JOURNAL OF ADOLESCENT H E A L T H CARE 1986;7:134-138

SPECIAL ARTICLE

A Patient's View of Cystic Fibrosis

G L E N N SMITH

I do not believe anyone can know what it means to have cystic fibrosis (CF). Those of us who have it do not know how to separate the disease's insidious nature from our basic humanity. We do not know what normal is like. Those of you on the "outside" know this humanity but have difficulty with the feel- ing of CF. People t ry - - some try very ha rd- - to un- derstand, but thick, sticky breath blocking mucus continually plays the role of interloper. As a result of our discussions about these problems, my physician, Dr. Karl Karlson, suggested I write this paper to ar- ticulate my view of CF and the ensuing patient medi- cal world relationship.

My view of CF is formed by my experience as an insider, an experience that includes hospital stays, time spent in therapy, physical pain, changes in my mental attitude, explanations to friends, family, and others, and reflections and realizations about myself. I will discuss CF's effect on thought and the physical pains that describe much of my life. These aspects generate the mental ramifications of the whole dis- ease: my outside explanations to others and my in- side reflections and realizations. I do not want to appear a bleeding heart nor to represent all of the CF population. I pray for those worse off. I have to ac- knowledge the people who have helped me mature to the point that enables me to write this paper. My father and mother have done the most with their love and wisdom. The doctors and staff of the CF Center at Toledo Hospital were instrumental and very spe- cial people. Many others thankfully helped with the subsequent refining of my ideas.

I was diagnosed as having CF in the sixth grade when I was eleven, in 1974. I do not remember it as being a real shocker to me. Instead I have a vague

Address reprint requests to: Glenn Smith, c/o Cystic Fibrosis Center, Department of Pediatrics, Medical College of Georgia, Augusta, GA 30912.

Manuscript accepted July 24, 1985.

memory of the sweat tests and then hospitalization to learn postural drainage (PD) and aerosol therapy. Perhaps my parents were told of the ultimately tragic details, but I was spared. I gradually realized that few lived into adulthood with this children's disease. This diagnosis was just a new lifestyle. The promi- nent part of the therapy was the PD. For many years, I have not been able to remember what it was like to get up without a morning PD.

During my first eleven years without a PD, the most important thing was my chance to grow up feeling "normal." I was competitive and well ad- justed with my peers during this time. I am very thankful for that opportunity. However, gastroin- testinal problems did mark my earlier childhood. Some events in prediagnosis time were traumatic, like having a bowel problem at an inopportune time, so having the new pancreatic enzymes helped. I feel badly watching the two- and four-year-olds who have known nothing but life instinctively holding an aerosol.

When Dr. Pierre Vauthy came to Toledo Hospital about 1976, he told me to run- -which I did under much duress. My Mom ran with me, and we used the run in place of a PD. Later I ran cross-country in high school all four years. Morn and I ran all year round, in the cold, snow, drizzle, or heat, even if it was a half mile around the block on a bleak day. I was never very fast, but I missed maybe fifteen or twenty days in four years. I am very glad my mother push- ed, and I am very proud to say I did run. I had done the best I could.

Running and hospitalization for intravenous anti- biotics, a cleanout, were new treatments. Both of these treatments were fully supported by my par- ents' philosophy of doing the best you can all of the time; when you look back, you realize you did your best. No chance for "I wish I had . . . . " They also believed in trusting the doctor. If we hedged on his advice, why did we pay him? These beliefs became

134 © Society for Adolescent Medicine, 1986 0197-0070/86/$3.50 Published by Elsevier Science Publishing Co., Inc., 52 Vanderbilt Ave., New York, NY 10017

MARCH 1986 A PATIENT'S VIEW OF CYSTIC FIBROSIS 135

mine. Too often I have seen people disregard all or part of a doctor's advice. A good doctor should not tell you what you want to hear, even though some- times it is hard to heed that advice.

My parents and I agreed with my doctor's ag- gressive app roach of exploring everything that would help. This approach gave us strength and hope. The running therapy allowed only one hospi- talization, about 19',77. Of course it improved other things like my pulse and pulmonary function, and it probably delayed tile really acute onset of the dis- ease. Another idea at this time (1980) was a bilateral nasal antrawindows. My maxilliaries were cleaned out, and I have had a blocked nose about a total of only seven days since then. Before it had been a com- mon occurrence so this was a good investment, i was also tested for allergies.

My real problems started early in 1981 with mas- sive hemoptysis. Then I had IV Tobramycin therapy at home a few times, including the day ! graduated from high school. More hemoptysis in late October while in college. Then my left lung collapsed. It reinf- lated on its own, but I eventually needed a chest tube after Christmas. This was the end of my running days. In early 1982 came a couple more hospital stays, some during school. I had more massive he- moptysis that summer. Another hospital stay about Christmas time.

