreed we need to get to the bottom of what is going on with Bethany, but said he suspects it could take awhile. We talked about short-, medium- & long-term goals. He said he strongly believes we will have to get some surgeries checked off the list before we're going to start seeing improvement in Bethany and he asked me if I think the cardiologists just don't want to do the heart surgery at all since he has known them to operate on kids who are on steroids & he has never heard them give the excuse they keep spouting off regarding Bethany (they've told him & the pediatrician the same thing as me ~ that she won't heal quickly enough for her heart tissue to hold together once the internal stitches in the heart dissolve after about 10 days). I answered that maybe the cardiologists were of the opinion that since Bethany has Down syndrome along with her many other conditions, it isn't worth bothering to do surgery for her. He didn't flat-out agree with me, but he didn't disagree, either. He said he's aware that some physicians hold that attitude & I told him I know that is true. Dr. K then said that what he wants to do is continue weaning Bethany off the oral prednisolone ~ we're a few weeks from her being completely off again ~ and yes, he fully expects that her body is going to try to flare because already she is more stiff, a little swollen & is beginning to develop rashes again and she's at 6mg of steroids per day, so it's going to get worse when she's not getting anything. He said that instead of throwing her back on oral steroids, though, what he wants to do is increase her Actemra again (to 32 mg/kg instead of 24 mg/kg ~ the FDA-approved dose is 8-12 mg/kg, btw, and since this is a new drug, no one knows what the long-term effect of giving megadoses is. That's reassuring, right?), which we'll start this Friday. Then, when she starts flaring again, he will inject her joints with cortisone in order to buy us some time. He'll report to the cardiologist that Bethany is not on oral steroids and he will ask when they will do surgery & nail them down on a date, then we'll continue using cortisone injections to keep her arthritis from flaring badly until after she's had her heart repaired. I asked him, "Will they do surgery if she's got systemic steroids in her?" Dr. K answered, "It's not oral steroids and what they keep saying to me is they won't do surgery if she's on oral steroids." I guess that means he isn't going to specifically tell them anything about the cortisone so he can try to get them to do her heart surgery, though he did not specifically say that.

Once we get her heart surgery done, Dr. K said we'll push Dr. W to get the tethered cord/syrinx taken care of ASAP and then we'll have the ENT do her adenoids & ear tubes. By that point, Dr. K said that either

Bethany will begin improving or we will have a clear picture of what else is wrong.

The general surgeon yesterday mentioned it is probably likely that Bethany has some syndrome that would tie all of her various issues together (aside from Down syndrome). I had mentioned in my email to Dr. S (which subsequently got sent to Dr. K) that I'd read that different leukemias have genetic markers (chromosome translocations) and if we did a FISH chromosome analysis, it could work as a "back door" way to evaluate for leukemia since we can't get a bone marrow biopsy done. Dr. K agreed that it was worth checking out and referred Bethany to the geneticist (same doc who diagnosed Joshua). Megan & I went to Dr. A's office after the appointment with Dr. K was over. That was actually pretty fun since the scheduler listened to my explanation of how Dr. K had just seen my daughter & was going to refer her to Dr. A, so I would like to make an appointment while I was still in town, then she told me to go sit in the waiting room while she looked up Dr. K's notes to see what, exactly, he wanted from Dr. A. About 10 minutes later, she came in to tell me that Dr. K hadn't said anything about Bethany seeing Dr. A in his chart note. I asked, "Really?!?" then grabbed my phone, flipped it open & scrolled down to Dr. K's number, and just before hitting "call", I told the scheduler, "Give me a second & I'll call Dr. K to ask him to tell you directly that he wants her referred." She quickly told me, "It's okay; I can make that call." I snapped my phone closed, smiled & said, "Thank you! I'll wait here!" Another 10 minutes later she walked back in to tell me that she understood we'd be at Emanuel on August 22nd and Dr. A had an opening on August 21st at 1pm, so could I come up a day early for that? I readily agreed, thanked her, and that was that! LOL It was funny because I KNOW she wanted to put me off until they got the official referral, but I know how the game is played and was like, "Nope; I don't THINK so! I want an appointment before October, thanks!" :)

Soooooooo..... that's basically the gist of it all. Bethany is grumpy when messed with & fine when left alone. In other words, same as she's been the past 3 months. Dr. K agreed that she is capable of much more than just sitting around doing nothing all day long ~ as he said, he's SEEN her standing & cruising & crawling & interacting. He knows she used to be able to do it & would like to help us get her back to being able to do all of that and more.

I asked why her red cells are always abnormal in morphology (shape) and he admitted that he didn't know. I asked why her neutrophils were SO abnormally high, especially when Actemra is supposed to cause low levels of neutrophils, and again her told me with a shake of his head that he honestly did not know. This time, though, he wasn't blowing

everything off & said that while he didn't know what her abnormal lab values meant, he did agree with me that we need to get it figured out and the first goal is to get her off steroids so we can hopefully start getting some lab values that AREN'T skewed by steroids.

Megan asked what would happen ~ long term ~ if we can't get Bethany off steroids. I chimed in, "Because we know that's not good for her, too." Dr. K looked sad and serious as he shook his head slowly, urging, "Don't say that..... don't say that." Megan pushed further, saying, "Sorry, but we're realists, not optimists." I added, "We know the goal is to get her off steroids and keep her off, but it's been over 1.5 years and we haven't achieved it yet, soooooooo....... what happens if we can't get there?" He thought about it for moment, then replied, "We'd have to go to the nasty stuff." I asked, "Kineret?" He nodded. I asked, "Could we give her IV Kineret instead of daily injections?" He froze for a second, then smiled really big and said, "IV! She'll have a port! Hey, we could DO that! What a great idea!" I told him I knew a teenager who gets Kineret via IV. He asked, "At home?" I nodded, saying that I had a few friends who did IV meds at home. He said I could handle that & I agreed that I could, so if it comes down to Bethany needing Kineret, I think I've "saved" her from getting the horribly-painful daily shot version in favor of getting it via IV through her port. It'd be nice if we don't wind up needing to go that route, but at least we have a plan if it becomes necessary.

I'm glad Dr. K and I are finally on the same page (here's hoping we STAY on the same page). Megan and I could tell that he really does care about Bethany and he's just as puzzled by her as we are. For the first time, though, I feel like we might actually be able to start moving forward toward getting her figured out, and that's a relief.


Bethany saw the general surgeon today for a consultation about getting a port. There's no question she will benefit from having one. The only question is where they'll put it. Apparently, Bethiebean had a bunch of central lines placed during her hospitalizations while in Ukraine, as evidenced by a bunch of scars she has on her upper chest region. Charley & I hadn't known what those scars were from, but the surgeon immediately identified them as being from previous central lines. Dr. B wants some imaging done prior to scheduling surgery so she can see where Bethany has accessible veins. The test, an MRV (http://stroke.about.com/od/glossary/g/MRV.htm), will be done in the next 3-4 weeks (I'm waiting to hear back from the scheduler). After the surgeon gets the results, we can get the surgery done, so hopefully the port will be in place about 6 weeks from now.

Tomorrow, Bethany sees her rheumatologist. I don't really know what to hope for at this point. I mean, it'd be great if he would get on-board with the idea of trying to figure out what the "thing we're missing" actually is, but I've pretty much given up hoping that it's going to happen. It seems the doctors are going to wait until things get ugly before doing anything and I can't MAKE them do anything differently. Still, tomorrow's appointment provides another opportunity to politely "bug" him and who knows, maybe he'll surprise me. It could happen!

We'll see.

At the current rate she is being weaned off oral steroids, she will be done on August 10th. Whether she'll get to that goal & then be able to stay off steroids for more than 2 weeks remains to be seen. We're hoping that the higher doses of Actemra that she's been getting will be enough to keep her arthritis from flaring once she's off steroids. What I personally am hoping is that once she's off steroids, whatever this "other thing" is that she has going on will pop up & announce its presence so we can know definitively what is going on & start treatment. My ultimate goal is for Bethany to get back to a place where she is happy & able to progress in her development again. It's gotta happen sometime, right?


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Bethany's pediatrician is increasing her daily caloric intake to 1800. That is an 80% increase for a child who has been completely fine with her 1000 calories/day from the day we brought her home (from Ukraine) until recently, when she began losing weight for no explainable reason. She hasn't grown in height due to steroids, her activity level is zero (and it is important to mention that when she WAS active ~ playing, standing, cruising, etc ~ her daily caloric intake of 1000 was totally adequate), and she has maintained her weight between 13 and 13.5 kg for the past year (fluctuations have been influenced by the amount of steroids she's been on). Now she's been symptomatic with some "mystery thing" for 3 months (as of today) and suddenly her weight starts dropping DESPITE being on steroids (which

typically cause her to gain weight) & continues to drop despite our adding an extra 200 calories per day, and the answer is to almost double her calories every day? Shouldn't the answer be to FIGURE OUT WHAT IS GOING ON INSIDE HER BODY TO CAUSE HER TO LOSE WEIGHT UNINTENTIONALLY?!?!?!?!?!?!? Oh, and figure out what is making her lower back hurt? And all of her other symptoms????

Forgive me; I'm obviously an idiot who thinks that identifying the underlying problem would be the best thing to do. Treating symptoms is fine if you can't cure the underlying problem, but if you CAN cure the underlying problem, then shouldn't that be what we try to do since getting rid of the problem will remove the symptoms, as well?????

************* SCREAM ****************

I don't even know where to turn anymore. I don't know why no one wants to get to the bottom of this. If Dr. X doesn't know what's wrong, shouldn't they refer to Dr. Y or Dr. Z or to Dr.'s LMNOP ~~ in other words, keep seeking until they get an answer? Silly me for wanting answers, I guess.

For the record, I know that if Bethany has something causing her to burn through calories, she does need the extra calories each day, so we'll give her the 1.5 calorie/mL formula once it's delivered & maybe she'll feel a bit better. My frustration stems from the fact that the doctors aren't trying to identify WHY Bethany is losing weight; she's just tossing extra calories at the problem and ignoring what's going on to cause the problem in the first place.


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I haven't updated not because nothing is going on, but because we still do not have answers for what is wrong with Bethany. STILL. Three months of symptoms. Three months of weird things showing up on her labs. Three months of bugging doctors & seeing specialists & hearing that yeah, they agree we're missing something... there's *something* going on besides the arthritis... but no one knows what it is, so we are told we'll just have to watch & see what happens.

What's happening is that Bethany is still not doing ANYTHING. She either sits or lays in one spot all day long. If we try to play with her, she cries. She doesn't want to move. She doesn't want to be moved. She's okay to be held as long as it's done exactly as she wants. Unfortunately, she's nonverbal & does not point or otherwise give any clue how she *wants* to be held, so the challenge lies in trying to get her comfortable on your lap before the discomfort & frustration irritates her to the point she starts pitching a fit & you're forced to put her down again. She doesn't want to bear weight on her legs, can't stand up straight without crying & still acts as though her lower back & hips are causing her a lot of pain. Why? No one knows.

The curious thing is that despite being on steroids, which typically cause weight gain and have always caused Bethany to chunk up in the past, she is losing weight. This past week, we had added an additional 180 calories into her daily diet (g-tube feeds, so we know exactly what she's getting), but yesterday, when she saw the pediatrician prior to her Actemra infusion, she was weighed & we found she'd lost another pound. She is now under 27 pounds. It doesn't make ANY sense & there has been no logical explanation given for it. Bethany is metabolically stable ~ her labs prove that. She is less active than she used to be. She's getting more calories than she was since she isn't throwing up her feeds AND we added in the extra 180 calories per day. This next week, we are adding in an additional 240 calories via a 6th feed per day. If she loses weight despite this, I am going to lose my mind. Seriously. I am so tired of no one wanting to step up & try to get to the bottom of Bethany's symptoms!!!

Her labs yesterday had some irregularities... as always. Her white cell count was 36,700. Steroids lower white cell count, as does Actemra, yet Bethany's is abnormally high. Her neutrophil level is *way* high ~ over 33,000 (normal is 1,500 - 1,800). Actemra typically depresses neutrophils, so why are Bethany's so high? Steroids can raise a person's neutrophil level, but in the middle of June, when Bethany was getting multiple doses of methylprednisolone (high-dose steroid via IV) & a high dosage of oral prednisolone, her neutrophil level did not get higher than 17,410. Now, despite not receiving methylprednisolone & being on half the dose of oral prednisolone (ie: overall steroid levels being much, much lower), her neutrophil count has almost doubled from the highest its ever been. How is that logical?

Bethany's ongoing symptoms are hip/lower back pain, fatigue, malaise (not wanting to play or move around), irritability, weight loss (unintentional), & occasional fever (used to be every day, but the increased dosage of Actemra seems to be controlling them for the

most part). If it weren't for her inability to move around & enjoy interacting with people the way she used to, I wouldn't be so concerned. But the malaise, coupled with her hip/back pain & weight loss, *does* concern me. Especially since she *is* on steroids, which have always caused her to gain weight, not lose it. As I've been saying for the past 3 months, I can't shake the feeling we are missing something. I wish God would direct me to a doctor who would want to figure this out as much as I do. I know Bethany's doctors care, but none of them seem to be interested in getting to the bottom of this puzzle, which is both frustrating & disappointing. If it turns out there's nothing seriously wrong with my daughter, great. But if it turns out there IS something seriously wrong with Bethany & no one bothered to try to figure it out months ago & her treatment/prognosis is compromised because of that delay, well.... that could get ugly.

I understand Bethany is complex. I understand that her arthritis complicates the picture, especially since steroids can mask conditions that would otherwise be more obvious. However, when a handful of specialists agree that yes, there's "something else" going on aside from arthritis with a child, then someone ought to be willing to take the next step of trying to determine what that "something else" is. Call me crazy, but that seems to be the logical thing to do. 'Course, I'm just a mom with no formal medical training, so what the heck do I know, right?

Ugh & sigh.

I keep telling myself that God's timing is perfect, so whatever is wrong with Bethany will be diagnosed in His time, when He wants it revealed. I sure don't understand what He's waiting for, though, so I will continue to pray that He'll give wisdom to myself & Bethany's doctors & that He'll give SOMEONE besides me the desire to get answers for what ails my little girl.


On Tuesday, Bethany saw her rheumatologist, hematology-oncologist & neurosurgeon. It was a busy day.

Dr. K (rheum) agreed that she looked better than she had, but was not yet back to her baseline "good" that we achieved from mid-February to mid-April. He said he'd like to find out if the increased dosage of Actemra is going to be helpful in controlling Bethany's arthritis, but we can't know for sure until her steroid dose is much smaller. We're weaning her down 1.5mg every 3 days, so in another couple of months, we should have a better idea of what (if anything) the

Actemra is doing for Bethany.

Dr. W (neurosurgeon) confirmed that Bethany needs surgery to release her tethered cord. At this time, her syrinx is small enough that it does not require treatment; however, if we have to wait an extraordinarily-long time to do surgery, that could change. Dr. W said she won't operate until it is safe to do so. I asked what qualifies as "safe" in her mind and she answered, "When her heart is safe to undergo surgery with me." She wants Bethany to have her heart surgery first & then she (Dr. W) will do her back, which is exactly what I thought she'd say, so I wasn't surprised to hear it.

I learned that Bethany has a deformed coccyx ~ her tailbone, instead of curving under like normal, flips outward toward her spine. It's not a big deal, but can cause discomfort when sitting for long periods of time, so when Dr. W does surgery, she'll cut that bone off completely. Well, IF she ever does surgery. With everything riding on getting Bethany off steroids long-term, I have doubts we'll ever get her surgeries done. We'll see, though.

After Bethany's appointment with the neurosurgeon, it was Joshua's turn (his page is updated at http://www.caringbridge.org/visit/theyoungestof7). Once we were through, we headed across the hospital to the blood disorders clinic to see Dr. G, the hematology-oncologist (heme-onc, for short). Labs were drawn, height & weight taken (she's actually 34.5 inches tall, not 35 like we thought, and her weight is down to 12.2kg with clothes on), and then we waited for the doctor to come in.

Dr. G examined Bethany & talked to Megan & me about Bethany's symptoms and our concern that we're missing something because Bethany isn't getting back to her baseline "good" and she still has the horrible pain in her lower spine/hips. Dr. G asked how we know she hurts in her back and I reached forward to tilt Bethany's hips so she would have to sit up straight, causing her to grimace & begin crying. I immediately let go & comforted her & she settled back down, but it was worth demonstrating for the doctor so she could SEE what Megan & I were talking about. Anyway, Dr. G understood our concern and told us that yes, it is absolutely possible that something is being masked by the steroids Bethany is on and yes, she agrees there is something else going on aside from systemic juvenile arthritis because Bethany's labs indicate abnormality in her bone marrow, but because Bethany is jacked up on steroids, there is no way she can do a bone marrow biopsy and get an accurate result. As such, all we can do is wait. Dr. G said that whatever is going on WILL show its face eventually and then we'll know what to treat & how to treat it. Until then, all we can really

do is wait, wean her down on steroids, and watch her labs to see when something shows up.

No one knows why she is losing weight. She is getting more calories now since she isn't throwing up anymore like she did for 11 weeks, plus she's on steroids, so she *should* be "porking out", as her rheumatologist put it. Why she isn't, and is losing weight, instead, is puzzling and a bit concerning to the heme-onc. It's another thing we need to watch & see what happens.

Wait & see. Watch & see. I'm really beginning to loathe those phrases, especially when it pertains to my little girl.

Bethany *is* doing better than she was before we started her back on oral steroids. Still, she doesn't like to move around very much and she's only "okay" as long as no one is touching her in any way that bothers her. That means you can't touch her lower back or bend her wrists or move her fingers or bend her in any way that she hasn't already bent herself. Basically, she's happiest when she's left totally alone but has someone talking to her & loving her from a nearby distance. We all want to get our Bethiebean who likes to play in our lap back. Maybe once the "whatever" we're missing finally shows up and gets diagnosed, we'll see the return of our little girl (assuming we keep her arthritis under control, too, of course).


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Written Jun 25, 2012 5:04am

Bethany has me perplexed. The steroids she's getting are helping a little, but not a lot, and she is still not feeling well.

Historically, IV methylprednisolone + Actemra + oral prednisolone could knock down a flare of any severity and give us back our happy Bethie. This time, though, has been so different. Two infusions of methylpred gave us 36 hours of improvement before things turned bad again. Doubling her Actemra dose didn't do much at all. And now she's been on oral prednisolone for 5 days at a high enough dose that we'll have to wean her down in two more days because the rheumatologist doesn't want her to get toxic, yet she is still not moving around much

at all & when I tried to get her to stand today (for just a few seconds), she erupted in tears from pain. Her hips & back are still painful, as are her knees when we bend, then straighten, them. The inflammation in her hands & feet is gone, but she continues to keep her fingers straight & not use them.

I don't know what's going on. I do wonder if this is all arthritis-related or if she does, in fact, have an underlying malignancy such as leukemia. I'll be calling the heme-onc in Portland tomorrow to see if she has received the referral for Bethany to have a bone marrow biopsy. If so, I'm going to schedule that procedure. If not, then I will call the ped's office to determine when they intend to send the referral. I know we have to wait a few weeks for the methylprednisolone to be out of her system and thus not skew the test results, but I would like to know we've got the biopsy scheduled so I can know how long it will be before we know, definitively, whether or not Bethany's poor response to her recent treatment is being influenced by cancer.

On the plus side, she isn't crying as much as she was, she isn't super swollen, she's tolerating feeds better & doesn't throw up every day, and she is able to scoot around a little bit when she chooses to. They're steps in the right direction and at this point, I am thankful for ANY movement toward improvement.


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Sorry that I have been a lousy blogger as of late. A friend from Australia has been in town & that has distracted me from updating like I should have.

Bethany spent 2 days in the hospital before being discharged Tuesday afternoon. The whole issue with her lung turned out to be a non-issue. The fluid build-up around her lung is something that happens with systemic JIA. Dr. K (rheumatologist) informed Dr. S (ped) that with severe s-JIA, fluid can build-up in all of the serous spaces in the body ~ around the lungs, heart, intestines, etc. So as it turned out, Bethany didn't have an infection, did not need antibiotics, and after getting more methylprednisolone via her IV (which was placed on the left side

of her head after multiple attempts in her extremities were unsuccessful) and a whopping dose of Actemra (24 mg/kg versus the usual 12 mg/kg), there was really nothing else she needed at the hospital, but they kept her so they could observe her reaction to the medications.

Not surprisingly, she perked up Sunday afternoon after getting steroids & Actemra. She was tolerable ~ okay, but happy only as long as she was left alone ~ on Monday, and by 2am Tuesday morning, she was back to being in pain & irritable & inflamed. VERY disappointing!!! On Tuesday morning, Dr. S talked with Dr. K & it was agreed Bethany needed to be seen by him. She wasn't in any kind of emergent state, so an office visit was scheduled for Wednesday and Bethany was discharged so I could drive her to Portland the following day. Just prior to her discharge, Dr. W, one of the heme-onc's, came in to talk with me. She said Bethany's bone marrow is dysregulated & she still needs the bone marrow biopsy, but because she was given the two big doses of methylprednisolone, we'd have to wait 2-3 weeks in order to get an accurate result from the biopsy (big doses of methylpred are used to kill leukemia cells, so if she had leukemia, it could be "masked" by the methylpred she'd received). The biopsy will be done in Portland by Dr. G, the same heme-onc who did Bethany's biopsy in January, 2011. After chatting with Dr. W, I was ready to take my girl home. It was a huge disappointment to be taking her home in the same condition we had admitted her. The hospitalization felt like a huge waste of time.

The next day, Wednesday, Megan, Bethany & I traveled north to meet with Dr. K. Over the course of a couple hours, we exchanged information & clarified some issues & came up with a plan for Bethany's treatment. For now, she will be on high-dose oral steroids plus the increased dose of Actemra & she'll continue receiving weekly injections of methotrexate. In a week, if her symptoms are significantly improved, I will reduce her evening dose of prednisolone and then we'll slowly begin trying to wean her down. Dr. K doesn't anticipate that we'll be able to get Bethany completely off steroids since we haven't been able to achieve that in the past 18 months of trying, but that would be the eventual goal if we could ever achieve it. For now, we're hoping to get her on a low dose of steroids versus the high doses her body seems to prefer because so much damage can be caused by staying on high doses over a long period of time.

If her symptoms are not significantly improved after a week of the high dose prednisolone, I'll take her back to Portland to get cortisone injections in her major joints, as that might help her body get its immune system back under control and stop it from attacking itself. Then we would continue trying to wean her down to a low daily dose of

prednisolone with the ideal goal being to get her off of it completely, but a realistic goal being to get her to a low enough dose that the bad long-term side effects of steroids get delayed (no way to avoid them if she never comes off steroids).

If we need to, Dr. K will increase the dosage of Actemra once more & see if that helps. It would be ideal if we could eventually find a dose of Actemra (a SAFE dose) that would control Bethany's arthritis. If that can't be achieved, however, there is one other medication that we could try. It isn't anything Dr. K WANTS to have to try, however, since it requires daily injections which, by his description, are "horribly painful.... really awful shots." I don't know about you, but in my experience, when a doctor admits that something hurts a lot, it must be really bad & that makes me want to avoid it completely! It is my hope we never wind up at the place where we have no choice but to try the Kineret.

Dr. K said s-JIA is a bad, bad disease. Bethany has a severe case of this disease. It is incurable. He does not think she has an underlying malignancy (leukemia) since all of her symptoms fit s-JIA ("like a glove", he said), but admitted that at least leukemia has the word C-U-R-E attached to it, which makes it more hopeful than severe s-JIA. I told him that I had been told it was unlikely Bethany would survive chemo because of all of her other problems & he agreed with that assessment. At that point, we just sort of looked at each other for a moment before he told Megan & me, "Yeah, we are really between a rock and a hard place with her." Yep. No arguing that.

Megan and I asked what the ideal situation for Bethany would look like. Dr. K answered that it would be us being able to get her disease under control by whatever means we could. At this point, that is the goal. The only goal. Get this disease under control. He said that ideally, she would respond to the Actemra & that would enable us to get her to a low dose of oral steroids or, if he was dreaming big, maybe even get her to where she didn't need steroids at all.

Then I asked, "Since Bethany has not been even close to ideal yet, what is the plan if she does not respond favorably, long-term, to the increased dose of Actemra." Dr. K said that he didn't really think the Actemra was going to do it since it hadn't yet, but he would give the increased dose a few tries before giving up on it. Then he said that if the Actemra doesn't work, even in conjunction with low-dose steroids (because we can't keep her at high doses indefinitely), we'll have no choice but to try Kineret. When he saw me cringe involuntarily, he said, "I know... I know... but if it's all we have..." and then his voice trailed off. I told him I understood what he was saying, but if nothing else

worked, maybe the Kineret would, and while I did not *want* to give Bethany daily painful shots, if there was a benefit to her in doing so, I would. He agreed, adding that if the benefit outweighed the lousy aspect of getting the shot, then it would be worth it. Still, we all agreed we hope it doesn't come to needing that medication.

Megan then asked, "What if we end up having to try the shots and those don't work, either?" Dr. K looked very sober as he answered, "That would be bad. Really bad." He went on to explain to Megan and me how sometimes, this disease eludes being controlled, and in those cases, people can lose joints & they live with chronic pain & it's just really horrible.

Dr. K will be seeing Bethany in his office every 2 weeks for the foreseeable future. We'll make the 4.5 hour drive up, meet with the doctor, then turn around and drive back home. Thankfully, Bethany travels well, so it won't be unpleasant making the trip up & back. It'll just be tiring! It will be good, though, for Dr. K to be able to lay hands on Bethany on a regular basis and to be able to make adjustments in her treatment according to how she is doing. He and I are going to be working together more directly versus my calling the pediatrician & her calling the rheumatologist to give him information about what's going on with Bethany. We'll still keep Dr. S "in the loop", of course. It'll just be more like how I manage things for Joshua with Dr. W (his neurosurgeon) ~ I call her directly & we decide what we need to do, and then I call Dr. S to let her know what the plan is. Now I'll do the same thing with Dr. K for Bethany. Hopefully, it'll cut down on frustrations & improve his & my communication. I also have his email address & can contact him via that route.

Bethany is getting tylenol with codeine on a scheduled basis now to help with the pain her achy joints cause her. She'll also be seeing a surgeon to place a port-a-cath. Yes, it's surgery, and no, it's not without risk, but everyone is in agreement that it is inhumane for Bethany to not have long-term central access. Trying to get blood or place an IV has become increasingly difficult & it's cruel to continue inflicting the pain of multiple needle sticks every week on a small child whose extremities are swollen & already painful from arthritis. Dr. K is hopeful that we can get an appointment for the port scheduled soon.

I honestly am shocked by how bad Bethany's JIA is. I mean, I knew it was a lousy condition, but I thought all kids had a somewhat similar experience. Truth is, this disease spans a broad spectrum, from mild to severe. In explaining to Megan & me how severe Bethany's arthritis is, both Dr. S & Dr. K gave examples of other kids in their practices who have this condition ~ Dr. S has a kiddo whose ankle gets sore

sometimes. That's it! And Dr. K has a kiddo who has just a few joints affected, but over-the-counter anti-inflammatory medication is enough to give him relief. Megan listened, then half-joked, "Why can't we have one of THOSE kids?" It really is disheartening sometimes when I think about the big picture. My sweet little girl, with her severe arthritis & heart defects & tethered spinal cord & syrinx & obstructive sleep apnea ~ all of which cannot be repaired while she is on steroids or is unstable while off of them ~ I feel so sad for her when I think of everything going on inside that little body of hers. So many systems are not functioning properly & there's no way to fix any of it unless we can get the main disease controlled, yet even that eludes her medical team thus far. Will we ever get the upper hand on this condition? Only God knows and only time will tell. For now, at least we have a revamped plan and who knows, maybe this time it will work.

You know how people say the definition of insanity is doing the same thing over & over but expecting a different outcome? I've decided the definition of insanity is treating a severe rheumatological disease. We put a child on high-dose steroids, slowly wean them down in dosage until their disease flares, at which point we put them back on high-dose steroids before eventually beginning the weaning process again, hoping that maybe THIS TIME it will work even though it hasn't worked the past 12 times we've tried the exact same process. Yep... insanity. It's why I'm not holding my breath that weaning steroids while giving Actemra is going to work. It hasn't worked yet, and nothing has changed to make me think this time will be any different. That isn't me being pessimistic. That is me being realistic. I'd be happy to be proven wrong. I'd be happy to have the combination work this time. That would be a nice break for Bethany & honestly, she deserves it.

Written Jun 17, 2012 7:05pm

Bethany was admitted to our local hospital this morning. It took multiple attempts to get labs drawn & an IV placed, but the persistent nurses were eventually successful at getting a line in the left side of Bethany's head. Dr. S came to check on our girl and was sad to see Bethany hurting & crying. She ordered pain medication (oxycodone) & then turned her attention to talking to me.

She and Dr. K (rheumatologist) had talked this morning about where this is going and what we're going to do. It's very difficult for the rheum because Bethany is his sickest kid who lives the farthest away from Portland, and he said that if we lived locally, he would be seeing her at least twice a week. Since we're 250 miles away, it's tough for him to tweak her treatment without being able to see her & really SEE how she's doing. He has access to photos & videos, but it's not enough.

Dr. K said the fluid around Bethany's lung & the infiltrates in her lung are part of s-JIA and therefore aren't anything to be concerned about.

We're waiting to hear from the heme-onc regarding when she thinks a bone marrow biopsy can be scheduled. If Bethany doesn't improve after receiving steroids & Actemra, she might be flown to Portland to be admitted at Emanuel so that Dr. K can have hands-on access to her every day. If she goes to Portland, they'll do the biopsy there, which would probably be a lot quicker than the hassle they're going to have trying to get it scheduled down here with the one doctor who does the procedure on children & has a full schedule for the next month.

Dr. S & Dr. K said that at this point, the goal of getting Bethany to where she can have her surgeries is off the table. It isn't going to happen any time soon because she can't have surgery on steroids & she can't have surgery if she's flaring, yet we can't get her off steroids AND keep her from flaring, so we are stuck between a rock & a hard place. The new goal is to get her disease under control, which could require a hospitalization in Portland and/or weekly trips to Portland to see the rheumatologist in order to achieve. For the next 48 hours, the plan is to give her a pulse dose of steroids (400mg methylprednisolone) & her next infusion of Actemra, then observe how she's doing and go from there. There really is no set plan because Bethany's arthritis is so resistant to treatment, which is not the norm, so during this hospitalization, the docs will be taking things one day at a time.


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Bethany is still not doing well & in the past week, her specialists have put their heads together & come up with some unpleasant conclusions. There is no way to put it in an ultra-condensed nutshell, but suffice to say that we spent 5 hours at the hospital on Wednesday so that Bethany could have blood tests for "cancer markers", an abdominal ultrasound to evaluate for either malignancy or other abnormality that might explain her pain there, & an IV infusion of methylprednisolone

(steroids) because after just 2 weeks off steroids, her systemic arthritis is flaring again & they have to knock her immune system back down. I was told yesterday that her disease is severe and out of control, which is not what I wanted to hear with regards to a child who needs open-heart surgery, tethered cord surgery, surgery to get her adenoids out & tubes put in her ears & surgery to place a port, but cannot have those procedures until she has been off steroids (and remained stable) for 2 months. I was also told she is higher-risk for leukemia after the heme-onc evaluated all of her labs from the past 18 months.

Just before leaving the hospital on Wednesday, I was handed a copy of some of the lab results that had come back already (they were all 'stat' labs) & one of the cancer marker tests was abnormally high (as in the normal range is 90-210 mg/dl and Bethany's result was 622 mg/dl, so not insanely high, but more than just barely elevated). On Thursday, the heme-onc called to tell me that she wanted Bethany to have a chest x-ray (to make sure they aren't missing anything in her chest) & a bone marrow biopsy.

Bethany perked up a little bit right after her infusion of high-dose steroids, moving around more easily & without grimacing quite as much, but it lasted less than 24 hours, and by 48 hours after the infusion, she was in even more pain than she'd been prior to receiving the steroids. I took video to show her doctors just how difficult it was for Bethany to move & how awful she was feeling. It really is heartbreaking to watch her.

Yesterday (Friday), the pediatrician, rheumatologist & I had an email conversation regarding what we are going to do. Dr. K (rheum) said it might be time to "throw in the towel" ~ ie: give up & put Bethany back on oral prednisolone since she isn't stable off of steroids. I asked if we could wait until after she's had the higher dose of Actemra + second methylprednisolone infusions before we give up & he said that would be all right. At this point in time, the plan is to give the infusions as soon as the hospital pharmacy can get the meds (hopefully on Monday) and if there isn't any improvement within 24-48 hours afterward, we'll add oral steroids back into the mix.

No one knows if Bethany will ever be able to stay off steroids long enough to have her surgeries. Dr. K seems very discouraged right now. I understand how he feels, as I am in the same boat.

Yesterday afternoon, Bethany had the chest x-rays the heme-onc wanted done. I had thought that would be the one thing that came back as normal, but when I logged onto my computer this morning, there was an email from our pediatrician telling me that she'd received

a call late last night alerting her to the news that Bethany had some fluid outside her left lung & some "streakiness" in the upper part of the same lung. She had put in a call to the radiologists at the hospital to clarify the significance of the findings and then, later, she called to tell me that Bethany will need to be admitted to our local hospital tomorrow morning. Her left lung is not inflating properly & the radiologists are unsure what the streaks on her x-ray are indicative of. The fluid on the outside of her lung would typically be associated with infection, but they said that in the absence of any symptoms, they are inclined to think it may be something else. Infectious disease in Portland is getting involved. Bethany will get IV fluids & no tube feeds to give her gut a rest while they try to figure out what is causing the vomiting & pain there, and she will get IV antibiotics in case there is bacterial pneumonia or some other infection trying to form (we normally would not give antibiotics indiscriminately, but Bethany is too immune-suppressed to take the chance of NOT treating a potential bacterial infection, which would then develop into a serious problem).

The prevailing theory right now regarding her arthritis is that the Actemra has been partially able to control her symptoms, but not completely, and as we weaned her off oral prednisolone, her arthritis was able to get out of control. By doubling her dose of Actemra & using pulse steroids (the high-dose IV methylprednisolone), Dr. K is hoping to bring everything back under control. I will admit that I am a little skeptical right now since the pulse steroids she had on Wednesday did not help, but I understand there isn't really anything else we can do. It's very concerning, though. If we end up not needing to put Bethany back on oral steroids, we'll still have to wait for the methylprednisolone to clear her system before we can start the clock again for the 2 months without steroids (so we can do her surgeries).

As for her bone marrow biopsy, Dr. S emailed this afternoon to say that Dr. B, the heme-onc, is running into some problems because her partner, Dr. W, is the only doctor in southern Oregon who does bone marrow biopsies on children & her schedule is not amenable to getting Bethany's test done ASAP like Dr. B wants. As such, Dr. S is going to call Portland to ask if the same heme-onc team that did Bethany's bone marrow biopsy in January, 2011 can do another one soon. Ah, the joys of living in a small town & not having easy access to lots of pediatric specialists! I am not overly worried about waiting on the biopsy. If Bethany has leukemia, she most likely would not survive the treatment for it (that is not just my personal opinion; I've heard it from medical professionals), so waiting for an extra few days to get confirmation if she has cancer isn't going to make any difference. If she has leukemia, Charley & I will have to have some serious discussions with the oncologists in Portland regarding therapeutic versus palliative

treatment. Hopefully it isn't going to come to that, but honestly, the alternative ~ living this perpetual cycle of using high-dose steroids to control an arthritis flare, then slowly weaning Bethany down until she flares again & needs high-dose steroids once more ~ is not any more appealing. Bethany is HURTING. She suffers all day long. She barely even moves anymore. She lays on her side or sits, unmoving and grimacing if anyone gets too close & she thinks they might touch her. It took her almost 2 minutes to sit herself up last night & it was the saddest thing to watch. Here... see for yourself... http://www.youtube.com/watch?v=PKrOUc8w2E8

I don't want her to have leukemia, but I also don't want her to have to live the rest of her life like what has been happening for the past 18 months. There is no quality of life for her. None at all. We can't even comfort her because holding her for very long causes her pain. We give her ibuprofen and tylenol with codeine. At this point, no one is offering or suggesting stronger pain medication. I know Dr. K and Dr. S are hopeful that if we can get Bethany's arthritis under control again, we can have our happy girl back. What they don't seem to understand is that we had "happy Bethie" for less than 4 weeks. There was that magical month where Bethany was feeling good enough to really interact with everyone & was learning new skills & seemed to enjoy life. That occurred when she was getting Actemra and oral steroids at a high enough dose to keep her arthritis from causing symptoms. Once we weaned her steroids below that unseen threshold, her body began revolting yet again & since we did nothing to stop it over the past 8.5 weeks, she's progressed to where she is today. Yet we can't keep her on steroids indefinitely without causing problems in her body AND we still need to try to get her to where she can safely have the surgeries she needs. How is that going to happen? I honestly don't know. I don't think her rheumatologist or pediatrician know, either.

This is an awful, awful disease. Dr. S told me last Tuesday that Dr. K said Bethany's disease is severe & out of control and that we are going to be fighting this for the rest of her life. Knowing how horrible it is & seeing how miserable my baby girl is because of systemic JIA, hearing that prognosis from Dr. K (via Dr. S) made me feel sick inside & quite hopeless.

I don't know why Bethany has to suffer this way. I don't know why she can't be one of the more than 75% of kids whose s-JIA goes into remission fairly quickly after treatment begins & whose lives are not significantly impacted by their disease. I don't know what God has in mind for her. I can't see anything good coming from this, yet my faith in God compels me to believe that He IS working out something that is good from all of this, regardless of what I can't see or how I feel. I

guess this situation, like Joshua's, will require time to see how God is working. Really, there's nothing else that I can do but trust Him.

It's hard, though. This hurts. Watching my little girl in pain is gut-wrenching. Seeing the frustration in her eyes because she doesn't understand why no one will help her or make her feel better leaves me feeling like the world's worst mother. Sometimes I even feel guilty for bringing her here. I don't regret adopting Bethany ~ that is NOT what I am saying, so please do not misunderstand my words! I do feel badly, though, because instead of "rescuing" her from an orphanage & bringing her to America to enjoy a wonderful life with a family who loves her, I brought her here & now she hurts & suffers & is utterly miserable. In Ukraine, she was kept drugged to the gills & was not aware that there was a problem. Here, though, she is very much aware that there's a problem, and the people who she thought she could trust are not doing anything but making her hurt more (by feeding, changing & dressing her and giving her shots & other medications that make her feel lousy). Yes, I know, she's loved & has a family & gets fed & gets medical care & all that... but it doesn't seem like a very good trade-off for the hell she is going through.

She became part of our family & we have all grown to love her, which makes it hard to watch her hurt. I don't understand why God would tell us to adopt a child who He KNEW would go through everything sweet little Bethie is going through. Now our hearts get to be ripped apart by watching Bethany suffer AND by watching Joshua die. It really feels awful. I know God knows what He's doing, though, which is good because I certainly don't.

Please pray for God to work out the best path possible for Bethiebean & for her hospitalization to be a short one. When I asked Dr. S how long she thought Bethany would remain inpatient, she didn't know. She said at least a few days, but possibly much longer ~ it will depend on what her lung does & how it responds to treatment. They need to see improvement on the x-ray before they'll send her home again.


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I'll be taking Bethany to Portland for appointments with dermatology, neurosurgery, cardiology & rheumatology at the beginning of July. I am hoping we come home with surgery dates for her heart & spinal cord and maybe even some definitive diagnosis (diagnoses?) for the rest.

Tomorrow, she has labs & her Actemra infusion. I'm not looking forward to the nurses having to place an IV in one of her swollen extremities or phlebotomy having to draw blood from her puffy arms, but there's no way to avoid it since she lost her PICC line.

Bethany remains the same, which is pretty sad. If we had any way to make things better for her, we would, but there's not much that brings her comfort or cheers her up these days. I am *really* hoping that she'll experience a big turnaround (in the right direction) once her surgeries are completed & whatever autoimmune "thing" she has going on is brought under control.


So much is going on with Bethany that it's difficult to know where to start.

She's now at 6.5 weeks of daily low-grade fever, intermittent rash (looks like hives), darker red splotches showing up on her lower leg, increasing pain and she's at 2.5 weeks of increasing swelling in her extremities, decreasing energy, decreasing oxygen saturation levels & intermittent vomiting of her feeds. Meanwhile, her labs continue to look fine with regards to inflammation markers (this despite her obvious systemic inflammation!). It's been very perplexing, but in the last 3 days, progress has been made toward obtaining answers.

What it looks like right now is that Bethany has other autoimmune diseases & her heart is having more difficulty pumping blood to her body due to the increasing severity of her atrial septal defect & mitral valve insufficiency (having 2 other leaking valves & a small ventricular septal defect isn't helping matters any, either). I hesitate to be more specific until further testing confirms the diagnostic suspicions, but I am hoping we'll have concrete answers in the next couple of weeks.

Bethany will be having biopsies of her rash & the splotch above her ankle (the two are not similar & are suspicious for two separate diseases), more labs & potentially another bone marrow biopsy (pending the hematologist-oncologist's review of her CBC's for the past year). Pending the outcome of those tests, the tentative plan (which all of her specialists have not yet heard about nor approved) is to do her heart surgery, wait until she's sufficiently healed, then do her tethered

cord surgery, then wait a couple of days before doing her adenoids (and maybe also tonsils) & putting tubes in her ears & a port in her chest. In other words, have one long hospitalization to get all of her surgeries out of the way. The reason for this proposed plan is that if the suspected autoimmune conditions are confirmed, Bethany will need to be put back on Prednisolone to get them under control, and with her heart being in increasingly-bad shape, we have to get her surgery done before we can give her steroids again (she's been off the Prednisolone since May 30th).

Dr. S (Bethany's pediatrician) said she & the other doctors she's been talking to about Bethany are astounded at the number of problems going on in this one small child. So many systems affected & not in a good way, and just like Joshua's conditions, things overlap & contribute to making each problem harder to figure out. It's a discouraging situation, but today I feel better if only because the doctors are working together to try and help Bethany. I don't feel like I'm banging my head against the wall to try and make them listen now, which is a huge relief.


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Written May 16, 2012 7:33pm

Bethany's labs weren't normal, but they weren't so whacked-out that anyone wants to do anything aside from "wait & see". Her white count is up, her red count & hemoglobin & hematocrit are all down, her platelets dropped substantially this past week, two of her three liver function tests were above the high end of normal (the important one is still in the normal range, which is good), her triglycerides are high (222) & her CRP, sed rate & ferritin are all creeping up, but are nowhere near the levels they've been when she's been really sick.

She seems to be having a lot of pain in her hips. She won't stand & when she is sitting by herself & pulls her legs up, if she moves them in a certain position, she will make herself cry. I don't know what the issue is, as usual, but am just making note of it here in case it becomes important at some point down the road.

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Another couple of weeks go by and nothing has changed. Today marks 4 weeks of fevers, rash, daily vomiting, irritability, intermittent joint swelling & pain. Good thing nothing is wrong, though. That's reassuring, right?

Please forgive my sarcasm. It's just really, REALLY difficult to believe there is nothing wrong with Bethany since the symptoms she has are typically associated with something (aren't they?). Maybe all of her lousy symptoms are caused by the Actemra. Maybe it's just her body not being happy about being weaned from steroids. Regardless, this is unpleasant for everyone involved and it would be great if there was an end in sight.

I took Bethany to the hospital to get blood drawn for her weekly labs. I haven't heard anything yet regarding results), which means they're probably all fine. And yes, that's a good thing (really), but in light of what we are living with right now, it's difficult to feel completely happy about normal labs because it doesn't get us one iota closer to understanding what is going on.


Yesterday was a long day, as I drove 250 miles to Portland, met with Dr. K (Bethany's rheumatologist), then drove back home. We had packed to stay in case he admitted her, but as it turns out, we didn't need to because there had never been any plan to put Bethany in the hospital at all.

Dr. K's impression of his & Dr. S's conversation yesterday afternoon (when Dr. S called to tell him she wanted Bethany directly admitted to the hospital to have multiple specialist consultations) was that *I* was the one who wanted Bethany admitted to the hospital because I was freaking out thinking Bethany had cancer. Apparently, however Dr. S explained things to him left Dr. K thinking the hospital admit & specialist consultations was MY idea. He also had been led to believe that I was insisting that he see Bethany right away, which is why he

had agreed to skip his lunch hour & see Bethany the following day.... to appease me, the freaking-out mother, and to chill me out.

Nice. That does explain why, when the rheumatologist's nurse called on Tuesday afternoon, she had said, "Dr. S told Dr. K that you wanted him to see Bethany tomorrow, so he said that he can fit her in at noon. Can you be here then?" I had set her straight, telling her what I had been told before I left Dr. S's office, which was that it was Dr. K who had told Dr. S that I needed to bring Bethany up the next day. Turns out I owed the nurse an apology because her version of events was correct, not mine.

After hearing Dr. K share what he'd been told, I asked him if I had EVER struck him as a hysterical parent. He denied that, saying that actually, I was very level-headed. I asked if it appeared to him that I was freaking out thinking that Bethany has cancer. He said, "No." I explained that I had emailed Dr. S and told her that I felt we were missing something since she was not responding to the increased steroids & her Actemra infusion hadn't resolved her symptoms, & I had written that I had a concern that she might have leukemia. In my email, I had explained why I thought it was a possibility & I had specifically stated that I was not looking for a problem that isn't there, but I was trying to figure out what her symptoms could be a sign of. I ended my email saying that I would be happy to take Bethany to see Dr. K whenever he & Dr. S felt it would be reasonable & appropriate since Dr. S had said she thought Bethany was overdue for seeing him. I asked Dr. K if he wanted to see the email exchange Dr. S & I had, because her response had specifically said that maybe I should plan on going to Portland to see Dr. K & stay a few days so Bethany could see heme-onc & ID (infectious disease), as well. I had my iPod in my hand & was ready to pull up the email because honestly, I was very upset by how things had been misrepresented to Dr. K. I work very hard to establish honest, trusting relationships with my kids' specialists & when that gets threatened, I take it seriously. Dr. K told me he did not need to see anything because he believed me. He said we've always been candid with each other & he was very sorry if what he had said caused a problem for me with our pediatrician. I assured him it was fine & then took a deep breath and said, essentially, that regardless of the circumstances that had brought us to Portland, I was here with my little girl, so let's just go on & see if we can get Bethany figured out.

Bethany had been doing her usual fussing & intermittent crying when Dr. K first walked in the door, and when he held out his hands to hold her, she had cried & fussed at him at first, too. He asked me if this was how she was 24x7 & I said, "Basically, yeah." Within minutes, though, Bethany had stopped crying & after about 10 more minutes went by,

she began smiling & interacting with Dr. K. He grinned at me & I laughed & said, "It figures!" It reminded me of how your car can be making a weird noise & as soon as you take it to the mechanic, it stops making the noise. LOL Bethany spent the next hour being happy & showing off her flexibility & how well she can move. Dr. K told me that he knows she's got something else going on, but from his specialty's point of view (rheumatology), Bethany looks fantastic! He has never seen her pull to a stand or scoot around on her booty or use her hands to flip at paper or anything else because she's always been super-sick when he's seen her in the past, so it was really great for him to have the opportunity to see her when she's NOT having any arthritis problem.

He & I talked back & forth about what could be the cause of Bethany's fevers, rash & increasing irritability. We did discuss leukemia & he said that yes, it's possible Bethany has cancer, but at this time, he does not think it is probable. We talked about a lot of things, but ultimately, what Dr. K thinks is that the rash is JIA-related & a side effect of the Actemra, and that it may have an autonomic factor, as well, since it gets worse when Bethany cries or gets agitated. He is unsure about the low-grade fevers, saying they could be a sign that her immune system is trying to launch a response against something or they could be JIA-related & a side effect of the Actemra. After hashing it out for awhile, we agreed that one of three things is going to happen: the fevers will go away on their own, they'll stay the same, or they'll get worse. If they go away, great. If they stay the same, we'll assume it's an autonomic dysfunction & if they get worse, then something will eventually "declare itself" in her labs and that will give the doctors direction to know what to do. So, for now, we're going to give it time & see what happens. As for the irritability, Dr. K thinks it's associated with her reduction in steroid dose. He agreed that she is having pain & prescribed tylenol with codeine to see if that will help, then said that when Bethany is admitted for her first surgery, we'll get palliative care/pain management involved with her care (that'll be Dr. T, Joshua's doc), and then Dr. T can be involved every time Bethany is in the hospital.

Dr. K is setting up a referral for Bethany to see Dr. C, the ENT (we know him ~ he did Adam's nose surgeries), so she can have her adenoids removed & tubes put in her ears soon by someone who won't be stressed-out by her. He is also referring her to a general surgeon to schedule her surgery to place a port (central catheter in her chest). Dr. K said I should have no trouble getting both surgeries scheduled for the same time and that it is totally safe to do all 3 procedures before Bethany is fully weaned from steroids.

Speaking of steroids, we agreed that we need to get her off of them. Period. Dr. K designed a weaning schedule that will have Bethany off Prednisolone in a month. Every 5-7 days, I will lower her dosage. He said she'll definitely be grumpy, but we agreed that getting her to where she can have her heart repaired & spinal cord detethered is the highest priority, so our family will deal with a grouchy Bethie in order to reach the longer-term goal.

It was a productive appointment & I am glad we went. Dr. K instructed me to call his nurse any time I have a question about Bethany's labs or any other concern regarding her arthritis & said that he would call me back, even if it's in the evening, to discuss what is going on. He thinks that by speaking with me directly & more frequently, we can avoid having a repeat of the misunderstanding that occurred with Dr. S. I told him that would be great. It is always difficult to have snafus in communication with doctors who you've come to trust & respect.