More hemoptysis in February, 1983. I have noticed there is no higher contrast than arterial red blood on fresh white snow. Another hospitalization in late May. By now I had missed only one require- ment, chemistry, in my mechanical engineering cur- riculum. I am proud of my 3.2 accum too. More blood in July. I dumped about 350 cc at one time while I was at Rainbow Babies and Childrens in Cleveland. Prac- tically choking it out. Then I had a bronchial artery embolizati0n, which was why I was there in the first place. This procedure was a good investment too. Next I missed 80% of my fall quarter with another left side pnuemothorax~ This time it was surgery and weeks of recovery. I 'went back to Cleveland again for another embolization in December because of more blood. Another hospital visit in February. Then my father was transferred to Augusta, Georgia. So four days after I arrived in March of 1984, the blood gremlins were at it again, and I had more bronchial arteries embolized. My next hospital stay was Sep- tember 1984.

I am a hospital veteran now. I know a hospital's systems and procedures and how to put them to work for me to be comfortable. The transition be- tween passive patients and veteran patients takes an

adjustment time for the nurses and residents which may be difficult; it takes a while for everyone to be compatible. For instance, I know how my bedside stand should be arranged or what little amenities need to be ordered like saline nasal spray or Tylenol. On the other hand, the medical staff can sometimes depend on me too much and dump their responsibil- ity on me, supposing that they need to be less vig- ilant. I am expected to pipe up when something is not right either in procedure or in how I feel. I try to be good tempered, but after all, I am sick enough to be hospitalized. It is a complex relationship and needs to be worked all the time.

Synonymous with hospitals is waiting. Waiting is to the patient as long hours are tO the med student and resident. That is how much waiting there is. Wait for admission, wait for the nurse, wait for the orders to be written. Wait at x-ray coming and going. Wait for the pulmonary function test (PFT) or what- ever. Wait when you press the call light. (There ought to be a differentiation between mere house- keeping calls and "i need a pain shot now!"). The CF patient comes to the medical world feeling sick. All this waiting then makes him feel even sicker, or at the least exhausted.

As insiders we deal with CF in many ways, thera- py, socially, but the most frequent way is in thought. In the beginning after the initial shock and agitation of diagnosis, the disease is widely separated from "normal life." Contact is made with the abnormal at clinic visits when sick. Even the purpose of the rou- tine therapy gets subverted in the mind. Once I was reading the CF newsletter when it hit me that the aerosol I was doing was because of the disease the newsletter mentioned. I was not associating the two. However, CF gradually works its way into the center of our lives like a vine growing alongside a tree. This vine intertwines and eventually chokes the tree off. More and more of our time gets invaded by the ef- fects of the disease: time explaining more pills, time spent in absences and coughing, time spent with more therapies. This increased squeezing of CF af- fects life more dramatically influencing everything I do. I have limited stamina at this word processor. I am at home because college is too physically trying. The college I did attend was chosen in part because of its access to my family's and CF center's support.

The different therapies for CF take a significant amount of the day's time. This is probably a reason for noncompliance. Take the aerosol out, set it up, do it, clean the aerosol up, put it away. Do the pos- tural drainage--i t takes longer when you cough. Get the pills from the pharmacy, count what you

136 SMITH JOURNAL OF ADOLESCENT HEALTH CARE Vol. 7, No. 2

need for the day or a meal, do the insurance for the drugs. Make sure they are taken at the right time! Buy the proper foods. Take out the nutrition supple- ment additive, then put it away. Clean the humidi- fier in the winter. Change the oxygen water and cannulae. DO the inhaler, then clean it. Make sure it's taken at the right time! Go to the park and run. Go to the clinic monthly. I believe physicians and others pile up therapies without realizing the time factor involved. "Increase aerosols." "Increase PD."

I streamline some of the procedures by taking a few liberties: the essence remains. If everYthing is good, no snafus or coughing, an aerosol and PD takes 30 minutes. If in the hospital, the most perfect Setting, all these procedures are not accomplished smoothly all of the time; how then can families do it with other children and parents needing attention too? There is a lot of tinder in these therapies that needs only the spark of "I forgot to . . . . " to cause a fire.

Getting ready for anything like church always takes longer because doing the aerosol/PD therapy is usually involved. Then there are the sundries for going out: the bathroom, handkerchief, tissues, plas- tic bag for emergencY hemoptysis or vomiting, phone numbers, inhaler, snacks. I also usually irri- gate my nose and put some vaseline in it to combat dryness at this time.