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Written May 2, 2012 2:18am

Bethany has not gotten any better despite her steroid dose being quadrupled, having her Actemra infusion last Wednesday & being on Augmentin for the infection in her g-tube stoma. She is still running low-grade fevers, has a rash on her face, lower back, arms & legs, is very irritable, is grinding her teeth so much her molars are now at her gumline & her canines are almost to her gumline ~ she grinds when she is hurting ~ and we cannot figure out what is causing all of these symptoms.

Her labs continue to show absolutely no sign of any arthritis problem. Her inflammation markers are perfect. Her labs show no sign of infection. Everything is essentially great on paper. Unfortunately, the clinical picture is much different.

Dr. S, our pediatrician, saw Bethany today & told me she wanted to get Bethie directly admitted to Legacy Emanuel to be seen by hematology-oncology (and have a bone marrow biopsy), rheumatology, dermatology, ENT (since the ENT down here is too freaked out by Bethany to operate on her & she still needs her adenoids out & tubes

put in her ears), infectious disease, pain management & palliative care (same doc), and neurosurgery (she wonders if some of Bethany's pain is due to her tethered cord/syrinx). I was SOOOOOO relieved, thinking that FINALLY something is going to get moving & we might be closer to getting answers for what is going on with Bethany. Then Dr. S left the room to call Dr. K, the rheumatologist, to set up the direct hospital admit.

She came back about 15 minutes later saying that Dr. K said 'no' to the admit (we would need a receiving doctor to accept Bethany on his/her service in order to do the direct admit). He said that her labs look fine, she doesn't have leukemia, and he would see her in the office for an appointment tomorrow at noon. When Dr. S asked him about palliative care/pain management, he told her, "Oh, that's tricky, because she'd have to be admitted to be seen by Dr. T." (yes, that would be partially WHY Dr. S wanted her admitted! *** Bang head ***). He doesn't know what is wrong with Bethany, but apparently feels confident in saying what isn't wrong with her from 250 miles away. I am shocked that he dismissed the recommendations of a doctor who has seen Bethany repeatedly over the past 2 weeks of her having these symptoms, but hopefully that means he knows exactly what is going on (though, if that's the case, why wouldn't he have told the pediatrician?). I guess we'll find out tomorrow when we drive to Portland for the appointment at noon. I was told to pack to stay in case Dr. K does wind up admitting Bethany after all, so that is what I'm prepared for. I am praying that whatever decision he makes will lead to our gaining definitive answers about what is happening with my little girl. We really need to get this figured out soon!


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Written Apr 24, 2012 2:03pm

Bethany was back at the ped's office on Sunday & Monday for more shots of Rocephin & to continue monitoring how she's doing. How IS she doing? Lousy.

Okay, maybe that's not entirely accurate. Her sinus infection seems to be cleared up. When she sneezes, she has only white/clear discharge, and she doesn't have anything running out of her nose any other time.

That is a good thing. The swelling in her hands & feet has decreased. That's another good thing. Her rash is still present & has spread down the outside of her left leg in spots, she is still running low-grade fevers every day, she is still in a foul mood 98% of the day, and she has thrown up a couple of times. Those are all lousy things.

Her lab cultures showed nothing in her blood or urine, which is great, but she had some bugs in her g-tube gunk (alpha hemolytic strep, gram positive cocci, gram negative rods). She will be starting Augmentin for 7 days to try and clear it up. Tomorrow, we'll get the final sensitivities for the cultures & I won't be surprised if her antibiotic gets changed again, but for today, we're using a broad-spectrum antibiotic.

She'll have her Actemra infusion tomorrow, which I anticipate will either help knock down this arthritis flare or make things substantially worse (I think it'll be helpful, though). I am hoping Bethany will be feeling a lot better by the end of the week.


I hate when I get behind in updating here because by the time I do get around to writing, there's so much that has happened that I don't know what I should say! I'll just go with the abbreviated version of things that happened this week. :)

Bethany was supposed to have surgery to remove her adenoids & place tubes in her ears on Monday. That's been cancelled. I took her to her pre-op appointment at the hospital only to find they had not scheduled either her pre-op or the surgery because her ENT had decided she wanted written consents saying Bethany could have surgery from her rheumatologist & cardiologist. Then anesthesiology decided they didn't want to risk putting Bethany under because of her heart defect. I guess it didn't matter to them that the cardiologist said it would be fine. What does he know, anyway, right? (yes, I'm being facetious) So... because it turned into such a hassle, we're waiting until after she's had her heart surgery, spinal cord surgery & has had a port placed. THEN we'll deal with the ENT issues.

On Wednesday, Bethany developed a rash that looked very JIA-ish in nature. Her labs looked good, though (I was told), so no one took my concern about the rash seriously. I mentioned at the ped's office that she was much more irritable & had begun some self-injurious behavior. It was blown off. "But she's grinding her teeth almost nonstop & that is always a sign of pain for her." "Kate, her labs look good, so don't worry." The only thing the ped's nurse said the ped was going to do

was call the rheumatologist to ask him if we need to do anything different this upcoming Wednesday (infusion day) since Bethany's triglycerides had had a "slight increase" from 112 (normal) to 203 (high). I questioned how having any lab value almost double in 7 days could be considered a "slight increase" and was told only, "That's what Dr. S said." Truthfully, I drove home feeling frustrated. I felt that no one was looking at Bethany as a human being... they were focusing on her labs & ignoring how she looked, clinically. Megan (my oldest daughter) shared my frustration, saying, "Mom, she could probably die & the doctors would scratch their heads in confusion, saying, 'But her labs looked great!'"

By Friday night, Bethany's hands, feet & ankles were swollen. She had a low-grade fever, green snot was coming out of her nose & from around her g-tube, she was throwing up part of every feed, her rash was worse & she was so miserable she did not want to be touched. I emailed our pediatrician, asking if she was going to be on-call this weekend, explaining what was going on with B & I sent a picture for her to see.

Saturday morning (today), I received an email response from Dr. S telling me to take Bethany to the office to be seen & not to delay going. I took her in & she was seen by a nurse practitioner who, thank you God, LISTENED to what I was saying. It was obvious Bethany was sick, too. She looked horrible. The NP called the on-call doctor to come check Bethany out. The MD called the rheumatologist, who agreed with the NP & MD's diagnosis of sinus infection as a side effect of Actemra that triggered a flare of her arthritis. The question was whether the infection had worked its way into her system since she had green goop oozing from around her g-tube, as well. The goop was cultured & sent to the lab to see what bug(s) grow out, then Bethany was cathed & urine was sent to the lab to be cultured, as well. We went across the street to the hospital so labs could be drawn & then we came back to the doctor's office so Bethany could receive Rocephin (via injection) in her leg.

Her lab results showed multiple abnormalities, some of which could indicate a serious bacterial infection (or worse). Because of that, Bethany will return to the ped's tomorrow for another set of labs & more Rocephin. If anything grows out of the cultures (urine, blood or green gunk) that isn't covered by Rocephin and/or can't be treated with oral antibiotics, we'll have to admit Bethany to the hospital for treatment. Obviously, I am hoping to avoid that scenario.

The biggest disappointment to me in all of this is that the rheumatologist quadrupled the dose of steroids Bethany is on. We had

her *almost* to the point of being weaned completely off Prednisolone & to have her need to go back up in dosage, even for a short time to control this arthritis flare, feels like a huge step backward. I can't help but wonder how we are going to get her off the steroids AND keep her infection-free long enough to make it possible for her to have all of the surgeries that she needs. Right now, it doesn't look very encouraging for my Bethiebean.


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Written Apr 12, 2012 12:30am

Today was an insanely-long day. Joshua had an MRI at 6:15am in Portland. Afterward, we drove home, where I dropped Joshua off & picked up Bethany in a 5-minute pit stop, then headed to the hospital 40 miles away for her Actemra infusion.

Since Bethany has no PICC (we're going to try to use peripheral IVs for her infusions until we can get her a central line ~ a port ~ during her open-heart surgery), phlebotomy was called over to draw her labs (she's a very hard stick). Then we waited for the results to come back. When they did, and everything was deemed by the pediatrician to look good, pharmacy was called to mix the Actemra & then the infusion was given. We were at the hospital for "just" 5.5 hours tonight. Still, it was a loooong 5.5 hours since I had to hold/wrestle with Bethany for most of it. She wanted to pull out her IV & I had to keep her from doing so. To say she was unhappy would be putting it mildly, but she survived it & was fine by the time we got home.

She is still grumpier than usual & she's grinding her teeth all the time, but her labs look good, so they tell me she's fine. Since I don't know what Bethany's default personality is (she's been flaring or on high-dose steroids ever since we brought her from from Ukraine), I don't know if she's just naturally a grouchy child. It could be the autism affecting things, as well. Who knows?

She's 3 weeks away from being weaned off steroids, which is great news. Once she's off them, I'll contact her cardiologist to ask how soon we can schedule her surgery.

Oh, speaking of surgery ~ Bethany will be having her adenoids removed & tubes placed in her ears on April 23rd. The ENT won't need to use stitches (she cauterizes the vessels after removing the adenoids), so she doesn't believe Bethany being on steroids will cause a problem. We're hoping that removing her adenoids will open her airway enough to help reduce her sleep apnea since there's no way Bethany would keep a CPAP on her face at night (the ENT said she wouldn't even bother trying). We're also hoping that the ear tubes will help with the mild hearing loss Bethany has. She doesn't have fluid behind her eardrums, but she does have severe retraction of one eardrum.


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Yesterday, I took Bethany to the infusion clinic at the hospital to get her PICC dressing changed. When the nurse tried to flush the line, blood & saline leaked under the dressing. When the dressing was removed, we were shocked to see an infected-looking ulcer surrounding the insertion site of the PICC. Sigh.

The line was pulled, the ulcer scrubbed (which was as painful as it sounds) & bandaged, and then we were sent over to the lab to get blood drawn. It took only two sticks to get the blood & then we were told that our pediatrician had called (the nurse at infusion clinic had called her to let her know the line had been pulled & why) and she wanted us to come straight to her office. So we left the hospital & went to the ped's. The ulcer was cultured & a prescription for Septra written. It's a broad-spectrum antibiotic that we'll give until we hear back from the lab & learn what specific bug is growing, at which point a different antibiotic may be necessary.

The ped's nurse will be calling Portland to see if they can get Bethany scheduled to get a new PICC placed early next week before her next infusion on Wednesday (the 12th).

Bethany's labs looked good, so we know the infection is superficial at

this time. Our prayer is that it stays superficial & does not get into her bloodstream & that whatever bug is causing the infection responds to oral antibiotics and does not require IV meds to clear it.

Written Mar 28, 2012 6:39pm

I'm at the hospital with Bethany as we wait for the pharmacy to send up her Actemra for her infusion. The nurses couldn't draw blood from her PICC, but instead of using TPA to break up the clot like the infusion nurse did last week, the pediatric nurses opted to go the typical route & used butterfly needles in Bethany's feet to draw the blood for her labs.

Her lab results today were as follows:WBC 18,900 (down from 20,400 last week) RBC 3.66 (down from 3.82 last week)CRP 0.01 (really couldn't be any lower, which is great)sed rate (they forgot to do this one today)ferritin 99 (down from 142 last week) hemoglobin 10.7 (slightly lower than 10.9 last week)hematocrit 34.5 (slightly higher than 34.2 last week)platelets 356,000 (down from 358,000 last week)AST (SGOT) 49 (up from 35 last week)ALT (SGPT) 39 (up from 31 last week)GGT 16 (down from 21 last week)triglycerides 171 (up from 130 two weeks ago)

She's got a rash on her face & has been grouchy for a couple of days, but her pediatrician thinks she is a bit ill, so we're chalking it up to that.

Not much else to report right now. The nurse just started Bethany's infusion, so in another 1.5 hours, we should be walking out of here. I'll be glad to have another Wednesday-at-the-hospital done with.


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Bethany had her PICC dressing changed & labs drawn today. That's a quick in-and-out procedure usually. Today, though, it took over an hour. Her PICC was clotted off, so the nurse used TPA to break up the clot so she could still get blood from it. Due to the dressing being partly off the other day (for who knows how long ~ could have been up to 10 hours ~ Bethany had been in long sleeves all day & I didn't notice until I went to give her a bath before bed), 8cm of the central line worked its way out of her vein, so now the end of her PICC is situated at her shoulder instead of being above her heart where it belongs. There was a green "booger" under the bio-patch where the PICC goes into her arm, but she didn't have a fever, which was good. We're to watch for signs of infection in the line since that green gunk should not have been there.

Her lab results today were as follows:WBC 20,400 (up from 13,900 last week)RBC 3.82 (same as last week)CRP 0.01 (really couldn't be any lower, which is great)sed rate 11 (same as last week)ferritin 142 (down from 405 last week)hemoglobin 10.9 (basically the same as last week's 10.8)hematocrit 34.2 (up from 32.8 last week)platelets 358,000 (up from 253,000 last week)AST (SGOT) 35 (down from 42 last week)ALT (SGPT) 31 (down from 40 last week)GGT 21 (down from 22 last week)triglycerides Not tested this week

There weren't any immature cells in her blood sample this week, which I *think* is a good thing.

I'm not sure what the plan is regarding the PICC line being out of place. I was told our ped was talking with Dr. K to get direction about what to do, but the only message the nurse gave me when she called tonight with Bethany's lab results was that Dr. S would talk to me about it on Friday (when I take Joshua in to see her). So... not a clue. I'm assuming it's not a big deal; otherwise, I would have expected to hear from Dr. S about it.

We have weaned Bethany down another 2mg today. Thus far, she is tolerating the decreases in her Prednisolone. She has bouts of increased irritability and she is grinding her teeth more each day (a sign of pain), but for the most part, she's managing really well, which is a relief since we're waiting to do her heart & spinal cord surgeries until she has been off steroids for a couple of months. At this point, I am beginning to feel encouraged by Bethany's response to Actemra +

Methotrexate and I am hopeful that she will actually be able to get off the Prednisolone completely & we'll be able to finally move forward with getting her the surgeries she needs. Maybe she'll finally start feeling better & will start making some developmental progress (she's currently functioning at the 8-9 month level). I can't wait to see what she is like once her heart is repaired & her spinal cord untethered. :)


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Today is Wednesday, and that means Actemra day. Bethany received her 3rd infusion & tolerated it well.

Her lab results today were:WBC 13,900 (up from 11,400 last week)RBC 3.82 (down from 4.48 last week)CRP 0.04 (absolutely perfect for 3rd week in a row!)sed rate 11 (up from 6 last week)ferritin 405 (down from 786 last week)hemoglobin 10.8 (down from 11.8 last week)hematocrit 32.8 (down from 37.8 last week)platelets 253,000 (down from 299,000 last week)AST (SGOT) 42 (up from 34 last week)ALT (SGPT) 40 (up from 27 last week)SGGT 22 (down from 27 two weeks ago)triglycerides 130 (up from 82 two weeks ago)

I find it interesting that Bethany's hemoglobin has dropped 1 point per week over the past 3 weeks. Her hematocrit has dropped 8 points in two weeks (3 points last week & 5 points this week). Today was the first time she had myelo cells in her blood, too. Whether they are myelocytes or myeloblasts, I don't yet know. If they're myelocytes, that would indicate her bone marrow is kicking out immature cells, which is abnormal, but not necessarily worrisome. If they're myeloblasts, that would be concerning since blasts are indicative of leukemia. Dr. K, Bethany's rheumatologist, left a voicemail saying he would be looking over her labs thoroughly tomorrow & would call me to let me know whether or not there is anything to be concerned about. He said he thinks it's going to be okay, but he wanted to not speak too much

about the labs until he knew whether the myelos referenced were cytes or blasts.

While I hope they are myelocytes, I wouldn't be at all shocked to hear they're myeloblasts simply because we've had two doctors & several oncology nurses tell us that it would be more surprising if Bethany never develops leukemia than if she does. Her body is so messed up at this point that I think almost anything is possible.

Thankfully, Bethie is pretty happy these days, so it's easier to not fret about what is going on inside her when she's smiling on the outside. Still, I'm looking forward to hearing from Dr. K tomorrow. Since he said he thinks things are okay, I am anticipating a good report. I'll update after I hear from him.

UPDATE: Dr. K said the cells are myelocytes & he's not worried about them, as he believes they're a sign that Bethany's bone marrow is trying to rebound (which is good) and is kicking out some immature cells as part of that process (not the norm, but nothing to be concerned about). That's a relief! :)


Because truth is stranger than fiction & Bethany doesn't have ENOUGH going on, a neural tube defect has now joined the party. Her MRI showed scoliosis, a tethered cord & a small syringomyelia at the thoracic level (a cyst of cerebrospinal fluid that collects within the spinal cord, itself, and can destroy the spinal cord). Unfortunately (VERY), we have to wait until she's off steroids to do her heart surgery and then, after she's healed from that, we'll schedule her for neurosurgery. In the meantime, she'll continue having symptoms from the tethered cord & she could wind up with permanent nerve damage to her bladder, bowel & legs (the odds of nerve damage increase the longer a tethered cord is left untreated).

To say Bethany has a mix of conditions that do NOT go well together would be an understatement. For those keeping score, she's got a chromosome abnormality (Down syndrome), an autoimmune disease (s-JIA), an orthopedic abnormality (AAI ), a neurological disorder (autism), a heart defect (AV canal) & now a neural tube defect (tethered cord). I'm not sure what category the syrinx falls into, but there's that + colonized ESBL e-coli in her kidneys & bladder + scoliosis, too. She's the female Joshua, poor baby girl. I was told that when she is next admitted to the hospital (I'm assuming that will be for her heart surgery), it would not be inappropriate to consult with the palliative care doctor. You know, the same one overseeing Joshua. No one is saying Bethany is terminally ill, but with her conglomeration of

conditions, the adults in my family are getting the sense from every professional who knows our little girl that they aren't anticipating that she will have a long life. I can't say I'm surprised, but I can say I'm saddened. Bethany's suffering is different from Joshua's & in some ways, it's worse because she isn't medicated for pain (past Tylenol, which, when she is hurting badly, is as effective as slapping a Band-aid on a hemorrhage) & she can't talk or sign to let us know what she is feeling or what she needs. Her autism makes it difficult for her to tolerate close contact, so trying to cuddle her when she doesn't feel well is not always successful. Sometimes, just being held seems to contribute to her pain.

I can't believe that after giving birth to 4 children with neural tube defects (3 who have had multiple brain/spinal cord surgeries) & choosing to not have any more children because of it, one of the girls we adopted also has an NTD. I know some will say that God knew what He was doing by placing Bethany in our family since we are experienced with NTDs & autism & dealing with rare/weird medical "things". Know what? That may be true, but it doesn't make it easier when the diagnoses for one child keep coming & each one by itself sucks. Put all together, it's overwhelming, even for a mom experienced with this kind of stuff.


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We've weaned Bethany down just 2mg on her Prednisolone & now, 5 days later, she is increasingly irritable & beginning to vomit again, both of which are early signs that a flare is right around the corner. She gets her 3rd Actemra infusion on Wednesday. I'm interested in seeing what her labs look like. We'll wean her down another 2mg on Wednesday, too, so I guess we'll get a really good idea of how well the Actemra is working when we see whether or not it prevents her from flaring.

Here's hoping it's doing what it's meant to. I am really not up for dealing with another flare so soon after the last one. I'm fairly certain Bethany isn't, either.

I haven't heard any results from the MRI she had on Friday. I'll share results when I know anything.


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We're back in Portland. Yesterday, Bethany had her PICC dressing changed & labs drawn, then we headed over to see her rheumatologist. He was pleased with how Bethie is looking & I was pleased because he outlined a plan to get her off steroids. We are going to wean her very slowly since Bethany's little system has shown us repeatedly that weaning is tough for her. I'll reduce her steroid dose by 2mg every week, so if everything goes as planned, she will be done with Prednisolone in 8 weeks. That means that if all goes well, we could be scheduling Bethany's heart surgery in another 4 months or so.

I asked Dr. K what happens if Bethany doesn't tolerate being weaned from the steroids. He said, "Then we would have a problem." We're hopeful, however, that Bethie will do just fine & that in another 2 months, things will be looking much better for her with regards to having her arthritis controlled & being able to look at getting her heart taken care of.

Her lab results yesterday were as follows:WBC 11,400 (down from 37,700 last week)RBC 4.48 (down from 4.78 last week)CRP <1.0 (about the same as last week's 0.61) sed rate 6 (up from 2 last week) ferritin 786 (up from 712 last week) hemoglobin 11.8 (down from 12.7 last week)hematocrit 37.8 (down from 40.8 last week)platelets 299,000 (down from 330,000 last week)AST (SGOT) 34 (same as last week's 34)ALT (SGPT) 27 (up from 26 last week)

Tomorrow, Bethany will have an MRI of her spine to evaluate for an occult spinal dysraphism. Her appointment with the neurosurgeon isn't until July 3rd (yep, 4 months from now), but I am assuming Dr. W will look at the MRI long before 4 months from now. I'll get a copy of the

MRI before leaving tomorrow & if I see anything obviously amiss, I plan to walk upstairs to Dr. W's office & ask her directly to look at the scan. It wouldn't matter if the MRI showed something that would require surgery ~ until Bethany is off steroids, she can't have any kind of operation & her heart would take priority over her spine ~ but I'd like to know whether or not neurosurgery is also in Bethie's future.

After her appointment tomorrow, we'll return home, begin weaning B from steroids, do weekly labs & bi-monthly Actemra infusions & see how things go. I'm glad we finally have a plan of action & am praying things go the way we're hoping they will.

Written Feb 29, 2012 11:50pm

Bethany had her second Actemra infusion today. On infusion day, there is a process we have to go through. The first stop is our pediatrician's office so Dr. S can weigh & examine Bethany & ascertain how she is doing from a JIA standpoint. In other words, how are her joints? How is she feeling? How is she acting? If she is doing all right & is happy, then we don't have to give her Methylprednisolone along with her Actemra. If she is showing signs of her arthritis being problematic, she will get some pulse steroids.

After the exam, we head across the street to the pediatric unit of the hospital. A nurse draws blood from Bethany's PICC & sends it to the lab for stat readings of her WBC, liver function, triglycerides & platelet values & then we head to a room to wait.

Once the lab values come back & it's determined that it's safe for Bethany to get the Actemra, the pharmacy mixes up the medication & sends it over to the peds unit. Then, finally, they hook Bethany up to the IV & she gets her infusion. Afterward, she gets a saline flush & the PICC dressing changed, and then we're out the door!

Today, the process took a little over 4 hours. I'm hopeful that as time goes on & the nurses get more familiar with the process, it will go a little faster (Bethany is the only kiddo receiving Actemra at the hospital, so this is new for everyone).

At Dr. S's office, Bethany weighed 12.62kg (27lb,8oz). Her blood pressure was 114/40. She is doing well enough that we did not feel she needed pulse steroids today. At the hospital, after her labs came back, I asked the nurse, "How do they look?" The response was, "I think they look awful, but apparently they're okay enough to get this infusion."

Today's lab values were as follows:

WBC 37,700 (up from 32,600 last week)RBC 4.78 (down from 5.26 last week)CRP 0.61 (normal, down from 1.95 last week)sed rate 2 (fantastic, down from 19 last week)ferritin 712 (up from 466 last week) hemoglobin 12.7 (down from 14.0 last week; still high)hematocrit 40.8 (down from 44.6 last week; still high)platelets 330,000 (down from 419,000 last week)triglycerides 82 (up from 77 last week)AST (SGOT) 34 (down from 38)ALT (SGPT) 26 (down from 29)SGGT 27 (these last 3 values refer to liver function. Results are all normal)

I know those numbers don't mean a lot to most people & that's fine. I am documenting them for myself so I can compare from week to week how things are trending for my little girl.

Bethany chilled during her infusion. It was late & she hadn't had a nap, so I thought she might actually fall asleep, but it wasn't going to happen. She laid quietly on the bed, however, content to stare at her hand or scratch the blanket she was resting on. When the infusion was complete, the nurse & I noticed a rash had popped up on Bethany's arms & legs. It was identical to the rash that she had in Portland, but if you remember, her rheumatologist hadn't known whether the rash was caused by the steroids, blood or Actemra. This time, with Bethany not receiving blood or steroids, it was patently obvious what caused the rash. Again, she was clinically stable, so we drove home, fed Miss Bethany & put her to bed for the night. It had been a very long day for her!


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Today was a hard day. Without getting too much into the emotional side of things, I'll just share the facts.

Bethany was diagnosed with severe autism (classical ~ meaning they believe she has been this way since she was an infant) & strong

suspicion of either tethered cord or a closed spina bifida (like lipomyelomeningocele ~ what Joshua has). She has decreased movement in her lower extremities, reduced movement & sensation in her feet, muscle wasting in her calves & no reflexes in her lower legs. Those things, along with her chronic constipation & frequent urinary tract infections & deep sacral dimple, concerned the developmental pediatrician & physical therapist enough that they are recommending Bethany get an MRI.

The only news received that was unexpected was that the muscle wasting in her legs is not related to the steroids she's been on for over a year. I thought it was, but the developmental ped said nope, steroids don't cause the degree of muscle wasting that Bethany has. We knew she had a sacral dimple ~ we saw it the first time we changed her diaper after getting Bethany out of the orphanage ~ and our pediatrician had said when we first brought the girls home that one day we'd need to MRI Bethie's back, but it totally got tossed to the back burner & no one had thought more about it since we have been preoccupied with Bethany's other medical stuff. Apparently it's time to get it checked out. I can imagine what our neurosurgeon's expression will be when I walk in with Bethany... she's going to be as blown away as I am that we need to evaluate Bethany for ANYTHING in her field of medicine.

If it turns out we adopted a child with a hidden neural tube defect, I will be floored. I mean, really, what are the odds of a family with 4 kids who have neural tube defects adopting a child who they THINK only has Down syndrome & a repaired heart defect, but actually has those two things plus Atlantoaxial Instability, Autism, systemic Juvenile Idiopathic Arthritis AND a neural tube defect of some sort? I'm wondering if it's about as high as a family having both of their adopted daughters who have Down syndrome getting diagnosed with autism (the dual diagnosis that occurs in about 8% of children with Ds). Yep, Hannah was diagnosed with autism today, too. Both girls are on the severe end of the spectrum. For those keeping count, that means I now have 6 children with some form of autism (and 3 who do not): 2 with Asperger's, 1 with PDD-NOS, 1 with moderate autism & 2 with severe autism. What do I think of that? Suffice to say I've been shaking my head in disbelief for several hours now. It is crazy. I would be inclined to think my life was fiction if I weren't the one living it!

Do I still believe God has a plan and is in control? Absolutely, yes. Do I have any idea what God's plan IS? Absolutely not. Tonight, all I can do is hold on to what I know to be true, which is that God is good, all the time, even when circumstances seem anything but good to me. For now, that will have to be enough.


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Bethany had labs drawn on Monday. She did not receive an infusion of steroids since her WBC's (white blood cells) were so low on Friday. The results of that lab draw showed all of her inflammation markers for arthritis were nice & low, indicating that the massive amount of methylprednisolone she'd gotten over three days in Portland worked to control the flare she'd been having. Her WBC's, though, were up to 32,600. To remind anyone reading this, her WBC's were 26,500 on Wednesday (when she was obviously flaring, as her inflammation markers were sky-high, too), 10,600 on Thursday, then 4,200 on Friday. Three days later, they were higher than they'd been last week & yet all of her other labs indicate she's not having any kind of flare. Courtney, who is Dr. K's nurse, called to tell me today to say that Dr. K is happy with all of Bethany's lab results except her WBC. I asked what was up with that & she admitted they had no idea. It doesn't make sense to have a high WBC count when Actemra typically lowers that lab value, as do steroids. She said Dr. K spoke with the other rheumatologist in the office & they ultimately agreed that they were stymied & had no explanation that they could point to right now, so the general consensus is "wait & see". Bethany will have labs drawn prior to her infusion of Actemra next Wednesday, so we'll see what her little body is doing then & go from there.

Tonight, I am posting from a hotel room across the street from the University of Oregon (go Ducks!). I'm here with Megan, Bethany & Hannah. The two little girls each have 5 hour evaluations tomorrow with the neurodevelopmental team from the Child Development & Rehabilitation Center that is part of Oregon Health & Sciences University. Both girls have the professionals who work with them (& everyone in our family) convinced they are autistic & Hannah also strongly displays symptoms of Reactive Attachment Disorder. Tomorrow's evaluations will (hopefully) provide official diagnoses & some direction for how we'll manage the more-challenging aspects of the girls' conditions.

Bethany seems to be feeling a bit better, which is nice. I told Courtney this afternoon that on a scale of 1 to 10, with 10 being Bethany at her

best & 1 being Bethany at her worst, she's at a 3. There are some improvements, but she's a long way off her baseline happy. Still, at least she is showing some signs of progress. Right now, I'll take every step in the right direction, even if it's a tiny one.


Written Feb 19, 2012 12:09am

Yesterday was a day that mimicked Thursday. I took Bethany to the Day Treatment Unit at the hospital & she had blood taken from her PICC for labs, then received 375mg of Methylprednisolone & then a blood transfusion.

Her WBC had dropped to 4.2 ~ it went from 26,500 to 4,200 in about 36 hours. That was disturbing to everyone & the rheumatologist didn't know why it had happened. Her platelets dropped again, ferritin was up, CRP was down, hemoglobin & hematocrit were up (to be expected after blood transfusion she received on Thursday). What does it all mean? No idea, really. Dr. K said, "She's not going to make this easy for us by responding the way we would expect her to, is she?" That about sums it up. Bethany broke out in hives just before we left the hospital. From what? No one knew. Could have been the Actemra. Could have been the blood. Could have been the steroids. Her coloring was weird (using the words of the oncology nurses) & not what they usually see post-transfusion. The nurse who took care of her said repeatedly that Bethany is a 'conundrum'. Dr. K had no idea what the rash was (it's not the normal kind they typically see, I was told), but said that since Bethany looked stable from a clinical point of view, we could go home & we'll just see what happens.

We drove home today. Bethany will go to our local hospital for further (outpatient) treatment on Monday & more labs. Dr. K will be in contact with our pediatrician after the lab results come back & he will decide the next step. His tentative plan is to give Bethany pulse steroids a couple times this upcoming week & then maybe once more the week after that before she receives her second Actemra infusion. He'll see Bethany in Portland again in 3 weeks.

We're not seeing any great improvements in Bethany yet. She growled quietly to herself & ground her teeth most of the way home. Adam carried her into the house & set her down in the family room where all of the other kids were playing. Bethie did not move from that spot until we moved her to get her ready for bed. She just sat, stared at her

hands or at the ceiling, and actively avoided all interaction from the members of her family. When Megan was getting Bethany fed & in her jammies before bed, she asked me (I was taking care of Hannah), "Does she EVER smile anymore? The only expression I have seen besides blank staring is the grimace of pain. Is she ever happy anymore?" I answered, "No." And that sums up things with Bethany pretty succinctly. She's miserable & unhappy. I am hoping the Actemra will change that because honestly, at this point, her quality of life is worse than Joshua's and that's really lousy for my little girl.


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It was a long day. Oh wait, that's what I said yesterday, isn't it? Well, it applies today, as well. :)

Bethany's labs didn't look great today. Her white blood cell count fell from 26.5 to 10.6, her platelets dropped & her hemoglobin/hematocrit both dropped, as well. The trio of cell lines falling, especially the white cell count tanking the way it did, automatically qualified Bethany for yet another day of steroids & another transfusion tomorrow. It also surprised her rheumatologist, who had anticipated Bethie's labs looking better after the mega-dose of steroids & Actemra infusion yesterday. I guess Bethany isn't one of the kids who shows amazing improvement after their first dose of Actemra. I'm not surprised, though; the literature states clearly that it can take up to 12 weeks of treatment before a decent percentage of kids see significant improvement. As such, I'm not worried that Bethany didn't suddenly feel fantastic after the first infusion. It's too early to form an opinion about this new medication.

When we showed up this morning, Bethany had blood pulled off her PICC for labs & then her infusion of steroids was started. Dr. K came in & chatted with me for a bit, played with Bethany, & said that he expected her labs to show some improvement, so if her hemoglobin went up even half a gram, we would talk about whether she should get that transfusion or not. I agreed & he said he'd check back after the lab results came in.

About 30 minutes later, one of the nurses popped in to tell me, "She's getting the transfusion. Her hemoglobin dropped." Huh. Okay. When Dr. K came in, he went over the lab values that had come back (we were still waiting on WBCs) & told me I'd need to bring Bethany back tomorrow for another pulse of steroids & another transfusion. He said that he wants to "beat this thing down", so that's what all of these steroids & blood are aiming to do.

When Bethany's WBC level came back with such a huge drop, Dr. K & I chatted about what might be going on in her little body. He still thinks this is a bad flare, but admitted that the scarier MAS can't be ruled out. The primary treatment for MAS is huge amounts of steroids, though, so by infusing Bethany with 375mg every day for 3 days in a row, he's hedging his bet & pretty much going to knock out MAS **if** if were trying to develop. I agree with his approach. I'd rather error on the side of caution where Bethany is concerned, which is what he told me he wants to do, too, so we're on the same page (always a good thing).

If Bethiebean's labs look worse rather than better tomorrow, she'll get admitted. For obvious reasons, I am praying that her labs start moving in the right direction by tomorrow morning so we can avoid a hospitalization.

Bethany had some intense "roid rage" today, followed by a reaction to the steroids that caused her eyes to swell & redden & made her super-itchy. Some IV benedryl eased her itchiness & basically knocked her out, which was a good thing since she was so obviously tired from her long day. She's currently being cuddled in her biggest brother's arms & is snoozing, which I'm thankful for. After holding her for hours while she raged and fussed, I was happy to get a break so I could hug Joshua & spend some time cuddling & chatting with him. He is holding steady while we are here taking care of Bethany, which is a HUGE blessing!

Thank you to everyone who is praying for my Bean. The support & encouragement helps me a lot.


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It has been a long day.

Bethany & I were at the hospital at 9:45am. She went to the fluoroscopic lab to get her PICC placed, a process that took about an hour & went smoothly. When I got back to peds sedation to see my girl, she was shaking & the nurses asked me if I knew why. I promptly responded, "She's in pain. She shakes when she hurts." The anesthesiologist thought that was unnecessary & ordered fentanyl. Bethie was given the pain med & within 15 minutes, she had relaxed & was dozing. Her oxygen saturation level dropped & she was given some blow-by O2, but overall her recovery was uneventful & once she was feeling better, we transported her upstairs to the day treatment unit for her Actemra infusion.

Prior to the infusion, the rheumatologist, Dr. K, came in to tell me that with Bethany's hemoglobin being at 5 (normal range for her age is 11-14) & her hematocrit being just 20 (normal would be 35+), she was in need of a blood transfusion. After some discussion, we agreed that doing the transfusion tomorrow morning would be better so that if she had any reaction to the Actemra, we would know it was the medication & not the blood she was reacting to. Bethany's white blood cell count is at 26,000 (elevated), her platelets are 484,000, which is very elevated, but in light of her other numbers, the rheumatologist is concerned because he said her platelets should be a lot higher. Her albumin is low, and a few other lab values are wonky, too, so Dr. K ordered a 'pulse' bolus of high-dose steroids (methylprednisolone) to be given before the infusion of Actemra (which was given at 12mg/kg ~ the typical starting dose is 4 mg/kg, but Dr. K felt the higher dose was appropriate), plus he ordered more labs. He told me his concern was that Bethany was developing Macrophage Activation Syndrome & said that if her labs don't look better tomorrow after the huge dose of steroids he was giving her (375mg ~ for comparison purposes, Bethie's daily dose, which is "moderate" for her size, is 12mg), he would have to admit her. Then he left to go do some procedures downstairs with other patients & the nurse came in to pull labs off the PICC & begin the infusions.

When Dr. K returned, Bethany was crawling out of her skin, writhing around on the bed & fussing. The nurse had stopped the infusion for a little bit to give her a break, but started it after Dr. K came in. When he walked in, the first thing out of his mouth was, "Her CRP is 299." CRP is c-reactive protein, an inflammation marker, & the lab value should be below 10. 299 is crazy high ~ not record-setting, but significant (that's what Dr. K said). Her ESR (sed rate ~ another inflammation marker), which should be below 1, was through the roof at greater than 100. Dr. K looked at Bethany, then told me she is really sick. We already knew

that, though, so this was just confirmation.

Tomorrow, we'll return to the hospital so Bethany can receive another pulse dose of steroids (another 375mg), more labs & a transfusion. The infusion takes 45 minutes, the saline flush afterward takes 5 minutes, & then a transfusion takes 4 hours. We won't be driving home tomorrow as planned. As long as Bethany doesn't get admitted, we'll go home on Friday. And as long as her labs don't look any worse (and with the huge dose of steroids she got today, they *shouldn't*), she shouldn't need to get admitted. Dr. K said that if Bethie isn't admitted, then he wants her to get more pulse steroids at our local hospital next week ~ two infusions in 3 days (ie: Monday & Wednesday) ~ then another infusion of steroids the following Monday & then her 2nd Actemra infusion on Wednesday). He also needs to start seeing Bethany more frequently... like every 3-4 weeks... so we'll be making the drive a lot more often.

Bethany's oxygen saturation level has been hanging out between 86-91%, which is most likely the result of having such a low hematocrit (not enough red blood cells in her veins right now to transport oxygen). I anticipate that improving after she gets transfused. Maybe she'll get some color in her face, too!

It was a long day, but Bethany was a little trooper & handled everything as well as could be expected for a little girl. I'm so proud of her and so sad for her, that she has had to deal with so much pain in her life.


I got a call from the rheumatologist's nurse this afternoon ~ our insurance company approved the use of Actemra for Bethany. We will return to Portland on February 15th to get her PICC line & to receive her first infusion. We'll return home the following day.

The next prayer request would be that the medication works & enables us to begin weaning Bethany off steroids. I don't really know at what point weaning could potentially begin; I'll ask on the 15th. I just hope & pray it will be soon.


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Today's appointment with the cardiologist left me feeling truly dejected. He was a nice guy ~ infinitely more personable than our usual cardiologist ~ and he impressed me at the beginning of the appointment by sharing that he had called the rheumatologist prior to our arrival to discuss the unique situation that we have in Bethany. That was a point in his favor, for sure, & I appreciated that he already had an understanding of what was going on with my daughter before he ever met her.

He had me bring Bethany into the echo room & lay her on the table. Bethany never fights any kind of medical procedure, so she lay still & quiet for the duration of the echo, which was about 45 minutes long. During the exam, the cardiologist would quietly speak to the tech, asking her to do different things & focus on different areas. They spoke low enough that I could not really understand anything being said, but then, after repeatedly requesting one view that the tech was struggling to get, the cardio raised his voice enough that I clearly heard, "You have to hold the angle! Everything is leaking in there!" Then he told her to let him do it, himself, and they switched places.

Let me tell you, "Everything is leaking in here," is nothing a parent wants to hear with regards to their child's heart. I bit my tongue to not say anything for the remainder of the test.

After the echo was complete, the doctor told me to get Bethany dressed & then he'd come in to discuss the results with me. Five minutes later, we were sitting opposite a table & he was laying out the details: moderate leakage of the mitral valve, mild-to-moderate leakage of the tricuspid valve, mild leakage of the aortic valve, moderate pulmonary hypertension (increased since her last echo), an ASD (hole between the 2 atrium of the heart) and a VSD (hole between the 2 ventricles of the heart). The ASD has gotten a little bigger since her last echo. The VSD is not large. Oh, and "her lungs aren't perfect," which was explained to me as being the result of her pulmonary hypertension causing her lungs to look "fuzzy" during the echo.

Surgery is needed, but with Bethany being steroid-dependent & having her arthritis out-of-control, there is absolutely no way the cardiothoracic surgeon will touch her yet. At this point, her heart defects are not life-threatening. If they were, I was assured the surgeon would do the necessary repairs regardless of Bethany's inability to heal properly. For now, we can wait & try to get her arthritis

under control by using the Actemra infusions & (hopefully) subsequently wean her down on steroids. The cardiologist said they'd be willing to do the surgery if Bethany can be brought down to a low dose of steroids (though they'd anticipate problems with healing and/or slow healing, so she might wind up staying in the hospital for longer), but when I asked what dose that would be, the cardiologist admitted he didn't know yet since they don't have a set protocol for this. He only said it would have to be substantially lower than what she's currently at. Thankfully, her heart CAN be fixed (with the exception of the tricuspid valve; the cardiologist told me there's not much they can do about hers). Prayerfully, we'll be able to get her heart repaired sooner than later.

This news, coming so soon on the heels of the rheumatology appointment yesterday, hit hard. I want to cry ~~ Bethany has needed heart surgery for almost a year & we can't do it because we can't get her arthritis into remission & her off of steroids long enough to make surgery possible. Meanwhile, her heart slowly gets worse & that is negatively affecting her lungs. Her joints are swollen ~ she won't use her hands at all because her fingers are so puffy & painful. Her right ankle is trying to "freeze" into an abnormal position that keeps her toes pointed downward, so I have to gently force her foot into a 90 degree angle & then use her AFO to keep her foot locked in proper position. My maneuvers cause her pain, but I have no choice but to do it.

Sometimes I am so utterly overwhelmed by how much can go wrong inside one little person's body. First Joshua & now Bethany.... it's just so sad. If I force myself to focus on the positive, all I can come up with is that at least there is one treatment option for Bethany. That's better than we have for Joshua. For Bethie, it's all riding on the Actemra infusions, which need to be approved by insurance in order to start. We need this medication to be approved & we need it to work. It's the only medication approved for children with systemic JIA. What we really, really need is for the Actemra to make it possible to wean Bethany off steroids in a fairly-rapid manner without lots of adverse side effects so that we can safely get her heart surgery done.

I feel horrible for her. This is worse in some ways than what Joshua is going through because at least we can control Joshua's pain. And on the flip side, at least Bethany isn't terminally-ill. The sweet dolly, though... she got hit with so much in her little life & it just doesn't make sense to me.

Once again, I am holding on to the things I know to be true ~ God is in control, He has a plan for Bethany's life that is being worked out day

by day & He is good all the time, even in situations that look anything but good to me. Those truths have gotten me through the hard times with Joshua & I believe they'll get me through the hard times now with Bethie. I just wish it weren't this way for my little girl.... after living through hell for almost 3 years in a Ukrainian orphanage, I wanted so much to give her a happier life. It's disappointing that I can't.


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Bethany's arthritis is flaring even on steroids. Her rheumatologist seemed disappointed. She was rashy, swollen joints, painful & had a 101 fever when he saw her today. Our next step is to have Dr. K petition our insurance company to cover Actemra, the only medication approved by the FDA for treatment of children with systemic JIA. He said they'll cover it; he doesn't foresee there being any hassle about it since she's been on steroids & methotrexate for a year without gaining remission, so she obviously needs something different. Once we have approval, Bethany will come back to Portland to get a PICC line & have her first infusion & then we'll do infusions every 2 weeks at our local hospital with our pediatrician overseeing things (in consult with the rheumatologist). If the Actemra helps her, then the rheumatologist said he would want Bethany to have a port placed for permanent central line access. I'm to ask the cardiologist tomorrow if it will be okay to do the PICC & will Bethany be okay to have a port placed down the road, as well.

Meanwhile, cardiology called again & said that the cardiologist won't be available tomorrow, but because we traveled 250 miles for the appointment, they are putting Bethany in with a different doctor, who can do her echo. I have no idea if this guy she'll be seeing is any good & I have no idea if he'll be able to answer my questions regarding being able to do surgery even with Bethany on steroids (rheumatologist said we are "nowhere near" being able to get Bethie off steroids, so cardiologist needs to figure out a Plan B if we're going to get surgery done any time in the foreseeable future). I'm feeling a bit like tomorrow will be a waste of time since we don't get to see "our" cardiologist, but who knows... maybe this other guy will be okay & will provide answers. What I think will happen is that he'll say that I need

to talk to our regular cardiologist since he (our regular guy) knows Bethany better & knows what's going on in conjunction with rheumatology. Still... maybe I'll be pleasantly surprised & he'll be a great guy who wants to be really helpful. I can dream, right?

Oh ~ I forgot to mention, Bethany is 29.1 pounds (13.2 kg) & STILL 35 inches tall. The rheumatologist said that kids with systemic JIA are stunted in their growth & that Bethany really won't grow as long as she's on steroids. Add that to Bethie having Down syndrome (where the average adult female height is 4'9") AND having been profoundly neglected & malnourished in a Ukrainian orphanage for 3 years (thus affecting her height, too) and we don't know if she'll get more than 1-2 inches over 4 feet tall. I guess that since no one ever expects her to be able to walk, at least she won't be super-big, so that'll make it easier to move her from place to place.

Written Nov 1, 2011 2:24am

My poor little Bethiebean is much more ill than we thought. She had repeat labs drawn last Friday to verify that her inflammation markers were coming down & that the steroids were working. Unfortunately, that is not at all what we found. Instead, her labs were worse & were looking suspicious for leukemia. She had more labs drawn today & her labs are worse than Friday's ~ her white blood cell count almost doubled (from 17,000 to 33,000), her sed rate is still above 140 (for comparison purposes, when she was sent to Portland in January, her sed rate was 100. Normal is less than 13), her ferritin is sky high & her CRP is not good, either. Her hemoglobin and hematocrit are very low & her neutrophil count is extremely high. The overall effect is that she no longer looks suspicious for leukemia, which is a really good thing, but she is obviously still sick & getting worse, which is worrisome.

Bethie's rheumatologist is freaked out that she has a blister on her finger that cultured positive for MRSA (it's the third blister she's had pop up unexpectedly in the past week) and he's worried that she may have an infection that we're missing, so tomorrow, I'll be taking her back to the lab to get more blood drawn & to get cathed for another urinalysis. Cultures will be done to see if any bugs grow in either her blood or urine & we will go from there. If she does not start showing signs of improvement, our pediatrician said tonight when she called me that they would airlift Bethany to Portland for treatment.

We've doubled her steroid dose in the hopes that we can get her arthritis back under control. She is SO miserable ~ she's got fevers (Tylenol brings them down to the 100.2 to 100.4 range), chills, swollen joints/hands/feet, she's exhausted & lays around doing nothing when

she's not being held, and she cries any time we move her arms or legs, so diaper changes & clothing changes are painful for her. After 6 days of steroids, I am surprised that she is still so sick. She should be so much better already! It makes me suspect that the rheumatologist may be right & Bethie might have an infection somewhere. Hopefully we'll get some more-definitive answers tomorrow because I am leaving for Portland on Wednesday to take Joshua for his MRI & neurosurgery (only the most-serious brain surgery of his life) and I would REALLY like to know what is going on with my baby girl before I have to go. I hate having to leave when she is so sick, but at this point in time, I have no choice whatsoever. As high a priority as Bethany is to me, Joshua's terminal status & need for pain relief is a smidgeon higher. I hate that I have to "triage" my kids, but again, at this point, I really have no other option.


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Bethany is having a major arthritis flare. Her inflammation markers are WAY up & she is miserable, with fever, shaking, swollen hands, fingers, wrists, ankles, feet & knees. She cries if we move her at all & she throws up some of her feeds. Poor baby girl is hurting terribly and I feel so sad for her.

She's back on Prednisolone to control her flare & Zantac to protect her gut while she's taking the steroids. It will be awhile before she is off of them completely, which means we won't be able to do her heart surgery soon after her brother passes away, as planned (Bethie has to be off steroids for at least 2 months before surgery can be done due to the risk of serious complications). I'm frustrated by this development because it's not in Bethany's best interest to be postponing the surgery & the only reason we have thus far is that I can't be 250 miles away from Joshua for 2 weeks. She was satting at 92% today, whereas she typically has been in the 95-97% range, so that makes me a little nervous, and her hands & feet remain cold despite having a fever of 102+, so that makes me hope her pulmonary hypertension isn't worsening.

I feel sad that Bethany has to be back on steroids. She has become SO

much happier since getting off of them & losing all of the steroid weight she'd gained (she now weighs a healthy-for-her-35-inch-height 24lb,11oz). I had to give her a "pulse" of steroids tonight ~ that's one big dose of steroids to kind of shock her system & hopefully begin knocking down her immune system so it will chill out again. Tomorrow, she'll start getting steroids twice a day. I'll take her in for more labs in a few days to determine if we're getting this flare under control or not. Hopefully we'll see her ferritin, CRP & sed rate lowering. If not, she'll have to be put on a higher dose of steroids until her immune system begins behaving again.

This bites.


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When Charley & I were driving in to see Bethany & Megan at the hospital, my phone rang and I was surprised to hear our pediatrician on the other end. She asked if I was in a place where I could talk, as she had some interesting information regarding my daughter. I told her I was & waited to hear what she had to say.

She had been puzzled about Bethany's infection because her labs today showed tremendous improvement, which really should not have been the case if this were a true pyelonephritis (kidney infection), but would have been expected if she'd had an illness that responded to the injections of Rocephin she'd been given on Tuesday & Wednesday (such as the cold that all the kids had & were still recovering from). Dr. S called Dr. L, the urologist who has taken care of Joshua & has evaluated all of Bethany's prior bladder & kidney tests. During the course of the conversation, it was determined that the colonized e-coli in Bethany's kidney had made its way down her ureters to her bladder, which then resulted in her urinanalysis showing the huge amount of bacteria in her urine sample. The sample only had a couple of white blood cells, though, and with a pyelonephritis, it would have been expected that she'd have a bunch in her urine.

Dr. S told me that we could leave Bethany in the hospital, do the PICC line & give her 2 weeks of antibiotics as a 'just in case' measure, but

since the ESBL e-coli is already very resistant to almost every antibiotic available and each time we give Bethie the big-gun meds to kill off the bug, we run the risk of it growing resistant to the meds, it would be smarter to stop treatment, discharge her from the hospital & let us do a "watch & see" at home. I completely agreed with her, so she let Bethany go. Charley & I picked up the girls & now they're home again.

For the next week or so, we'll watch Bethany closely for any sign of illness. If she gets a fever over 100.4, she'll immediately get cathed & the urine will be sent to the lab and she'll have labs re-drawn. If she shows infection at that point, we'll treat her aggressively with the IV antibiotics that will kill the bug. But if she stays healthy, as Dr. S & Dr. L think she will, then we will have avoided a long hospital stay, a PICC line & (most importantly) giving this colonized bacteria in Bethany's body the opportunity to grow in resistance against the 2 big-gun antibiotics that can still destroy it.

Bethany was THRILLED to be home & everyone was happy to have her back where she belongs. She has bruises in 6 spots on her arms from needle pokes, but other than that, she looks great. I am comfortable with Dr. S's decision to stop treatment & am feeling optimistic that the e-coli isn't going to cause any problems right now.


Got Bethany admitted to the hospital without incident. The final sensitivity report from the lab confirms that the bacteria causing Bethany's kidney infection is, indeed, ESBL e-coli, and it has gotten stronger (more resistant to antibiotics) since her last infection, which is not a good thing. Each infection of this bug increases the chance that it will become resistant to the 2 drugs that can effectively kill it.

Bethany is on both IV antibiotics available to kill the bacteria ~ gentimycin & meropenem. The pharmacist doubled her dose of meropenem because of the strength of the bug. Priority #1 is to kill it, of course, but to do so while keeping it out of her bloodstream. When I left Bethany this afternoon, they were taking her to ultrasound for a scan of her kidneys (Megan is with her).

Megan called after dinner to let me know that they didn't have the results for the ultrasound yet, and at this point, there is no scheduled time for Bethany to get her PICC line tomorrow. Apparently, the anesthesiologist wasn't at the hospital today, so the procedure couldn't get scheduled (don't get me started on the absurdity of that), and rumor has it that there's only a small chance it will get done tomorrow. Instead, they're saying it could be Monday or Tuesday before she can

get the PICC line, which would push discharge to Tuesday or Wednesday.

We move on Friday.

I'm feeling a bit frazzled here & very frustrated that we don't have a nurse who will "go to bat" for Bethany. In Portland, at Emanuel, we would have had today's nurse calling the anesthesiologist's scheduler to explain the situation & WHY it is so imperative that Bethany gets a PICC done tomorrow. In Portland, they would have made it happen. I know this; they moved mountains for my kids (Joshua & Bethany) last January. Anyway, my plan is to call the hospital in the morning & speak to Bethany's nurse and ask her if she will PLEASE try to convince the anesthesiologist to fit Bethany in for the procedure right away so she can come home on Monday, as originally planned.

My little lovey is doing all right. She doesn't like having the IV in her hand (who would?) and has spent a lot of time trying to push the tubing away from herself, but Megan said she had a pretty calm afternoon, so that's good.

I'll be heading to the hospital tomorrow morning to see my girls & will update again after I get home.


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Bethany will be admitted to the hospital tomorrow morning to begin IV antibiotics. The lab results showed a major infection of what is suspected to be e-coli & since Bethany's past 2 kidney infections were a multi-drug-resistant e-coli, we're assuming that the final sensitivities given by the lab tomorrow will reveal that's what we're fighting this time, as well.

The plan is to admit Bethany in the morning, get a peripheral IV going & begin meropenem while we wait for the lab to give final sensitivity report, which is where they identify what antibiotics will kill this bug (ie: what it is sensitive to). The ped is writing orders for Bethany to get a PICC on Friday & then she'll get discharged on Monday. If the

anesthesiologist can't get a PICC line in (they couldn't the last time it was attempted), then Dr. S wrote orders for an IJ central line to be placed (an IV that goes into the jugular vein). Getting discharged for home IV meds with an IJ line isn't generally done, but we'd do it if we needed to.

Bethie is all right. She doesn't feel great, but she's still perky & playing & enjoying being held. We were fortunate to catch this bug while it was just in her urine & kidney. If it had made its way to her bloodstream, I'd be singing a different tune.


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Bethany has a kidney infection. Poor baby girl had to get labs drawn & that took 3 different needle pokes to accomplish. Each poke drew blood, but her veins would blow before the phlebotomist was able to get the full volume needed for the tests that the pediatrician ordered. It was so sad! She also got cathed for a urinanalysis and, once we knew that she had at least a UTI, the nurse gave her a shot of Rocephin.

The labs showed that Bethie's illness does not appear to be related to her arthritis, which is wonderful. We really need to keep her off of steroids so that she won't have any complications with her heart surgery & I didn't want to see her 'set back' by months if she had to start steroids again and then wean off of them, so her not needing them is a huge relief.

We'll find out tomorrow (I think) what bacteria the lab grew out with the urine culture. If it's a drug-resistant e-coli like she's had twice before, it'll require IV antibiotics, but if that happens, I would push for her to get a PICC line so I could give her meds at home. I would not want to leave Bethany in the hospital without me (even though she'd have her big sister with her, so she wouldn't be alone), but I couldn't leave Joshua (http://www.caringbridge.org/visit/theyoungestof7) for 2 weeks, either. Adding in the fact that we're moving in 9 days and, well... the timing of a hospitalization right now would be tough, so I'm

praying that it doesn't happen. I'm praying that whatever bug has invaded Bethany's kidney will be treatable with oral antibiotics and that it stays in her kidney & doesn't get into her blood.

She was 24lb, 11oz today ~ that's a loss from a few months ago, but since she's been throwing up, no one was surprised ~ and she's 35 inches tall. Our ped's nurse calls her a peanut, which is an apt name for Bethie considering she will be 4 years old next month.


Bethany's rheumatologist called to chat. I was supposed to take her & Joshua to Portland for appointments, but with Joshua being terminally ill & unable to travel anymore, I had to cancel the doctor visits. Dr. K wanted to know how Bethany was doing off steroids, though, so he called me.

I told him that Bethany was doing really well, actually. She's lost 5 pounds (that was steroid weight), and her hair is growing & looking so much less brittle and 'straw-like' than before. Her moods have improved & she is happier than she was when on the steroids. She still has many challenges ~ not least of which is her autism ~ but her body is obviously happier when it's not being given Prednisolone. She's had times when we thought she might be beginning to flare, but then things have settled down each time (prayer works). Dr. K was very happy to hear such a positive report.

He told me that he does NOT think Bethany has a second autoimmune disease. He believes that the test showing her genetic marker for a spondyloarthropathy does not mean anything important. As such, we're back to her having "just" S-JRA. That's fine with me since that one can cause enough trouble all by itself. I'm relieved that Bethie doesn't have more going on; that's a blessing.

With regards to Bethany's cardiac situation, we are seeing more blueness in her hands & feet & she tires out more quickly than she used to. I agreed to schedule her surgery once things with Joshua are resolved, so we have that on the 'things to do' list.

From a medical point of view, other than her heart, Bethany is doing all right. Her arthritis appears to be in remission right now & the only medication she receives is a weekly shot of Methotrexate. She & Hannah will be getting their AFOs on Thursday and it's my hope that Bethany spends more time upright once she has a solid base of support. She has such low tone in her trunk, hips & knees that getting

her to a point where she can learn to walk will take a lot of work. I am hopeful, though, that we can achieve that goal sooner than the physical therapist & orthotist anticipate it occurring. Other milestones we are working toward include teaching Bethany to eat solid food & overcoming her extreme aversion to using her hands. I want to be able to send her to preschool next fall (I am not going to attempt to homeschool either her or Hannah) & it would be great if she could get something out of it.

When I schedule her heart surgery, I'll update the blog again, but I don't anticipate having much to say here until then.


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Got a call from B's rheumatologist's nurse, telling me the results of the labs that were drawn last Wednesday during her procedure. Her inflammation markers are a bit better than they were in April, but she still has active disease. The surprising result that came back was a positive result for a genetic marker called HLA-B27. http://en.wikipedia.org/wiki/HLA-B27 This marker is associated with a classification of diseases known as spondyloarthropathies (spon-doe-low-arth-rop-uh-thees) ~ specifically, ankylosing spondylitis, reactive arthritis, inflammatory bowel disease & psoriatic arthritis.

I'm waiting to hear directly from the rheumatologist for more information. What the nurse told me was Dr. K would have specifics for me & would discuss this new finding in detail, but he is certain it is not related to her systemic JRA. I asked if that meant she has a second form of arthritis & she told me, "A spondyloarthropathy, yes." Basically, she's got a second autoimmune disease on top of the systemic juvenile rheumatoid arthritis (on top of Down syndrome, a congenital heart defect, autism & possibly RAD). No wonder she hasn't gone into remission yet.

From what I've read thus far, it appears that spondyloarthropathies are treated the same as s-JRA, so i don't think anything will change as far as what meds Bethany gets. Once I talk to the rheumatologist and get more information, I'll have a better idea what the gameplan is from

here.

Another interesting tidbit from the rheumatologist that I forgot to mention before now is that Bethany's achilles tendons are deviated to the side rather than being straight down like they're supposed to be. He said that both tendons are slanted inwards. What's the significance of that? I have no idea. He didn't know, either, since he's a rheumatologist, not an orthopedist, but it's something to keep in mind.


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We're home after our week up in Portland. To summarize what we learned with regards to Bethany, the rheumatologist injected cortisone into her joints & we've taken her off Prednisolone completely in order to get her into good enough condition to undergo open-heart surgery before the end of the year so the cardiologist can repair her leaking mitral valve & fix the holes in her heart (one in the atrial patch that was placed in Ukraine & the other in her ventricle). He will also try to lower her pulmonary hypertension through some procedure, but I didn't fully grasp what that was & I figured it wasn't something I needed to pursue understanding of until the time got closer to her actually having surgery.

Bethany is almost 30 pounds and still 34 inches tall. Her rheumatologist said she won't grow as long as she's on steroids, so I'm hopeful that once the cortisone & Prednisolone are out of her system, she will be able to finally begin adding some height to her frame.

The decision to take her completely off steroids was one that Dr. K & I discussed in depth. Ultimately, the truth is that juvenile rheumatoid arthritis causes pain every day in a child's life & there is really no way to get rid of it completely. As such, as heartless as it may sound, Bethany will have to learn to live with a certain degree of pain. It is our hope that she will be able to live without daily steroids. It is our further hope that being off steroids will not result in her arthritis flaring up & that whatever pain she has will be tolerable for her. Only time will tell whether or not this goal is possible to achieve, but it is what I'm praying for. It's just not healthy for a person to remain on steroids

forever... it will wreck their body & cause lots of problems. I want what's best for Bethany, which would be to not be on steroids, so it's something we're going to try & see how she does with it. We will continue giving her chemo once each week, along with her other medications.

So far, she seems to be tolerating the change all right, but that could be because she got cortisone injected into her joints & they're not hurting as badly as usual. Again, time will reveal more & her treatment plan will evolve as the need arises. Hopefully, though, she'll be stable enough within 2-3 months to schedule her heart surgery & we can then get that over with. It would be great to get Bethany to a place where she's healthy (or as healthy as she can be) so that we could maybe see more of her personality when it's not clouded by symptoms of her heart condition or arthritis.


The cardiologist appointment didn't go as I had hoped. Bethany needs open-heart surgery to re-do the repair that was done in Ukraine, but her arthritis needs to be under better control first because her being on steroids puts her at risk for a lot of complications, primarily problems with wound healing. Her rheumalologist will try to get the JRA chilled out so the cardiologist can do surgery in a couple months.


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Bethany saw her rheumatologist today. She has been pretty grumpy due to joint pain since her steroids were weaned down. Dr. K seemed disappointed that she has not responded to treatment as had hoped she would & said he felt it was time to try injecting cortisone into Bethany's joints to try & knock the arthritis down that way. So, on Wednesday morning, Bethany will be sedated & will have injections in her ankles, knees, wrists, fingers & possibly her elbows & shoulders (it will depend on how things look to the doctor when he has her sedated & completely relaxed).

She's seeing her cardiologist tomorrow. I don't know if he'll want to schedule a heart cath yet or not, but I'm hoping he says it can wait for

another 6 months. I'm really in no rush to go the heart cath + surgery route with Bethany. We know it's in her future, but hopefully it won't be any time soon.


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Bethany was put back on high-dose prednisolone as a result of her most-recent flare. The rheumatologist hoped to be able to get her symptoms under control at 15mg, but that didn't happen, so Bethie got bumped back up to 30mg, which she stayed at for a week before we began implementing a slow taper. Again. It will take 10 weeks to get her off the steroids, assuming that it ever happens (that she gets off 'em completely). As of today, she is down to 18mg. She stays at each dose for 5 days before we again reduce it by 3mg.

At the high dose of prednisolone, Bethany was really happy. She was bending her wrists & attempting to crawl, even. Now that she's down to 18mg (still a high dose for her size), she's not bending her wrists as easily & she's not trying to crawl anymore. Her mood is not as pleasant, either.

She's got a raging yeast infection that is contributing to her misery. We've been battling yeast since her second hospitalization, but raising her steroid dose seemed to kick the yeast into high gear & now we're losing the battle. Yes, Bethany receives probiotics every day (a lot of 'em), but she also has to take a prophylactic antibiotic to ward off more UTI infections from the drug-resistant e-coli, so it's a never-ending fight. Charley & Megan have been using nystatin, but Charley will be taking Bethie in to see our pediatrician sometime this week to see what else we could try to finally get rid of this persistent infection.

I feel so sorry for my daughter. Chronic pain from arthritis is a horrible thing to live with, and being a little girl who doesn't understand why she hurts must add to her misery. I wonder if she thinks the world is a lousy place, full of pain. I wish we could take away her hurt. I wish we could get control of the arthritis and enable her to enjoy life without daily pain. It makes me sad to see her not feeling well.

She saw the ophthalmologist a few weeks ago and was diagnosed with nearsightedness. Hers is not too bad ~ just -2.00 ~ but Hannah's vision is -10.50! Both girls will be getting glasses soon. Both Bethany & Hannah will be getting AFOs, as well, and I anticipate those helping a lot with their attempts to stand. Their adaptive tandem wheelchair (ie: adaptive double stroller) will be delivered this week & I am SO excited to have it so that we can finally take the girls for walks! Bethany's stander will be delivered around the middle of May and that is another piece of equipment that I am looking forward to receiving. Having her stand every day should help strengthen her trunk & legs & will give her a new perspective to view the world from.

Bethie's g-tube feeds are working well & her speech therapist is working with us to teach Bethany how to eat solid foods. She can coordinate 6-8 good swallows of soft foods like pudding or yogurt before she gets tired & loses coordination, which results in more food coming out than going down her throat, but we are happy with her progress. Considering everything she has working against her, any steps forward are big ones for our girl.

Written Apr 6, 2011 3:33am

Nothing grew out in the first 24 hours of blood & urine cultures that were taken yesterday, which means there's no infection (thankfully) and Bethany's symptoms are "just" an arthritis flare. Believe me, I'm not minimizing the flare ~ it's horrible & Bethany is swollen & stiff & rashy & feverish & hurting badly & miserable ~ but I'm grateful that she won't need to be admitted to the hospital for more IV antibiotics. With Joshua doing so poorly (see http://www.caringbridge.org/visit/theyoungestof7), I really could not handle Bethany needing to be hospitalized right now, so it's a huge relief that she can be treated this time with "just" an increase in her steroids (and possibly another medication ~ I'll hear from the doctor tomorrow about treatment specifics) to get things under control again.


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Ugh ugh ugh ~ yesterday, Bethany developed a high fever (103.7) that didn't respond well to tylenol, only coming down to 103.1 an hour later. Her hands & fingers got puffy, as did her feet & toes, and her face developed the rash that we've come to recognize as being associated with her s-JRA. I vacillated between wondering if she was getting sick or if this was a flare of her arthritis. While her symptoms seemed to point to the s-JRA, illness can also cause a flare and we've had two kids who had mild colds this past week, so it wasn't out of the realm of possibility that she was "just" sick.

This morning, she woke up with a fever of 102.1 and stiff wrists, ankles, fingers, toes, knees & elbows. Her hands, feet, fingers & toes were even more swollen & it was obvious she was hurting. We gave her more tylenol (she can't take ibuprofen because she already gets prednisolone & methotrexate) & that helped her to get a bit more comfortable even though she still did not want anything flexed. She was in a fussy, grouchy mood today, but that is the norm for weekends since Bethie gets her chemo shot on Fridays & is always in a foul mood for 48-72 hours afterward. Having a friend who also has RA explain how methotrexate makes *her* feel makes me feel extreme sympathy for my daughter (and my friend). I don't blame Bethany for being a grump... she has every reason to be unhappy.

As of tonight, I'm convinced this is an arthritis flare. She keeps having high fevers that come & go and if we are late with her tylenol dose, she cries with real tears & that NEVER happens unless she is really hurting. You can tell she is in pain, especially when she has to be moved, and getting her dressed/undressed is agony for her. Poor baby girl is miserable & we feel so sad for her. I think one of the hardest things to deal with, for me, is having a child who is hurting and knowing I can't remove the source of their pain.

I haven't yet called the rheumatologist or pediatrician since it's the weekend, but I'll be calling first thing Monday morning. I assume, based on past experience, that the rheumatologist will want labs drawn to check Bethany's inflammation markers (CRP & sed rate), ferritin & fibrinogen levels, white blood cell count, platelets, liver & kidney function. The question will be whether she has some kind of infection that caused the flare (they can occur "just because"). I am also assuming I'll have to take B to the pediatrician's to get her looked at so the ped can report to the rheumatologist. I think that as long as she doesn't have an infection (which would need to be treated, of course), all we can do is wait out the flare & keep her as comfortable as possible.

I do wonder if this flare was caused by having her steroid dose lowered

too far (we've been gradually weaning her dose by 1.5mg/day each week since mid-January and she's now at just 1.5mg per day). I hope that is not the case because the goal is to get Bethany off steroids completely since long-term steroid use is horrible for the body. The reason I wonder about the decreased steroid dose possibly being the culprit is that when we were in Portland last week, I mentioned to the rheumatologist that Bethany's hands & feet looked a bit puffy to me & she was starting to grind her teeth more & be more grouchy than usual (signs of pain for B). Dr. K checked Bethie out very thoroughly and since she didn't have any problem with flexing her fingers, wrists, toes, ankles or any other joint, he said he thought she was okay. I agreed with him ~ I mean, he's the expert and she *was* able to move without it hurting while she was in his office and she was playful & happy while there, too, so I figured I was concerned about nothing (and maybe I was). I didn't think he was wrong. I still don't think he was wrong (just want to make that clear). What I *wonder* is if Bethany was on the edge of being "okay" with the dose of steroids she was on, so when we reduced it from 3mg/day to 1.5mg/day, it wasn't enough to keep her arthritis symptoms in check & that's why we are now seeing the flare-up a week later. I honestly don't know if that's a plausible explanation or not, but I plan to ask Dr. K when I talk to him on Monday.

I feel a bit discouraged about Bethany's health. Will she *ever* feel better again like she was pre-mid-December when she first got sick & if so, will it last for more than a few weeks? I really do not understand what God has planned for this little girl. Down syndrome + profound autism + systemic juvenile rheumatoid arthritis + a congenital heart condition (plus a few other, more minor, diagnoses such as failure to thrive & atlantoaxial instability) is a really lousy combination of conditions, especially for a little girl who spent almost three years in an orphanage, unloved & given minimal attention. She hurts & doesn't understand why & can't communicate any way other than grouching, growling, screeching & grinding her teeth. She has a family that she likes as long as it's on her terms but otherwise she can't really stand to be around them because she has attachment problems & her autism drives her to remain solitary & locked in her own world. As frustrating as it is for us (her family), I can't say I blame her ~ she has so many things "wrong" with her that it's got to be a huge fight for her to do anything remotely interactive. It's so sad, and quite honestly, it makes me really angry that the country she was born in blew her off as a waste of space & someone without value, opting to drug her to oblivion in a crib, alone & barely-cared for. I feel certain that had Bethany been given appropriate medical care from day 1, she would be a much-different little girl than she is today. We'll continue doing everything we can to help her, but at times like this, when I know she is hurting & she glares in our direction with sparks shooting from her eyes

(probably because she wonders why we're not DOING something to help her & she doesn't understand that we are actually trying to do just that through the procedures & medications that contribute to her feeling so lousy), I do wonder if it's ever going to make a difference.


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Bethany's procedure to change her PEG tube to a mic-key button went without incident. The endoscopy showed she has some ulcers in her duodenum, most likely caused by the prednisolone, so we're doubling her dose of omeprazole and that should take care of it.

I'm glad we have a button now to feed her through rather than the broken PEG tube. Having no more leaks is great. Bethany hates that we can feed her against her will, but I'm thrilled that it's possible & she can't prevent it from happening anymore (she was able to push formula out of her belly by tightening her stomach muscles & grunting/growling at us when we were gravity feeding her with the PEG).

She is still 34" and weighs 12kg (26.4lbs). Still wearing a size 2 (infant ~ barely past newborn size) shoe, but is up to 3T clothes to accommodate her chunky belly.


Joshua, Bethany, Adam & I are up in Portland again for three days of appointments for the two little ones. Bethany saw the rheumatologist today & she'll be having her PEG tube changed over to a mic-key button tomorrow morning. Joshua will see the neurosurgeon tomorrow afternoon & then I have an appointment with the geneticist to go over the results of Joshua's genetics testing that was done back in January.

Her appointment with the rheumatologist went well. He was pleased with how she is responding to treatment thus far, which was great news to hear. The plan is to continue tapering her steroid dose each week with the goal of eventually getting her off them completely. She'll continue to get her chemo med once a week, along with her other

medications, and we'll see how she does. She'll return to see the doctor in 4 months unless something happens that she needs to be seen sooner (no one is anticipating that, though).


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Written Mar 12, 2011 10:59am

Heading out with Emily & Sarah for what will hopefully be the last trip to the hospital in a very long time since we are going to bring our Megan & Bethie HOME!!!!!

I am SO happy that this hospitalization is OVER!!! Bethany will be taking Nitrofurantoin for the next 2 months in the hopes it will keep any more UTIs at bay. Other than that, her medication schedule stays the same. She is down to 6mg of Prednisolone per day now, which is good ~ she tapers 1.5mg per week ~ & our prayer is that she will be able to get off steroids completely without having any flare of her JRA symptoms.

Anyhow, I've got to go, but I wanted to let anyone reading this know that Bethany is on her way home again! Yippee!!!!!


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Bethany & Mom (weird angle since a much-shorter-than-me child was taking the picture)

Bethany is doing great as she finishes up this round of IV antibiotics. She'll be discharged on Saturday and I. CAN. NOT. WAIT to get her out of the hospital!

This past week has had its share of frustrations equal to last week's, but I didn't have the energy to share it all publicly as it was occurring. In a nutshell, her g-tube got broken, the nurses forgot they were supposed to be treating Bethany's yeast infection so that flared up again, she got overfed 20% of her daily calories for several days (until Megan realized what they were doing & got it stopped), which led to the development of symptoms that then led to her having to be evaluated (via chest x-ray & echocardiogram) for congestive heart failure, & she's been gaining weight very rapidly & no one really knows why or what to do about it (she gained 2 pounds in 7 days, then half a pound in 1 day, then she maintained the next day, then gained another half-pound the next day ~ she entered the hospital weighing 22lb,15oz and now weighs 25lb,14oz, which is A LOT of weight to gain in less than 2 weeks). It's all in her face & belly, but her legs & arms are still stick-thin. The weight gain is most likely due to the steroids she is still on (though they've been weaned every week since she left Portland 5 weeks ago), but we were told the reason steroids makes a person gain weight is that the medication increases the appetite, so the person eats more. Bethany hasn't had an increase in calories (except for the days she was overfed, but even that was just 300 calories per day) & she definitely hasn't consumed an extra 10,500 calories (3 pounds) during her hospitalization, so the fast weight gain is perplexing. I requested a visit from the nutritionist & she came and determined Bethany needs 1200 calories per day, which is exactly what she's been getting for the past 5 weeks since she got her g-tube. So... ??? .... like I said, it's perplexing. The weight gain would be cute if she had gained it all over, but since it's a 'cushingoid' distribution (face & stomach), it looks odd.

Tomorrow, the medical equipment guy is going to meet me at our physical therapist's office to drop off a couple of standers for us to try out with Bethany so that we can determine which one we want to buy for her. He will also get Hannah & Bethany's measurements for their adaptive stroller (an Otto Bock Kimba Tandem). It's a double pediatric wheelchair that is very cool (I tried to put a link in here, but it isn't working with Firefox, so if you want to see the stroller, just Google it). Anyhow, after the medical supply guy measures Hannah & determines what she needs to customize her seat, he'll meet me at the hospital to measure Bethany & customize hers. Then he'll write up the order, fax it to the kids' nurse case manager at Regence Blue Cross & then, after she authorizes what we want, the stroller will get ordered & soon thereafter, the girls will have their wheels! Can't wait!!!! :)

Written Mar 6, 2011 4:01am

Bethany bit a student nurse on Thursday. I wasn't there, so I don't know exactly what happened, but the story Charley & I were told was the nurse was holding Bethany and B suddenly leaned down & chomped on the nurse's forearm hard enough to go through her scrubs & break the skin. Considering Bethany doesn't bite us & we've never seen her attempt to bite anyone else, both Charley & I were shocked to hear that Bethany had bit the nurse. We also wondered what the nurse was *doing* that provoked our child. Even if it was something as benign as not paying attention to the signals Bethany was sending that she was "done" and wanted to be left alone/put back in her crib (she would have been growling), it would have helped us to know had happened. Unfortunately, no one was exceptionally forthcoming with details. That may have been because they'd put an unsupervised student nurse in charge of Bethany's care for 3 days since she (B) was a 'good teaching case' and they weren't supposed to do that (we had no idea she was a student; since there was never an RN overseeing what she was doing, we thought she was also an RN). We don't know. As a result of this incident, Charley & I decided we were done following the advice of our doctor to 'just visit' Bethany while she is hospitalized (this advice was given because Joshua needs me to be with him & since Bethany is profoundly autistic, the pediatrician did not believe she would care that I was not there all day every day. We had been reassured that the nursing staff would take excellent care of Bethany and that everyone understood the circumstances of our family's situation with Joshua & the need for my other children to have their mother at home more than she was at the hospital. Bethany showed us, clearly, that she disagreed with our assumption, and now we have someone at the hospital at all times. The result? She growls at every nurse that walks into her room, but she smiles & laughs with whichever family member is with her. Needless to say, we won't leave her alone in a hospital again. Autistic or not, she obviously DOES notice our absence & desires for us to be around, even if she's not interacting with us.

Last night, Charley & I went out to dinner with his siblings who had all come into town for their brother's memorial service while Megan stayed with Bethany. When we went back to the hospital afterward, Megan informed us that Bethany's g-tube had been broken & her IV had blown and the nurses were going to put in a new IV, but the on-call pediatrician had said they should just stop feeding Bethany & increase her IV fluids until morning, when the day crew could deal with the problem. Nice. I asked how in the world the g-tube had gotten broken and learned the nurses had been using the wrong port to hook up her feeds. There are two on the end of her PEG tube ~ a big one & a small one. The hospital's feeding kit can fit easily on the small port or it can be shoved into the big one, but if it's shoved into the big port, it is

REALLY hard to get back out and requires a lot of twisting & bending of the PEG. So... too many feeds given through the wrong port resulted in the port attachments (both of them) being broken completely off of the PEG tube. The nurses tried using tegaderm to attach the part that had broken off to the tubing, but it leaked badly. Today they messed around for awhile trying different things & came up with using a 2-ounce syringe with the tip fitted over the outside of the PEG tube and slowly pushing the formula into Bethany's belly. It works but since the syringe tip is on the *outside* of the PEG versus the inside of the tube, there is still leakage that occurs. Still, it's a decent-enough fix that we can make it work until Bethany goes back to Portland at the end of March to get her PEG switched over to a mic-key button. It's pretty frustrating that the wrong port was used in the first place (repeatedly) & that the tube got broken. This incident further proved to me that it's important that Bethany not be left alone at the hospital ~ when I'm there, I hook her up for feeds & I know the proper port to use. Yes, it's disappointing that I would have to be there to ensure it gets done properly.

This hospitalization has been frustrating & disappointing in a lot of areas, not just the two mentioned above. The last issue I'll share is that when Bethany was admitted last Sunday, the ped & I discussed how she (the ped) wanted Bethany to stay inpatient for a week, but if she was stable & doing well by the following Sunday, we could attempt to put in a PICC line & thus be able to bring Bethany home & finish her second week of IV antibiotics outside the hospital. As I mentioned in my last update, the nurses & ped were not confident that attempting a PICC would be successful, but I was under the impression that after a week inpatient, the anesthesiologist would be given another shot at it, and I'd been told by the ped that if the PICC attempt was unsuccessful, Bethany could be given a central line in her jugular & we could go home with that (and the doc who had attempted her PICC line last hospitalization was 100% sure he could put in a central line in her neck). Today, however, I heard from our nurse that the ped had said to let me know that she didn't want to subject Bethany to anesthesia, so the PICC line was out. While I understand not wanting to sedate a child unnecessarily, I think it's worse to force a child who spent the first 3 years of her life stuck in a crib to sit an extra week in the hospital *just* to get IV antibiotics three times a day when that child could get the medication at home if she had a PICC line (or another form of central access), which would allow her to resume a more-normal life again & would reunite the family (which is a good thing, in my opinion). I think it is a poor choice to force the second week of hospitalization on a child who is feeling GREAT and desires to be active, especially when it's a child who is severely developmentally delayed & has spent 6 of the past 9 weeks hospitalized! Unfortunately, our usually-awesome

pediatrician made this decision & left town before we could talk about it, so I have no idea what her reasoning was and therefore am left with only my opinion on the subject. And since I am not thrilled with the way things have been going for my child this past week with regards to her medical care, I am not at all happy about learning that she is going to have to stay there for another week since her pediatrician reneged on the offer of a PICC line & outpatient IV antibiotics.

The lesson I learned this past week? If you have a nonverbal child, stay with them while they're in the hospital (or arrange for someone else to be there), even if people tell you you don't need to be there & the nurses will take good care of your kiddo. A nonverbal child can't advocate for themself & it sucks to hear about an incident & sense that you're not getting the total truth, but be unable to ask your child what happened. Overprotective? Maybe it seems that way, but I'm certain Bethany will appreciate (and benefit from) not being left alone again.


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Sorry for the lack of updates on Bethany. A lot has been going on that is not B-related ~~ Charley's brother died & his niece was diagnosed with a mass in her brain. This came on top of his sister being diagnosed with ALS, too.

Bethany is stable & doing fine. Her labs today looked good & showed no indication of any kind of JRA flare, which is fantastic news. Our ped said we were really fortunate to catch this infection so early; it prevented a lot of potentially-ugly complications. Bethany will have a swallow study tomorrow, more labwork on Friday, a VCUG on Monday and after that, who knows? Hopefully nothing unexpected will show up on her tests and she'll get discharged after her 14 days of antibiotics, as scheduled.

She's got granulation tissue that has grown up and around her g-tube, which is pretty gross-looking. The nurses have been putting some cream on it, but it's not doing anything except making her site a bloody, raw mess. The ped said that she would cauterize the tissue early next week if the situation doesn't improve significantly. Bethany

isn't complaining about the area hurting, but since she doesn't let us know that *anything* is hurting, her lack of fussing isn't as reassuring as one might think.

There's not a lot to report since not a lot is going on, which is actually a really good thing. If anyone had any confidence that we could get a PICC line into Bethany, she would get one & then get outpatient IV antibiotics, but after her last experience with attempting the PICC, no one down here is eager to give it another try. I wish they'd just put in a central line & be done with it, but apparently only a few nurses and I think it is a good idea. Hopefully Bethany will be able to get completely over this e-coli infection and then won't need to be in the hospital again for a long time, which would make a central line unnecessary. If she winds up here again, though, I think I'll push to get her one. It could potentially cut short the amount of time she spends in a hospital crib & I think that would be a good thing for her, especially considering her background of living in an orphanage for 3 years.


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Bethany's kidney ultrasound showed hydronephrosis in both kidneys (a few weeks ago, during her last hospitalization, it was in just her left kidney) but no abscess or mass (which is good). Dr. S (ped) called the hospital to talk to me & said that she'd been in contact with the urologist & rheumatologist up in Portland. The urologist thinks the VCUG that was done prior to Bethany's discharge a couple weeks ago was erroneous & he wants it redone to evaluate for urinary reflux. He also believes that the kidney infection Bethers had a few weeks ago was an incredibly bad one & it wasn't fully resolved when she was discharged from the hospital, so the infection we're currently treating is the same one as before rather than a structural problem. If he's correct, that'd be a huge relief.

The plan of action as of today is to keep Bethany on her two IV antibiotics for 2 weeks and then switch her to an oral antibiotic for 2 months. She'll get another VCUG next week, too, using the guidelines the urologist specified. Hopefully, when all is done, the colonized e-coli will be completely destroyed & we won't have to deal with this bacteria

again.

The rheumatologist isn't worried about how things are looking right now, but in light of Bethie having swollen hands and feet (which is associated with the beginning of an arthritis flare), he wants to keep her on the same dose of steroids for the next few weeks rather than continuing to taper her dose downward as we've been doing. He'll be seeing Bethany on the 23rd for her follow-up appointment from when she was discharged from Portland at the end of January (she'll be getting her PEG changed over to a mic-key button on the 24th & another hearing evaluation on the 25th) & we'll discuss where we're going from here with regards to her JRA treatment (I know it's been changed to s-JIA ~ systemic Juvenile Idiopathic Arthritis ~ but after telling me that, the rheumatologist continues to refer to her condition as s-JRA ~ the "R" is Rheumatoid ~ so I think I'll go to that, as well).


This update isn't about Bethany, but it's something I want to share because truly, my family needs some prayer support in addition to what's happening with Bethany & Joshua.

One of Charley's brothers, Jim, was diagnosed with ALS (Lou Gehrig's Disease) just over a year ago & he is not expected to live more than a few more days because he has pneumonia & is no longer receiving food or medication aside from pain meds. He's at home with hospice. Then, this afternoon, Charley's mother called to tell us that one of Charley's sisters, Marilyn, was diagnosed on Friday with ALS, also. Jim is 49 years old and Marilyn just turned 43.

Charley is devastated by the impending loss of his older brother & the inevitable loss of his baby sister. There is also the reality of having a 50/50 chance that he carries the same genetic mutation that his father, Jim & Marilyn have. Of course, just having the gene mutation would not necessarily mean a person would wind up developing ALS, but just knowing it's a genetic possibility creates some stress.

Anyway, like I said, I know this isn't Bethany-related, but I wanted to share what else is going on in our family aside from Bethany's illness & Joshua's fragile condition so that those of you who pray for us might add Charley & all of his family to the list.


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Bethany is back in the hospital for at least 14 days. The ESBL e-coli (I was wrong when I was referring to it as EBSI) is back. It is not colonized in her bladder, so the goal during this hospitalization is to locate where this bug is hiding in her body & destroy it completely. The suspicion is that it's in her kidney, perhaps in the form of an abscess, so she will be having an ultrasound later this afternoon to see what, if anything, is going on.

Her fingers and toes are puffy, which can be the first sign of an arthritis flare ~ those often happen when a child is fighting another infection ~ so the pediatrician is watching carefully to make sure nothing 'blows up' unexpectedly.

We caught this infection quickly, so everyone (rheumatologist, pediatrician & infectious disease) is hopeful that she won't get super-sick and that the e-coli won't move into her bloodstream again.


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I cannot believe I'm writing these words, but Bethany is sick again. She has another UTI with the suspicion being the bug at fault is again the drug-resistant e-coli that caused her last UTI & led to her developing urosepsis & landed her in PICU. Her urine was cultured today & we'll know the name of the bug by Sunday. If it is, in fact, e-coli, she will again be admitted to the hospital for 14 days of antibiotic treatment. We would again attempt to get a PICC line into her so that she might be able to get outpatient IV antibiotics rather than inpatient, but since that failed the last time, it's uncertain if another attempt would be successful.

She hasn't shown any sign of illness at all. Today, I took her, Hannah & Joshua to physical therapy and then Bethany had to get labs drawn

(routine as part of monitoring how her body is doing with the steroids & chemo medication she's getting). Afterward, we headed to the pediatrician's for a follow-up appointment after her last hospitalization. When the ped came in, she looked on her laptop to see if any of Bethany's lab results had come in yet & was dismayed to find her WBC was high. That led to my mentioning that Bethany was peeing a lot more than usual & that the smell was very strong. Dr. S decided we should cath her & check for infection and that led to finding white blood cells in her urine, which immediately led to the sample being sent over to the hospital lab for a full urinanalysis. The UA revealed a significant amount of bacteria & the assumption was made that it was probably the same e-coli she fought a few weeks ago. Since there are only 2 antibiotics that are effective against that particular drug-resistant e-coli and they are both IV drugs, Dr. S got busy looking for an oral antibiotic that *might* be somewhat useful against the suspected bug (but wouldn't kill it). The only medication that came up on the list & ultimately got prescribed is one that doesn't react well with Methotrexate, which Bethany gets once a week. The pharmacist realized this when he consulted with me after I'd paid for the prescription. He had to make a few phone calls to find out what to do ~ first he called the ped's office & spoke with her nurse. Then we waited for the ped to call back so the pharmacist could talk to her directly. After their conversation, we waited while the ped called Bethany's rheumatologist to find out what he wanted done & then she called the pharmacist back. Ultimately, Bethany was given the prescription of the antibiotic plus another script for folic acid, which is supposed to negate the effect of the antibiotic on Methotrexate. Weird, but there ya have it.

We got home & the ped called at 7pm to talk some more about this new situation. She is feeling discouraged & concerned because if this new UTI is again caused by the EBSI e-coli, then Bethany will need to be admitted & Dr. S hates the idea of having to do that. Dr. S said she's tired of getting bad news about Bethany (I agreed with her). There is concern that if Bethany has drug-resistant e-coli colonized in her bladder, that could lead to problems as she gets recurrent UTIs that can only be treated with two specific IV antibiotics because once the bacteria became resistant to those two antibiotics, there would be no other way to kill the bacteria & so if/when she became septic again, there'd be no way to treat her. There is also the concern about her needing to be hospitalized for 2 weeks for treatment any time she gets a UTI.

A friend asked if Bethany might benefit from having a port so that she could receive antibiotics on an outpatient basis any time she gets an infection versus having to be admitted to attempt to place a PICC line

(which lasts for just 6 weeks). I think that's a good idea, but I am not going to bring it up to the ped until we know for sure that Bethany does, in fact, have the same bug growing now that she did during her last hospitalization. The ped told me tonight that she wants us to both relax for the next 36 hours until this bug has a name & then we'll go from there, so that's what I'm going to do. We are both hoping that the final report from pathology will show Bethany has an easier bug to kill than EBSI e-coli. The last thing Dr. S wants to do is admit B to the hospital and the last thing I want is to have to spend another 2 weeks there after just 2 weeks at home.

In other news, Bethany saw her PT today & we're moving forward with getting her a stander and an adaptive stroller (which will actually be a double adaptive stroller for her & Hannah). The medical supply guy will be in the office on March 11th and he is bringing a stander, the stroller (not B & H's, but a similar one so we can check it out) and a tilt-in-space wheelchair for Joshua. He'll get the girls' measurements for their seating systems and for Bethany's stander so that he can write up the order & we can submit it to Bethany's nurse case manager at Blue Cross for approval. Once the items are approved, the stander & stroller will be ordered and we should have them fairly quickly thereafter. I'm praying the process goes smoothly. We were able to get Joshua's first wheelchair really quickly (6 weeks) and I am hopeful that this next round of ordering durable medical equipment will go just as quickly. It will be beneficial for Bethany to spend time standing upright every day and I can't wait to have a stroller that provides the girls with the support they each need.

Her g-tube still looks gross & oozes a lot, but we're hopeful that once she gets it changed over to a mic-key button (March 24th), things will improve in that arena. She's still tolerating feeds just fine, so that's a relief.

She saw the orthopedist on Wednesday and he said her hips are structurally normal, which was good news. Her ability to sublux her hips out of place is due to her extremely lax ligaments & not any kind of deformity. She'll need to strengthen the muscles around her hips if she's ever going to be able to walk and only time will tell if that ever happens, but it's not something I'm worried about. I already have one kiddo who uses wheels more often than his legs, so the idea of Bethany needing a wheelchair eventually if she never is able to walk isn't a scary proposition to me.

On Thursday, she went to feeding clinic for a pre-swallow study evaluation. B can handle thick things like pudding & applesauce without problem, but she chokes on things like water or other thin

liquids. As such, she'll have a swallow study on March 8th to determine what's going on & what we might need to do to keep her airway protected when she's eating/drinking.

Oh ~ last thing ~ Bethany now weighs one ounce short of 23 pounds and is 34 inches long. She is finally on the Down syndrome growth chart for weight, hitting the 5th percentile, and she is at the 25th percentile for height (again, on the Ds growth charts, which are different than those for typical kids).

I'll update again on Sunday after I hear from Dr. S about the pathology report.


Bethany's personality is beginning to emerge again & we are getting some smiles out of her, but her illnesses have left her very weak. She is unwilling to sit up on her own & will only stay up for a few minutes if we put her in a sitting position. Rolling around, scooting, pulling to a stand & cruising are completely out of the realm of possible at this point in time, but we know she'll get those skills back once she regains her strength. She is not playing with toys or vocalizing (other than to cry) at all, which is hard to see. However, she *is* showing interest in eating some solid food, which is exciting. She has eaten (at different times) an ounce of either pudding or yogurt for the past 2 days, and tonight we cut up pizza into teeny tiny pieces & fed her a few of them, which she really enjoyed. With everything else being such a challenge right now, seeing Bethany happy to try eating a little bit each day is a real encouragement.

She is tolerating her tube feeds well and her g-tube site is *finally* starting to look better. The hole is filling in & there is not as much gunk coming out ~ that is *such* a relief!

We're laying low at home & not taking Bethany out except to go to doctor appointments or therapy. Our goal is to keep her healthy so she can fully recover and start truly feeling better again.


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I'm sorry for the lack of updates. There just wasn't a lot going on to report. Bethany's g-tube site is still oozing gunk that, as of today, had 5 bugs growing in the latest culture, but Infectious Disease gave the go-ahead to discharge my girly, anyhow, because the assumption was that they are all surface bugs versus anything deeper and could be treated with alternating doses of bactriban & nystatin (skin creams), so after her last dose of antibiotics this afternoon, her IV was removed & we got out of there. I am not 100% confident that none of the bugs are going to cause a problem, but I do trust our doctors & they seemed completely sure that there won't be a problem. Time will tell, I guess! Anyhow, this note is being written from the comfort of my living room, which definitely beats the vinyl recliner at the hospital.

Between Joshua & Bethany, we've spent 49 of the past 67 days inpatient. Everyone in my family is praying that both kids will remain stable so that we can stay OUT of the hospital for awhile... a LONG while. We're all feeling pretty burned-out after the events of the past 2.5 months and honestly, all we want is to adjust to our 'new normal' and get back to living life as a family again.


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I've only got a few minutes to update, so I'll do it bullet-point style.

~ MRI showed no cord compression, so we'll take precautions to not whiplash Bethany's head backward & do follow-up x-rays in 6 months.

~ VCUG showed no reflux of urine into the kidneys, which means the hydronephrosis was caused by her sepsis and not an underlying structural problem.

~ She's out of PICU & on the regular floor with her condition now listed as 'stable'.

~ She'll have people from the feeding clinic coming to evaluate her since she is now not able to swallow liquids without choking ~ prior to

this hospitalization, she could still swallow her medication every night (given via needleless syringe & dissolved in water) without any problem. Why she no longer can, I do not know.

~ An autism specialist (also an OT) came in to work with Bethany & asked me if she stims on her hands very often. When I answered that yes, she did it all the time, but we though she was autistic, the woman heaved a huge sigh & said, "Thank God you already know! I didn't want to devastate you by telling you." (*Note: her assessment was not based JUST on stimming. Bethany fits every criteria for an autism diagnosis) It was actally nice to get confirmation of what we've suspected all along. We'll get Bethany formally diagnosed once she's medically stable again.

~ She'll get out of here on Friday evening (assuming nothing new pops up), once her 14 days of antibiotics for the sepsis are completed.

~ She's back up to 20 pounds. Still 2.5 pounds short of where she was prior to this hospitalization, but still a step in the right direction.

~ Her scalp IV infiltrated Saturday night. She was taken to the treatment room but after 2 hours did not yield a new IV, the nurses gave up and brought her back to bed. On Sunday morning, the day nurses were able to get yet another scalp IV started on the left side of Bethany's head, just above her ear. We're hoping it will last until Friday since she really is almost out of veins to get an IV into.

So... that's where we're at as of this afternoon. Now I'm off to chat with a couple doctors about Joshua.


*Showing signs of feeling better today.

The MRI yesterday went fine. We still haven't gotten a report from it, but I got a copy of the scan & looked it over, and in my totally unprofessional opinion, I don't think there is any sign of cord compression. If I'm correct, that will mean that we won't have to do anything to treat the AAI right now. If I'm correct, then Bethany will have to avoid trampolines, roller coasters, bounce houses, gymnastics

http://cdnphotos.caringbridge.org/2/5/6/9/256912/l.XGXvFbRzkyRthtjj.jpg

~ basically all the same things my 4 chiari kids avoid, which is anything that could whiplash their necks ~ and she'll have to have x-rays every 6 months to make sure her AAI isn't getting worse. We should have her report on Monday & go from there.

The pus in her stoma at her g-tube site grew out candida & rare gram negative rods (no specification on what those are yet). At this point, the yeast is not systemic, which is a good thing (obviously). I don't know if it'll stay in her gut, but I'm hoping so. It's not surprising that she has an overgrowth of yeast considering that she's been on 3-4 big gun antibiotics for 8 days now.

Her weird stuff with her heart rate & temperature dropping when she's not being fed is still perplexing to the doctors (& Charley & me). She doesn't have apnea during the day, but occasionally at night she completely stops breathing for anywhere from 15 to 28 seconds. I wonder if she has sleep apnea; the doctor agrees Bethie needs to see an ENT for evaluation.

She got a new IV in her scalp yesterday & got the wrist IV removed since it was a week old & not working very well anymore. Today is day 6 of her IV antibiotics since getting a negative culture for e-coli, so she has another 4-8 days to go, at which point she can be discharged.

She is showing signs of feeling better today ~ being content to be held for up to 45 minutes & smiling a little & playing with paper ~ but she's so weak she can't sit up by herself anymore, much less pull to a stand or scoot around. It's kind of sad to see, but hopefully it won't be long before she gets back to baseline & begins progressing again.


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The x-rays showed that Bethany does, indeed, have Atlantoaxial Instability (AAI). Basically, it is a separation between the 1st & 2nd cervical vertebra that results in excessive movement where the atlas (C1) and axis (C2) meet. Neurologic symptoms can occur when the spinal cord is involved. She will have an MRI done tomorrow morning to determine whether or not treatment (spinal fusion) will be necessary at

this time.

Her VCUG has been bumped to next week because the need for the MRI is more important.

Her rheumatologist called to talk to me. He said that while Bethany's bone marrow biopsy/aspiration had not shown leukemia cells, it was still possible that she has cancer. He explained that leukemia can present as systemic juvenile idiopathic arthritis and thus be missed, but when treatment for s-JIA doesn't go as well as expected, sometimes it is because it's cancer, not arthritis. He then confused the issue even more by saying that he was sure Bethany had s-JIA because no cancer cells were found in her bone marrow before steroids were started, but with her bone marrow behaving so oddly this past week, he can't rule out her having leukemia *now*. I asked if we needed to redo her bone marrow biopsy & he said not yet. He went on to tell me that the steroids and methotrexate that Bethany is getting will both kill leukemic cells, so if she does actually have leukemia, it's early & there probably wouldn't be evidence in her bone marrow yet. Eventually, though, IF she has cancer, the number of cells will outpace the medication she's taking & at that point, a diagnosis could be made. I don't know if my explanation makes as much sense as his did when he told me. I realize logic would dictate diagnosing cancer as quickly as possible, but we can't safely take her off her medications long enough to do that. Ultimately, I trust the specialists in Portland & their expertise with both s-JIA and leukemia, so I'll follow their lead with this while praying that she does not have cancer on top of everything else.

Her g-tube stoma is still leaking green pus, so another culture was sent to the lab to see what grows out. If it's a second bug in addition to MRSA, another antibiotic will be started. If it's 'just' MRSA, then it's obviously not responding to the Vancomycin & a different antibiotic will have to be used.

She still is showing weird autonomic function and no one is really sure why. When she is getting fed (g-tube), her heart rate is normal, regardless of whether she is sleeping or awake. When she's taken off her feeds, her heart rate drops to the high 40's/low 50's. On feeds, her temperature is normal or slightly cool. Off feeds, her temperature sinks to 3-7 degrees below normal. Anyone know why? We certainly don't. All we know is that everyone is interested in seeing the results of tomorrow's MRI to see if it provides any answers.


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Yesterday afternoon, Bethany had cervical spine x-rays taken to evaluate for atlanto-axial instability (AAI) and atlanto-occipital instability (AOI). These conditions are more common amongst the Down syndrome population & every 3 year old with Ds should have the films done. If Bethers has AAI or AOI, that could provide an explanation for her heart rate/temperature/blood pressure not being where they should be. If she doesn't have AAI/AOI, then that's great news to know. Either way, the x-rays aren't a waste.

She'll be having a VCUG to evaluate for reflux of urine into her kidneys since the ped would like to know what caused her to get septic and she thinks that Bethany has urinary reflux, so she'll have the test to see. If she does, she'll take a prophylactic antibiotic every day. And, dare I say it? Bethany's labs look a little better... her white blood cell count is up to an almost-normal level and both inflammation markers (CRP & sed rate) have decreased. "She's still not out of the woods," our ped cautioned, "but at least we don't have to send her up to Portland today for another bone marrow biopsy." Thank you, God, for that. Now we wait to see if she continues to improve. I can't even put words to how much I want that to happen. I am SO ready to get her out of here and go home.


Her white blood cells went up slightly, but nucleated red blood cells were present, too, which can raise the wbc count & can signify something seriously wrong with her bone marrow.

As of now, she is not getting a central line in her neck today because the ped does not want to risk a line infection. That doesn't make much sense to me since a PICC is also a central line & they were obviously willing to risk a line infection with that, so what's the difference with a jugular line, but since the message came to me via a nurse who had talked with the ped, I haven't yet had the opportunity to speak with the ped, myself, so I will have to wait to have that explained.

They're finally going to start feeds through her g-tube again (10ml/hr), which will be nice for a little girl who hasn't eaten or drank anything for the past 5 days (IV fluids have kept her hydrated).

I've got a call in to her rheumatologist because I would like to touch base with him directly & find out what he's thinking about all of this.

Oh, and her temp is up to 95.6 now, her heart rate is hanging out in the 50's (& setting off the alarm on a regular basis when it dips), her O2 sats are in the high 90's (ie: totally normal ~ about the only thing that is) and her blood pressures are low but better than when she first got back to PICU after this morning's procedure.

Again, I'll update when I know more.


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After 11 attempts, the doctor gave up at trying to get a PICC line in Bethany. He was sincerely disappointed & apologized repeatedly & that he'd be depressed for days. The nurses told me he meant it... that he took failure very personally. I felt bad for the guy ~ it's not his fault Bethany's so tough to get lines into. He did say that if the ped wants him to try again, he'll put in a jugular central line this afternoon, so she may go back for that.

It took her a long time to wake up & finally involed the doctor stimming her repeatedly by lifting her, rubbing her chest & squeezing her cheeks. Once she got back to PICU, she was extremely cold despite having had warm blankets on her in recovery. Her temperature was 91.6 degrees, her heart rate was in the low 40's & her blood pressure was low. Her fingers & toes were a dark purple from poor perfusion. She was wrapped in warm blankets, had a hat put on to trap heat coming off her head & given meds to deal with the rest. At last check, her temp had come up just a bit over 1 degree and her fingers/toes were a light purple ~ a definite improvement.

Sadly, she had to have blood drawn from her arm once she was stablized in PICU because there was no PICC to get labs from. The only usable vein that phlebotomy can find is covered in a bruise that gets bigger every day since they go through the bruise to get to the vein. It's really sad. We are currently anxiously waiting to get the results from those labs. If her white blood cell count dropped more and her

inflammation markers increased, that would be a bad combination. I'll update again after I know more.


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Because sometimes truth is stranger than fiction, I will share the newest concern for Bethany from the specialists in Portland (as a result of what her labs are doing): leukemia. I am NOT saying she has it... just that the docs are very concerned that she might. The next two days of labs & potentially another bone marrow biopsy will give us more answers. Can you say, "You've GOT to be kidding!!!" Yeah, me, too. She has a handful of conditions that are exacerbating each other & thus making her very complicated. Apparently, we adopted the female version of Joshua. She'll be getting a PICC line tomorrow, assuming that the doctor can get it in, that is. Her veins are a mess & he told me that he was prepared to "work aggressively" to get one in, but she may come back to her room without one, at which point he would talk with her ped to determine what other route they can take to get her longer-term IV access.

It does seem from her labwork that they've got control of her sepsis & MRSA at this point, but the rheumatologist & ID doc in Portland want her taken off one of the antibiotics (Amikacin) and possibly they'll pull her off of Vancomycin, too (to try and help her bone marrow rebound), but that could allow the MRSA to go systemic, which would be a problem. Her steroids are being tapered a bit & we're stopping the Methotrexate for right now ~ again, as an attempt to bring her extremely-low white blood cell count back up & get her bone marrow started on producing the correct proportion of cells. This, of course, could allow her body to flip back into MAS, but the specialists all agreed that it was something to try. No one is sure what is going on or what Bethany's body is going to do next, but I'm reassured by the fact that everyone is communicating frequently & everyone seems to be on the same page each step of the way thus far. I am extremely happy that our brilliant pediatrician is back. Her partners (minus one) did a great job taking care of Bethany, but Dr. S knows Bethany & me & I trust her completely, so I am able to relax a lot more with her in control of my daughter's care while we're here. She told me she would do

everything she could to keep Bethany down here, but there may come a point where she has to be sent north. She understands how much I want to stay close to home & totally supports that since she doesn't look at just the patient, but the needs of the entire family, but she won't keep Bethie down here if it would be unsafe to do so. Thankfully, like I said, I completely trust her, so if she tells me it's time to go to Portland, I won't be happy, but I won't have to wonder if it is truly necessary, and there's something to be said for that. Still, I am hoping & praying that she's going to just start improving & that her labs will come into proper order & we'll get to go HOME.


Bethany is 'consistently unstable'. How's that for clear as mud? It's what I've been told today. She is staying stable while she's still considered extremely unstable. Yeah, I know it's contradictory, but that's what's going on.

In addition to sepsis caused by a highly-resistant strain of e-coli (it responds to just 2 antibiotics; she's on both of them) and a MRSA infection at her g-tube site that everyone is hoping & praying does not go systemic and a flare of her s-JIA caused by the infections, she also has a kidney infection, fluid in her kidney that could be anything from an abscess or an obstruction to a by-product of the infections she's got (we're still waiting for the ultrasound report to know for sure), and consistent bradycardia & irregular heart rate that no one can determine the cause of. Her labs each day are "complicated" (what I am told repeatedly by all involved in her care). One lab value (her sed rate, which is an inflammation marker) increased 50 times over what it was yesterday; meanwhile, the other inflammation marker being watched, her crp, decreased substantially. Contradictory? Yep. Why? No one knows yet.

She'll be getting a PICC line since she'll be on IV antibiotics for several weeks. I'm not sure what day the line will be placed, though; the ped told me tonight that there's concern about Bethany's coagulation levels (they're too low), as well as the question whether they should risk placing a central line when she's got an active bacterial infection in her blood. That decision isn't mine, so I've just got to trust whatever they decide. The other big decision to be decided by the docs is whether to risk letting Bethany out of the hospital (once she stabilizes) with a central line that is "open" ~ not under the skin like Joshua's port ~ to finish her antibiotics since she is SO susceptible to infection. If they decide against letting her out, that would mean we're going to be here for at least 2 more weeks. I can't really stomach the idea of that, so I'm not dwelling on the possibility. I'll wait to see what is decided.

The hope is that with 3 big-gun antibiotics being given (Vancomycin was added to the line-up to address the MRSA), Bethany will start to show signs of improvement. If she doesn't, then she'll have a CT of her abdomen to look for a peritoneal abscess, which could potentially have formed if she did have formula dripping into her peritoneal cavity through the not-completely-formed g-tube tract.

I asked tonight if she was still at risk of dying. After a pause, I was told no one would consider her out of the woods yet. She's stable insofar that she's not completely tanking, but she's unstable insofar that no one is entirely sure that she won't suddenly tank. All in all, it's not very comforting.


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The pathology report showed the infection at Bethany's g-tube is now methillin resistant staph (MRSA). There was a long list of antibiotics that the strain of staph that is growing is resistant to, but I don't know if that will mean they have to change the antibiotics Bethie is getting or not.

The bacteria in her blood & bladder & kidneys that is causing her sepsis is "extended spectrum beta lactamase (ESBL) e-coli. All that means is it is a resistant strain of e-coli that doesn't respond to the first-line antibiotics. The antibiotics she's on (started yesterday) are covering this infection.

I know the lab values that have come back, but am not sure what the overall significance is. The ped said they look complicated today, so she was going to call Dr. K in Portland to ask him what he thinks & what, if anything, he would change about Bethany's treatment plan for today.

She isn't showing any improvement yet, but that could be because the Rocephin wasn't the right antibiotic to treat the sepsis. Everyone taking care of Bethany here (as well as Charley, me & the rest of our

family) is hopeful that the new antibiotics will get control of the sepsis soon & she will start feeling better.


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Written Jan 31, 2011 10:20am

Bethany had her first apnea episode where she stopped breathing completely ~ monitors blaring & showing "0" & no chest movement ~ the nurses were in here immediately & dealt with it (w/stimulation & O2) & she hasn't done it again, but she remains in bradycardia. We're waiting on the results of her morning labs to see how things look for her on the inside. Since she's on 2 big-gun antibiotics, I anticipate hearing that things are moving in the right direction.


Bethany is doing so well tonight (sarcasm) that she has two nurses assigned to her. It's alternately reassuring & scary, depending on how I choose to look at it.

She's having bradycardia (slow heart rate) & her respiration rate is slow, as well. Her oxygen saturation has been fine, though, so that's good. Heart rate abnormalities are associated with severe sepsis, so we understand WHY this is happening; it's just a bit unnerving to have the alarms going off numerous times each hour. The ped said that there's nothing that can be done for the bradycardia, but if Bethany begins desatting, as well, then they'll have to intervene (I think they'd have to put her on a ventilator, but I'm not 100% sure about that).

Her temperature is hanging out between 94 & 95 degrees. With heated blankets on her, she managed to get up to 96.4, but that was it. Low body temperature is another sign of severe sepsis, so again, we know why it's happening, but it's not good that it's happening.

The antibiotics she is now on are Amikacin and Meropenem. In the guestbook, a friend asked why these meds will increase her misery. The answer is "the side effects." Amikacin can cause hearing loss & kidney damage, dizziness, vertigo, ringing in the ears, numbness, skin tingling, severe watery diarrhea & stomach cramps, muscle twitching,

nausea, vomiting and a rash. Meropenem's side effects include nausea, vomiting, diarrhea, vein inflammation in association with blood clots, rash & headache. Side effects are increased for people who are taking multiple medications. As such, the ped & ID doc feel it is realistic to expect Bethany to have side effects that make her more miserable than she already is. If it works to control the sepsis & help her recover, it's worth it. But it's going to be difficult if she has a bunch of side effects and we can't determine what they are since she can't articulate anything to us.

She is sleeping in very tiny increments of 5-10 minutes. The longest she has slept at one time during this hospitalization is 45 minutes.

That's about all I have to update tonight. I'm going to go watch some NCIS and then try to get some sleep.


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Pathology called the ped to let him know that the bacteria growing from Bethany's blood & urine cultures is not sensitive to Rocephin. The ped told me that he thought that was impossible since Bethie's labs looked better today, but to be safe, he was going to call Infectious Disease in Portland to ask them what they thought. He came in 20 minutes later to tell me the ID doc said it was entirely possible for a sepsis patient to have labs that improve temporarily once antibiotics are started, but then deteriorate due to being started on the wrong antibiotic, so they advised him (ped) to change Bethany over to a big-gun antibiotic that is specifically sensitive to gram-negative bacteria. The ped said the antibiotic he'll be starting B on is nasty and will add misery to misery, but he's unwilling to take the risk of having Bethany take a sudden turn for the worst and I agreed with him that yeah, we should avoid that. :) He and I also agreed that Bethany is already miserable, so what's a little more?


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Dr. K (rheumatologist in Portland) is encouraged that Bethany's labs seem to be trending away from MAS. Charley came here (with Megs, Em & Sarah) & he got to talk to the ped with me ~ we asked, "So MAS is out of the picture now?" and he answered, "For now, it looks that way." So it's "just" sepsis, which is dangerous, but we can treat her locally. While it still stinks to be here, at least I can still see my husband & kids, so that helps ease the sting of being in the hospital again.


This will be short because I have to rub Bethany's head or she cries, so I am typing one-handed.

She is septic and very sick. Her temp is very low (95 degrees even covered with heated blankets), her labs are still wonky, her coagulation is abnormal and the ped said this is a life-threatening illness in and of itself, but her underlying s-JIA makes things more complicated. I think that is because it's difficult to know which lab value is caused by which disease.

The ped is in communication with the rheumatologist and together they are determining the course of action. Bethany is on antibiotics, IV fluids & as of about an hour ago, word was that it could still go either way as to whether she'll get transferred today to Portland. The ped said he thinks we'll stay the course here, but if things change, he'll send her up.


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Bethiebean has a staph infection at her g-tube site and systemic bacteremia caused by a bladder infection that is suspected to be caused by e-coli (still waiting for the bug that grew in her culture to be identified). She's on antibiotics for both infections.

Dr. K (rheumatologist in Portland) has called to talk to me twice. We went over her labs & the trends over the past 3 days and discussed MAS versus infections being the cause of this illness. He is hoping that Bethany is so sick because of the bactermia (since that's easy to treat with IV antibiotics), but he isn't yet ready to declare her 100% out of the woods with regards to MAS & said we'd take it day by day & watch what her labs do. He told me the reason he has to bring up MAS as a possibility is because she had it before (it was controlled by the high-dose steroids she had in the hospital while in Portland), so she is more likely to flip back into it again, and because it has a high mortality rate, it's imperative to get MAS diagnosed as quickly as possible. That said, he's optimistic as of this afternoon that the two infections Bethany has are the culprits for her illness, so I am feeling better about the situation. Don't get me wrong; she's still very ill & being treated as such, but knowing her rheumatologist is thinking she probably has "just" a couple infections is such a relief! He told me that if the infections are to blame, we should see Bethany's CRP take a big turn downward (c-reactive protein ~ it's an inflammation marker being watched as part of her labs) while she begins to feel better. If her CRP continues to increase despite the antibiotics, then that would be more concerning for MAS.

Soooo.... all of that is to say, keep praying for the staph & systemic bactermia to be the only issues going on with Bethany & for her CRP, specifically, to decrease & for her other labs to trend in the proper direction so that MAS can officially be taken off the table as something we need to consider this go-round. I'd love for Bethie to only need IV antibiotics for a bit before we can go home!


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Bethany had a rough night. Her heartrate & oxygen kept dropping & setting off the alarms. I'm not sure why that is happening & the nurse taking care of her did't know, either. No one got worried, though, so I am taking their cue & thinking it's probably not a big deal. I'm sure that if I'm wrong, someone will set me straight.

The phlebotomist came by at 4am & the nurse shooed her away, telling her she (phleb) could come back in a couple hours. She returned at 6:15am & got into a vein on the first stick. Unfortunately, she didn't get enough blood for all of the tubes she needed to fill, so she had to come back for more. She was having a really difficult time finding a usable vein & was talking to the nurse about doing a blind stick & trying to find a vein (ie: digging around with the needle after it was in Bethany's arm) & I said, "No way!" I told her to go through the bruise just below Bethie's elbow because there was a good vein there ~ the phlebotomist across the street at the medical office building had told me so. She said she didn't want to go through the bruise because that would make it worse, but I told her I didn't think it could be worse than poking my child over & over, digging around looking for a vein & hoping for luck. I asked her to please just go for the sure thing. She relented & stuck Bethany again, going through the bruise & getting the vein. My baby girl did not like that one bit, of course, but I don't regret pushing for it because I believe the trauma of multiple needle sticks would be worse than one more-painful-than-usual stick. Either way sucks, but at least it was one (more) time and then it was done.

She's finally back to sleep, so I think I'll lay down and try to catch another hour, myself.


Bethie's got green pus coming out from her g-tube stoma ~ the GI doc told the ped that it would be "really rare to get an infection from this (the minor tear of her skin)," but apparently Bethany didn't get that memo or maybe the GI doc (who is not the doc who placed the tube) doesn't realize she's immunosuppressed. Rheumatologist is concerned about her labs & speculated that she may be converting from s-JIA to full-blown MAS. She's getting antibiotics (which may have to be switched since they're not sure if she's got a MRSA infection or just a 'regular' one ~ she's had a rapid culture done to determine that status, so we'll know in another hour) & IV steroids. The Portland docs are in close communication with the ped & they are going to TRY to get this under control here versus having to fly her to Portland.


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Bethany is back in the hospital.

She pulled at her g-tube yesterday & tore a bit of the skin, enlarging the hole, which then allowed formula to drain out the stoma around the tube. Problem is, the tract between her stomach & skin has only been healing for a week (since her g-tube was placed), so there may be openings along the tract for formula to drip into her peritoneum, too, which could cause an infection.

She had labs drawn & went to the pediatrician's yesterday at the request of her GI doc & rheumatologist up in Portland and the results were "bad but not dangerous" according to her rheumatologist. She also lost 2 pounds since she was discharged, which is crazy since she has been getting the exact same amount of formula (32oz) each day that she was in the hospital. However, the Infectious Disease doctor said that Bethany's disease sucks up an incredible amount of energy (ie: calories), so maybe that's why she lost weight. Regardless of the reason, we were instructed to increase her to 5 cans per day rather than 4.

This morning, she woke up writhing in pain & had a fever of 103.8 that went down to just 102.5 after getting tylenol. I put in a call to the GI doc & rheumatology & was told to take Bethany back to the lab & the ped's. The results from today's labs were worse than yesterday's ~ her white blood cell count almost doubled & her inflammation markers were high & everything else was out of whack, too. That plus her pain & swelling in her hands & feet earned her a direct ticket to PICU, where her condition is listed as "guarded".

Many more labs have been drawn at the request of her specialists in Portland & we're waiting to get the results from those. No one is sure what is to blame right now ~ her s-JIA, MAS or an infection from the g-tube incident, but I'll update again once they get it figured out.

If you're the praying kind, please pray that this can be treated at our local hospital with the phone conferencing of the specialists in Portland. I am desperately praying that they let us stay here because while we're 45 minutes from home, at least we're close enough that Charley can come stay with Bethany and let me go home to see our other kids.

For my local friends reading this update, I unfortunately have to request no visitors since Bethany is getting chemo & steroids and

therefore has a compromised immune system. As such, we have to minimize contact with people as much as possible.


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I got a 'post-hospitalization' summary written & put on Bethany & Hannah's adoption blog, complete with pictures & a few videos for anyone who might be interested.

http://snadoption.blogspot.com

Thank you for all of the prayers & words of support & encouragement this past month. Knowing so many people were thinking about my kids & praying for them (and the rest of my family) helped me to feel so much less alone during a very stressful time, which is something I am very thankful/grateful for.


We are home & beginning to settle into the routines of life again. Joshua is now using his wheelchair in the house for probably about 95% of his day. The deficits from this most-recent cord detethering are making themselves known & while it's disappointing to see the losses, Charley & my philosophy is that "brain trumps legs" ~ meaning that as long as his brain benefitted from the surgery (and it did), then it's worth the loss of function in his legs ~ so we will adjust to the new normal yet again and keep moving forward. Really, what else CAN we do? Whine & cry & shake our fists at God for the path we're on? Okay, yeah, there's been some of that, but it only lasts a short while before we again sigh and say, "Okay Lord, we trust you. Even when this is hard." And yes, I will admit, this *is* hard. Even after everything I've already been through with my children in the past 8 years, these new diagnoses for Joshua & Bethany take the proverbial cake. It sucks and I am both sad & angry that this is the new path we are being forced to walk, but I absolutely HAVE to believe that God is still in control & therefore He has a plan, and even if I don't see what it is (I don't) and I don't understand what is going on (I don't), He does... but the truth is,

even though I DO believe those things, that doesn't make it easy to accept & it doesn't make it hurt any less.


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Bethany has been discharged & we are now at the Ronald McDonald House until tomorrow morning, when we will leave for home. Little Miss got her methotrexate injection & one last tube feed before we headed downstairs to pick up her prescriptions & supplies and then we LEFT the hospital! Woo hoo!!!!


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Bethie's got a PEG tube now & has gotten her vaccines for pneumococcal & influenza. While those are immunizations I would typically skip, the infectious disease doctor was adamant that Bethany receive them since, as she explained, if Bethie were to contract the flu or a pneumoccal infection in the near future, she would not survive due to her compromised immune status. So... she got the shots and was not happy about it.

The GI doc showed me pictures from the upper GI and explained that even though Bethany's liver was overlapping her stomach a bit (her liver is still a bit enlarged), she was able to get the tube in. She also said the outside of Bethany's stomach had a cracked appearance, which could indicate inflammation, so she (GI doc) took lots of biopsies of her stomach. I don't know what the significance of inflammation might be, but unless someone tells me to worry, I'm not going to spend time thinking about it. There's enough going on without having to borrow trouble. :)

In a few minutes, she will start getting pedialyte through her new tube and after 6 hours, she'll start getting formula & we'll see how she does. Infectious Disease has signed off her case, as has Rheumatology, which means that as soon as Gastroenterology says she can be discharged, we can go home!!!


In about an hour, Bethany will be getting her g-tube & losing her NG tube. She'll also have an upper GI, with biopsies taken from her esophagas, stomach & intestine to evaluate for H. Pylori, reflux & any other abnormality.

If there are no surprises, we will be going home on Saturday. I'll have been gone from home for 26 days, which is way too long for my comfort level. I hope to never have to be in the hospital with my kid(s) for almost a month again!

Audiology finally got here yesterday to evaluate Bethany's hearing & found that her ears are so full of fluid she 'flat lined' on all of their tests. As a result, she'll have to come back to get re-checked in 6-8 weeks. That will coincide nicely with Bethany's need to return to get her PEG tube changed over to a mic-key button (for her g-tube) & her follow-up with her rheumatologist and Joshua's follow-up with his neurosurgeon. I'll schedule the 4 appointments over 2 days, which will make it a quick trip up here.

Not much else to report right now, but I'll update later, after Bethany's procedures.


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Finally some good news: Bethie's labs are all trending in the right direction! That means the steroids are finally kicking in & chilling out her immune system. Yea! Also, there were no hemophagocytes in her bone marrow biopsy, so she does not need the big-gun chemo with

hem-onc. She'll stay on "just" methotrexate & lower-dose steroids (oral). YEAAAAA!!!!!!!!!


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No biopsy report today. Perhaps pathology has been slammed with a lot of work & that's why there has been no report from them yet. I don't know. Rheumatology doc & I are hoping it'll come tomorrow. He's already treating her for MAS, but we'd like to know what the biopsy showed.

She pulled out her NG tube again today and then, once we got it replaced, she would not stop grabbing at her face to try and rip the tape off and get the tube out again. As a result, she got her right arm strapped to a board to prevent her from reaching her face. Let me tell you, that did NOTHING to endear the nurse or me to her for the rest of the day, but it did accomplish the goal of keeping her from yanking out her feeding tube again, so I consider it a win and totally worth her anger.

More labs will be drawn tonight (2am... no joke) to see how Bethany's body is responding to the pulse steroids. She looked better today (though her attitude was worse) and is moving more easily and without pain, but one of the odd things about MAS is that a kiddo can look like they're doing great ~ symptoms of their s-JIA disappear & everything seems under control ~ and then they totally tank in a rapid decline that's very serious. So until we get lab confirmation that yes, her white blood cells & inflammation markers & triglycerides & LDH are moving in the right direction (whatever is proper for each factor), we aren't relaxing our guard.

Assuming nothing surprising comes up, Bethany will have surgery on Thursday morning to get a g-tube placed. The original plan was to send her home with an NG tube & come back in a couple weeks to get the g-tube, but her risk for complications will be higher a few weeks out when she's even more immune-compromised than she is right now, so it was decided (between infectious disease & gastroenterology) to do the tube now versus later.

In Joshua news, he's looking good, but it's an artificial "good" ~ meaning, as long as he gets his pain meds on time & we keep him hydrated enough, he can smile and sit up at the computer to play games and push himself around the room in his wheelchair, but if we miss a medication dose by 30 minutes, he begins complaining of head pain, will retch (or actually vomit if there's anything in his stomach) and needs to lay down for awhile to feel better. I'm happy that we can keep him comfortable and feeling good enough to play computer games or legos, but I'm not fooled into thinking he's fine now because I know better.

His walking took another hit with the latest detethering. He's not doing much of it at all, and when he does walk, his gait is obviously different than it was pre-surgery. I don't really care; protecting his brain is more important than preserving the function in his feet or legs. It's just something we've noticed, so I'm mentioning it. He also has lost all sensation in his booty. He can't tell when he's had a bowel movement anymore, so we're often surprised when we go to change him. Before surgery, he had no control over when he'd go, but he was aware that he was & would let us know so we could change him immediately. Now it's a matter of us noticing via smell or when we open his diaper. Again, not a big deal in the grand scheme of things, but it's another "new normal" to recognize and adapt to.

The one HUGE thing that has changed for the better is that I have not noticed him turning blue ~ having apnea episodes ~ while in his wheelchair. I know those will return again once his cerebellum is again pressed against his brain stem, but it sure is nice to not be seeing them NOW and I am happy that he can be in his wheelchair for more than 20 minutes without being completely miserable. That's a huge gift right now in light of everything else that has been going on with both him & his sister.


Today started way too early, at 4:15am when the nurse came in to draw labs off of one of Bethany's IVs, only to realize after 15 minutes of flushing first one & then the other with saline that neither was going to allow the drawing of blood. By the time she came to this conclusion, Bethany was wailing (even though the nurse hadn't touched her skin at all and hadn't done anything to cause her pain), which woke me.

I had to hold her down while two nurses worked to find a usable vein (not at all easy in an extremely-anemic child) and obtained the 8ml of blood necessary for the labs that had been ordered. I know that

doesn't sound like a lot of blood, but since Bethany's hemoglobin is so low, any amount taken worsens her anemia. Anyhow, after they got the blood, one of the nurses casually announced that we were getting a roommate this morning & she was going to send housekeeping to clean the side of the room that Joshua had been occupying. Okay, no big deal, I figured... I mean, I dislike having to have a roommate since sharing a tiny hospital room with strangers who also have a sick kid is not my idea of fun, but when there is no other choice, I suck it up. I made sure there was nothing of ours on the side of the room that would belong to the roommate and then I laid back down on my cot to attempt to get some more sleep.

Fifteen minutes later, housekeeping showed up. For those who aren't familiar with hospitals, let me tell ya, housekeeping isn't super quiet, even when it's 6am and there is a child in a crib & a mother on a cot trying to sleep. So, needless to say, I didn't get back to sleep. And THEN, as soon as housekeeping was finished, the family from next door was moved over into the room. Why? I have no idea. Yes, their kid also has colonized MRSA, so he wasn't infectious to Bethany nor her to him, but I couldn't understand why they were moving him over since HIS roommate obviously had MRSA, too.

Anyhow, it was just before 7am when they arrived and it was obvious they were up for the day. They were loud & the child was unhappy & the end result was that I gave up trying to sleep altogether. Definitely NOT an auspicious beginning to my day, though Bethany finally did get back to sleep and I was thankful for that! At 9am, the roommate got discharged to go home and once again, I was left trying to figure out why on earth they'd transferred him into our room when they knew he'd be leaving in 2 hours. Regardless, it was blissful to have the room to ourselves again and I am praying no one else gets moved in for the duration of our stay.

The rheumatologist came in & said Bethany's labs were not doing what he expected them to. All of the lab values from the morning's blood draw hadn't come in yet, but her white blood cell count had dropped substantially & other values were "funky," as he put it. As such, he decided to wait on moving Bethany to oral steroids and instead said he would begin giving her "pulse steroids," or high-dose steroids at 8 hour increments throughout the day/night. Then he said he'd be back to talk to me more after clinic was done.

Bethany slept until 11:30am, saw PT for a short session that she did not enjoy but which was important for assessing the mobility of her joints now & helping to keep them from losing range of motion (for the record, her wrists & shoulders are still limited in range & sore, but her

hips & knees are not painful anymore).

At the end of the working day, Dr. K came back and said that the bone marrow biopsy results were not in yet, but based on the lab trends he's been watching for the past week, Bethany does have Macrophage Activation Syndrome as a complication of her s-JIA. She will continue to get pulse steroids (methylprednisolone, aka solu-medrol) at high doses, as well as methyltrexate (the chemo drug). When the biopsy results are available, it can be determined the course of action ~ if there is presence of hemophagocytes, then hematology-oncology (hem-onc) will take over her case. If not, then rheumatology will continue their course of treatment. The difference between the two protocols is the use of etoposide, a bigger-gun chemo drug. Hem-onc uses it and rheumatology does not. Don't ask me why; I don't know.

I kept holding off posting an update today because I was hoping the bone marrow results would come in and I'd be able to give a more-concrete explanation of everything, but what I've shared is all I know tonight. As always, I'll update when I know more, and thank you for the continued prayers for both Bethany & Joshua. Also, I must take a minute to thank all of the girls from BGGB for the incredible cookie bouquet they had delivered (picture at the top of this post)! It is SO cute & omigosh, the cookies are phenomenal (soooo yummy)! I was so touched by your gift, ladies... thank you very, very much for caring & praying & for sending something so sweet (literally) to brighten our day here. You made Adam, Joshua & me smile (I think Joshua was imagining biting the head off a nurse or his neurosurgeon, LOL) and all of the nurses & doctors who came in thought the bouquet was totally cute. Thank you thank you thank you!!!!!


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Bethany is a total grouch today. She's grinding her teeth incessantly & just griping a lot. I understand it's the steroids, but listening to a cranky kid who doesn't want to be messed with & therefore gets even more ticked off when someone tries to play with her or hold her or otherwise comfort her gets old after hours & hours. Yeah, that's probably not very PC to admit, but oh well; it's the truth.

Joshua has started throwing up again ~ he eats & then throws up. That didn't take long to start up once more, huh? We'll go back to having him on a slow continuous feed so he doesn't lose weight & adding in zofran for nausea in addition to the erythromycin he regularly gets, but it's really disappointing to me that 26 days out of the last 41 spent in the hospital & 4 surgeries during that time failed to give him more than a few days' reprieve from puking. Historically, vomiting has always been Joshua's first sign that his cerebellum is pressing against his brainstem; it's depressing to me to see it's happening again so soon after Dr. W decompressed his chiari.

A friend shared a verse on facebook that I have always liked and found comfort in: Isaiah 41:10 "Don't be afraid, for I am with you. Don't be afraid, for I am your God. I will strengthen you. Yes, I will help you. Yes, I will uphold you with the right hand of my righteousness." It helps me to know that regardless of what I see happening or what man might say is or is not going to happen, God is the One who is in control.


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Bethany has had 48 hours of steroids & early this evening, she got her first shot of Methotrexate. Aside from being more irritable than usual, she seems to feel a little better. She's sat up a few times on her own, even, which is definitely a step in the right direction.

I had thought that doing Methotrexate wouldn't be a big deal ~ after all, it's just a little injection once a week, right? Imagine my surprise when the oncology nurse came in with a box of "chemo gloves" & very seriously explained what I had to do to give Bethany her shots each week: double gloves (2 pair) & ensure that not even one drop gets anywhere other than inside her thigh, syringe disposed of in a 'sharps' container & the 'sharps' container gets emptied at the hospital. Then, for 48 hours after giving the injection, I'll have to wear double gloves for all diaper changes & her diapers will be put in a special "chemotherapy waste" bag that will have to be disposed of at our local hospital & her clothes will have to be washed separately, too. It is really crazy to me to think that this drug is SO toxic that it requires

such extensive precautions, yet we're injecting it into my tiny daughter's body. If it helps to modulate her immune system like it is supposed to, though, then it will be worth it. Methotrexate has a less than 50% efficacy rate for systemic JIA, but it's the foundation of treatment for s-JIA, so it's the drug we're starting with (in addition to the Prednisolone ~ steroid).

Joshua is feeling better today. Adam & I finally got on top of his pain last night (I gave him an extra half-dose of his meds) and we've kept him on them ever since, so he's functioning with a manageable headache & was pretty darn happy most of the day. I think the new shunt setting helped, too, because he is able to sit up for a couple of hours without complaint. He's still having the other symptoms that first brought him to the hospital, but he's definitely recovered from the surgeries that he had & is back to being "okay" again.

A friend (used to be Joshua's PT) gave me the brilliant suggestion of "growing" Joshie's wheelchair so that the back of his seat gets moved as far back as it can go and then buying a triangular piece of foam that can be used as a wedge so that Joshua can recline to 75-80 degrees in his chair & thus avoid desatting while sitting there. It is practical advice like this that I SO greatly appreciate. I love that I have friends who want to help make Joshua's life more comfortable & give me ideas that will work to achieve that goal.

Bethany has another day of IV steroids and then she'll get changed over to oral steroids on Monday. We'll get the results of her bone marrow biopsy on Monday, too, and she'll have a consult with a GI doctor regarding the g-tube. The infectious disease doctor informed me that if Bethany's labs don't look good enough, she won't be able to get the g-tube during this hospitalization (because she wouldn't heal well & would wind up with an oozing mess at the g-tube site). If placing the g-tube isn't possible, then she'll go home with the NG tube as a temporary way to feed her while we wait for her labs to improve, at which point I'll bring her back to the hospital to get the g-tube in an outpatient procedure. That would work out with doing follow-up with her rheumatologist, too. So... however it works out will be fine. I'm just relieved that we'll have a way to feed her that isn't going to be stressful.


Bethany is being started on 72 hours of high-dose steroids (which will then be converted over to oral steroids that she will take until she goes into remission) and tomorrow she will start on methotrexate, which will be administered as a weekly injection that I will be taught how to give

her. The ID doc told me she would be irritable & hungry on the high-dose steroids... I'm praying she does not have the same rages that Joshua does when he is on steroids. If she does, that will make for a very loooooong 72 hours!

The rheumatologist, who I really like thus far, discussed with me Bethany's treatment and his agreement that once she's stablized with medication, she needs to get a g-tube. He put a note in her chart stating his support of the procedure being done prior to her discharge, which I greatly appreciated him doing. It would make things easier for my family if we could get Bethany's g-tube done during this hospitalization versus going home & having to turn around and come back up for an outpatient procedure. The infectious disease doctor is the leader of Bethany's team, so she'll have to order the GI consult, but I don't anticipate it being a problem since she (ID doc) is the one who put the NG tube in so that she could pour calories into my tiny girl. s-JIA puts a kiddo into hypermetabolism & Bethany is burning up a ton of calories each day, so the need for a tube is obvious when you consider she will barely drink from a bottle & she is unable to eat any solid food yet.

As for her labs, everything came back showing the highs & lows where they were expected for a child with s-JIA. Also as expected, there is no news about the biopsy yet.


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Bethany is having her bone marrow aspiration & biopsy right now. They'll be drawing more labs while she's asleep, too, and I'm grateful they're doing that while she's out so she doesn't have to feel anything.

Last night, she began desatting, with her oxygen level dipping as low as 76%. She'd get back up to 95%, but drop back down again. I don't know why that is happening & so far no one has come in to talk to me about it, so maybe it's not a big deal. It WAS interesting, however, to note the difference in the nurse's reaction to Bethany's desats versus Joshua's. With Joshua, the alarm gets silenced & that's it. With Bethany, the nurse comes rushing in, moves her to stimulate her to

breathe & gives her oxygen (when needed). I guess the difference in approach is due to "fixability". They can't fix Joshua's apnea episodes, but they can deal with Bethany's, so they try. Joshua turns blue when he stops breathing & desats, but Bethany keeps breathing ~ her sats just drop ~ so maybe that plays into it, too. I "get that" on an intellectual level, but emotionally, it's like a knife being turned in my heart.

I am really struggling with the idea of leaving with a child whose symptoms haven't improved the entire time we've been here. I'm especially stressed about leaving without any kind of "game plan" for how we're supposed to handle things from here on out. I know that if the doctors up here don't help, our pediatrician at home will care enough to want to do something, but it still sucks that we had that family care conference and everyone agreed that we absolutely needed an outline, some parameters, for what symptoms needed to be dealt with (and how they should be dealt with) and what things couldn't be fixed so we'd need to treat supportively, but we couldn't expect them to go away. What good does it do to have everyone agree and then have no one DO anything to help us figure out how to live with this new normal? Again, I intellectually understand that no one knows with certainty what is going to happen or when it will happen, but emotionally, it hurts to realize that these doctors I have respected for years are ready to walk away from the situation by saying (essentially), "Well, we don't have a diagnosis for what's happening to Joshua, so you take him home & deal with it the best you can.... we aren't going to give you any advice for what you can do to help him have a good quality of life. Sorry 'bout that. Bye!"


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Been a bit of a frustrating day.

Bethany got transported to Day Surgery for her biopsy, only to have the anesthesiologist tell Charley & me that he couldn't be the doctor for her case because she needs an anesthesiologist who is trained for cardiac patients. Don't even ask me what the difference is... I didn't realize there were different levels of qualifications for anesthesiologist.

Anyhow, the guy didn't seem to realize ahead of time that Bethany had a heart condition even though it's in her chart notes & is prominently displayed as part of her diagnoses. End result was that Bethany got taken back to her room & we were told that she'd have the biopsy sometime later in the day.

Three hours later, Bethany's ID doctor was frustrated with the anesthesiologists and told them they had to commit to a time that they were going to do the biopsy because her patient was a tiny girl who could not go all day without food & they cannot start treatment for her until after the biopsy is done. The decision was made to do the biopsy tomorrow & start Bethany's feeds again so that she gets some calories today. She also was given some pain medication to make her more comfortable.

The kids are now in the same room, which makes all of this a fraction easier to deal with.

They're moving toward getting Joshua ready for discharge and I find myself protesting (in my mind), thinking, "Wait! He is still desatting when he's sitting up and he's got double vision and his head hurts and his balance isn't great! How can you discharge him when he's so obviously NOT okay?" And then I remember that they can't fix any of those things... it's our new normal... and then I feel really, REALLY sad.

We're trying to determine whether or not Joshua will come home with monitors & oxygen. Half of his doctors think it's a good idea, as they want us to keep track of how often he desats during the day & at what percent he begins breathing again & they feel oxygen would be a good thing to have in case it's needed to get his O2 levels back to normal after a big desat episode. The other half think it doesn't matter if we have monitors for Joshua because there isn't anything we can do when he desats & they say there's no reason for us to be hooking him up to monitors that will only cause us to worry. For me personally, I think I'll worry MORE if I don't have a clue what is happening with him other than noticing, "Oh hey, he's turning blue.... c'mon, Joshua, breathe!" I'd like at least a pulse ox so we know how low his desats take him. It'd be nice to know ahead of time that gee, his desats have him going to 70% or 60%... it would give us a clue that things are progressing. I don't want to take him to the doctor's, have him desat while there & find out that oh wow, he's now desatting to 60% when we've been thinking the lowest he goes is 80% - 85%. So... it'd be nice if they could all get on the same page. This is one drawback of having a lot of doctors involved in one child's care ~ they're bound to disagree at times & then I'm left wondering who's going to 'win' the discussion & what that will mean for my child.


I'm sorry about not explaining anything in my last update. Charley came up with Emily & Sarah (2 of our daughters) and things were a bit busy, but I wanted to put up a quick mention of what was going on, so I tossed out what we'd been told by the rheumatologist, hematologist/oncologist & infectious disease doctor without thinking that most people wouldn't know what I was talking about.

Now that Adam has taken the girls down to the Ronald McDonald House to play for a bit before going to sleep (Charley is in with Bethany, who is asleep, and Joshua is in bed beside me playing with legos), I can take more time to explain what's going on. :)

As I understand it (and please keep in mind that what I'm going to share is newly-acquired knowledge, so I may get a detail or two incorrect and have to clarify in the next few days), Systemic Juvenile Rheumatoid Arthritis is an autoimmune condition where the body's infection-fighting cells go a little crazy and begin attacking the joints and (sometimes) organs and bone marrow of their owner's body. Systemic JRA is the most uncommon and 33% of children with this form of JRA never achieve remission & can be very difficult to treat. We are hoping, of course, that Bethany will be one of the 67% who DO achieve remission & are not significantly impacted by their JRA on a day-to-day basis. This condition can be treated, but not cured. It is lifelong (chronic).

MAS is Macrophage Activation Syndrome. It is, essentially, where the macrophages (large white blood cells) that usually eat up/destroy the bad stuff in our bodies go totally crazy & begin eating pretty much everything. That's not a 100% perfect explanation, but it's the easiest one. It is a potentially life-threatening complication of Systemic JRA (which, incidentally, is also known as Systemic Juvenile Idiopathic Arthritis).

Bethany has every marker for MAS. The only one we don't know about yet is the presence of hemophagocytes (cells that destroy red & white blood cells & platelets) in her bone marrow, which is why she is having a bone marrow aspiration & biopsy tomorrow. If the test shows she has hemophagocytes, then it is my understanding that she will receive a diagnosis of MAS.

The treatment for MAS is high-dose corticosteroids & cyclosporine (immunosuppressants). If that does not work to chill out the macrophages, then the treatment would involve chemotherapy & a bone marrow transplant.

So... for obvious reasons, we are praying that Bethany does not have MAS. Her having SJRA (in addition to Down syndrome & Autism & a heart defect) is more than enough.

As for Joshua, we got him into his wheelchair and within 15 minutes, his coloring had paled & he was asking to get back in bed. We forced the issue & made him stay in his chair sitting at 90 degrees and within another 20 minutes, he had a desat to 85%. He was growing increasingly uncomfortable & the nurses & I were confident we would get an even bigger desat if we kept him in his chair, but then his head pain was so severe he began sobbing & the nurses cut short the trial, put him back in his bed & gave him some fentanyl & lortab. Tomorrow we will try again. The reason we are trying to get him to desat "big" is to see how long his brain will allow him to stop breathing before it kicks in again. So far, the lowest he's gone is 74%. I think everyone here knows that the desats are occurring, but no one has decided yet what the parameters are going to be regarding at what point we would administer oxygen. During a desat episode, oxygen is useless because Joshua isn't breathing, so he wouldn't be inhaling the oxygen. However, after he begins breathing again, if he is really low in O2 saturation, oxygen would be necessary to get him back up to where he needs to be (95% or greater) in order to avoid brain damage.

Joshua received his last dose of vancomycin tonight and the plan is to see if he begins spiking fevers again 24+ hours from now. If he doesn't, then he should be in the clear as far as infection goes. Thus far, nothing has grown in ANY of his cultures, which has the infectious disease doctor very confused. With his white blood cell count as high as it was, he SHOULD have grown something in at least one of those cultures (blood, urine, CSF), but we all agree that we're not going to question it if he doesn't have any more fevers. However, if he does start having high fevers again tomorrow night or the next day, then the infectious disease doc said we'll have to start all over again in trying to locate the source of the infection. Soooooo...... we're praying for NO FEVERS!!!

The recap, then, is to pray for no MAS for Bethany and no fevers for Joshua. Bethany's bone marrow aspiration/biopsy is at 10am tomorrow and it will take about 24 hours to get the results. There is a tiny chance we MIGHT have some information by tomorrow night, but it's more likely we'll hear the results on Friday.

Oh... one more thing... Bethany's abdominal ultrasound today showed enlarged liver & spleen, inflamed blood vessels leading to & from her liver and something with her kidneys but I can't remember exactly

what it is (I'll ask tomorrow). Her CT was normal, as was her echo, which showed no signs of endocarditis.

I'm off to bed now because The Joshua has requested I come lay down with him. There's something about being in the hospital that renders my son unable to fall asleep without a Mommy by his side. LOL


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Rheumatology & hem-onc have been here. They are 90% sure Bethany has systemic juvenile rheumatoid arthritis. They are still suspicious for HLH, as well, or the possibility that the JRA is moving into MAS (a form of HLH), so tomorrow she will have a bone marrow aspiration & biopsy to get a better idea of what the cells in her marrow are doing.


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Bethany will have an abdominal ultrasound this afternoon to check for possible tumors & consults with other specialists throughout the day. Joshua will have an ophthalmology consult to determine what, if anything, we can do to help with his intermittent double vision & "fuzziness" & we will get him in his wheelchair to see if we can cause him to desat & if so, will he start himself breathing again without help.


Thank you for praying ~ when we took Bethany to the treatment room, the nurse began searching for a vein & I asked if she would please try to pull the blood from her IV. She told me that it wasn't going to work because she'd tried to get a backflow when she was in her crib and it

didn't happen. I implored her, "Please try?" She then agreed to try and was absolutely shocked when the blood flowed easily into the syringe. She quickly pulled off 12ml of blood for the rest of the labs and didn't need to stick Bethie at all! She said, "I can't believe we got blood out of that line!" and I replied, "Prayer works." She was quiet for a moment and then responded, "I can't argue with that." :)

So thanks for praying! It worked and Bethany was spared an extra stick.


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It's been a rough morning already and it's barely 5am. Joshua has another fever, as does Bethany (hers is worse). Bethany keeps dropping her oxygen saturation level and is getting blow-by oxygen (which she hates). Her IV won't draw for labs so after she receives a fluid bolus & her fever is down, she has to go to the treatment room to get another 6 tubes of blood drawn. I came here to ask people to pray for a 1-stick procedure since she has been REALLY hard to get a vein and with her being so anemic it's going to be even harder.

Joshua had blood drawn off his port to culture for labs, which of course he slept right through (once again I have to say how much I love having that central line for him). He had a huge vomiting episode last night that led to uncontrollable retching & needed zofran to break him out of.

I am totally exhausted after less than 3 hours of broken-up sleep and I am praying today smooths out. Please pray that it does.


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I'm sorry about no update for so long. It's been a VERY busy 5 hours since Bethany got here. She had a CT scan, an echo (showed no endocarditis), got 6 vials of blood drawn, got an NG tube placed, had her nose & booty swabbed & is now in her crib settling down to sleep.

Dr. Mato, the infectious disease (ID) doc, said Bethany's inflammatory markers on her labs are off the charts high. There was one test that the normal value is between 100 & 300 and Bethany's level is 3,000. She is having "third spacing," which is when the blood vessels leak and allow fluids out of the vessels into the surrounding tissue. As a result, Bethany is puffy, especially in her hands and feet. Dr. Mato said whatever is wrong with Bethany is potentially very, very serious. The conditions she is most concerned about (that are possible Bethany has) are a hematologic disorder abbreviated HLH ~ Hemophagocytic lymphohistiocytosis ~ which basically has the body destroying its own cells... or lymphoma/leukemia/some other malignancy... or a rheumatological condition (she didn't specify a name). She also said it is possible it's an autoimmune condition that is untreatable, but she didn't think we needed to get into that today.

Bethany will have a bunch more labs drawn tomorrow and will be seen by hematology-oncology, rheumatology, and by Dr. Mato again. She said that right now, we have a very sick little princess on our hands and we are going to take things one hour at a time, adjusting treatment and plans as new information is obtained (from lab reports and other testing).

Her hematocrit dropped 8 points overnight and she is very anemic, a condition not being helped by how much blood is being drawn. After all of her labs are done, she will get a blood transfusion. As soon as the labs show she does not have a bacterial infection of any kind, she will get a PICC line. There is a strong possibility she will need a bone marrow biopsy.

She has low oxygen saturations (hanging out at 89-90%) but that is attributable to her heart condition, I think. I'll have to ask to be sure.

So... that's where we are at tonight.

Thankfully, Joshua remains stable. Today is the only day in the past 15 that he has not had a desat episode all day. God KNEW Joshua would need to be stable & He made it happen. I have no doubt and I'm thankful for that. Bethany absolutely needed me to be able to focus on her and since Joshua was stable and Adam is taking care of him, I was

able to do that.

I'm exhausted but I've been okay despite everything going on. I'm thankful for the prayers that have been said for Bethany & Joshua and for the peace that surpasses human understanding, which has surrounded Adam & me all day. We know God is in control & He's going to work everything out according to His purpose. Adam & I have been asked repeatedly today HOW we are getting through this & each time, we answer, "God. He is giving us the strength & peace of mind & ability to handle anything that is set before us." It may sound trite, but it's the truth. Without Him, I'd be a crumpled mess of tears & fear, but with Him, I can face each hour & whatever comes with it. I'm so grateful to know Him & to be able to trust that He is holding me through this difficult time.


Bethany is en route to Portland. She should be here sometime around 1pm (PST). I am praying she sleeps during the trip so that she doesn't realize she is alone with strangers. I am praying, too, that the paramedics are kind to her & take good care of her. It breaks my heart to know my baby girl is alone without me or her daddy for even a couple of hours at this time. She was left alone at the hospital when she had her two heart surgeries in Ukraine and she spent every day of her life in the orphanage in a crib, alone & not interacted with. I don't want her to be scared... I am consoling myself with knowing that THIS time, she has a family that loves her and will take care of her. I am extremely anxious to hold her again (it's been 15 days) and to get a diagnosis of what is making her so sick.

Joshua is still stable (thanking God & modern medicine for this). He is getting regular doses of tylenol & motrin to keep his fever at bay, rocephin once a day & vancomycin 4 times per day to address the infection, erythromycin to keep his continuous feeds digesting, neurontin for the neuropathic pain in his legs (which is completely controlled by the med), and lortab every 4 hours for pain plus intermittant doses of fentanyl and/or morphine, as needed, for headaches that aren't controlled by the lortab alone. He's stable, but still medically fragile and being treated as such. Still, it's encouraging to see him starting to feel better and everyone taking care of him here is hopeful that he's turning a corner toward getting well enough to go home. My understanding is that he will get discharged with an apnea monitor & pulse ox to be used daily and oxygen that we will use only if it's needed.


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Joshua's fever is down, his heartrate is back to normal & he is sleeping peacefully right now.

Charley called to tell me Bethany has tested positive for MRSA, which means she will not be able to room with Joshua once she's transferred here. The plan is to put her in a room next door to Joshua, but that will take some moving around of kids, so please pray the nurses here (who are trying to help in any way they can) are able to make this possible. Being in adjoining rooms will be easier than if Bethany is halfway across the peds unit.

Charley also told me that once they gave Bethie some ibuprofen, her fever went away & her rash, which had exploded all over her body once they stopped treating her fever (they wanted to see how high it would go, which makes zero sense to me, but I'm not there, so I figure I must not be getting the entire picture) is about 75% better. She is also sleeping at this time, according to my husband.

I'll update as regularly as I can. The updates may be short if I'm lacking time, but I will do my best to not leave people hanging because I know I hate it when someone does that to me. Thank you for all of the prayers and support and encouragement.... maybe it's cheesy to admit it, but it does help. At the very least, I don't feel like we're completely alone in all of this and it helps to know others care. So thanks for your comments and prayers.

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