Then there is the impact of all of these therapies on lifestyle. I would love to remember rolling out of bed and immediately eating breakfast in the morning. It's not much fun eating breakfast alone 30=45 minutes late while everyone else begins their day. Having relatives visit is rough because you have to go off for therapy when things seem [o be happening.

My college schedule at Ohio Northern University is a good example of how therapy makes inroads on time. I had to get up early enough to shower and do my aeroso ! and PD in the Health Center. The night nurse did the PD, and she left at 8 A.M. I did not want to throw up breakfast because of coughing that I might avoid. I had to get to the cafeteria while it was open; no fat to live off. This precluded "getting up early" to study. Now it was therapy again between 4 and 5 P.M. Many times I had a second antibiotic aero- sol that took forever, hence the second half hour. Then getting enough sleep meant in bed by 10 to 10:30. This limited study time and cementing friend- ships with any late night activities like bull sessions or a pizza.

Doing the prescribed therapy is a hard responsi- bility. If the situation has not deteriorated too much,

there is little positive reinforcement. Now, when I have a bad day, I go out of my way to do therapy, maybe even hoping extra will help. Like diabetes treatment, CF forces responsibility and discipline early. Sometimes it may be too much too early. When a doctor institutes a new therapy, left unsaid is, "Y0u'll be doing this aerosol or oxygen or whatever for the rest of your life!" That monstrosity is tough to handle. That is part of why people shirk responsibil- ity. Real CF discipline is an Iron Lord.

A facet of CF's influence on mental abilities is its effects on concentration. Imagine being aware of your breathing all the time. Sometimes if the dysp- nea is bad enough, even the noise of breathing be- comes distracting. It even affects conversation. By breathing between every other word, and a few more breaths after a long thought, it is easy to lose the train of thought. Obviously lack of concentration im- pinges on the time factor. Tasks like homework be- come more difficult and drawn out. I love to read, and I have noticed my decrease in concentration as measured by time spent in any one sitting. The high- er average breathing rate contributes to the time lim- it. My low weight is a major factor in sitting ability too. I am 100 lbs stretched over 5' 10 ", so I have only bone to sit on. I do not drive regularly because I lack confidence in my concentration.

The chief distractor is coughing spells. A good paroxysm and the recovery time could subtract an hour from my already limited study time in college. Remember they are unpredictable and might inter- rupt things every half hour for a while. I believe you get used to it, and the effect is less. Of course cough- ing hard and weird breathing rates during the recov- ery use the secondary muscles a lot. Much energy is used up and fatigue begins. This fatigue also inhibits concentration. It may be gained back with a nap, but when I awake maybe I might have to cough again.

I often say the mind is willing but the body is weak. And the body is in the driver's seat. This de- crease in concentration can affect motivation, it gets more difficult to do active tasks. This situation be- comes a real dilemma. I wonder if my lack of moti- vation and ability is the effect of the disease or basic human laziness? The tree and the vine are impossible to separate here. Where do I as a person begin and the CF begin and end? This is a real problem. Com- plicating this in my Case was anemia that set in as the result of hemoptysis. I wonder how much and when my concentration was affected before this was diag- nosed. What do I tell the professor or anyone the day of an exam? "i had this cough; it compounded my anemia fatigue . . . . "

MARCH 1986 A PATIENT'S VIEW OF CYSTIC FIBROSIS 137

There are different types of coughs. Some are more tiring than others. Some coughs exhaust the stomach muscles rapidly. Some tire the chest or tighten all the body's muscles to an ache. Coughing even a little blood is more debilitating than an equal amount of sputum. Some coughs seem to generate vomiting, which I have learned to control and keep in the esophagus. (It is hard to do and not much fun.) If there is any sputum, though, then the stomach does not flip. Interesting. Of course, sometimes I end up vomiting or having the dry heaves anyway.

I might mention now that chest pain is more de- bilitating than an equal amount of say leg pain or a headache. It promotes a general malaise and little appetite. It is difficult to convey to a doctor the type, location, and level of chest pain. Gross differences are obvious, but there seem to be many subtleties that are apparent to me during the day. Importantly, this idea of subtle change applies also to judging the progress of the disease. This becomes especially dif- ficult in the hospital when "How do you feel?" gets asked constantly. Medical students and residents are very prone to this. They do not have the intuitive feel to understand the change that center directors do. Closely allied with this is feeling bad or worse but not having measurable clinical symptoms.

So far I have outlined many thoughts, therapies, and other pain. In the more advanced stages there seems to be something constantly bothering me. I am always conscious of breathing. This awareness changes from hour to hour: congested in the morn-

J ing, then merely OK at noon, to a few wheezes to dyspnea and some pain at night. I 'm constantly aware of the air: too hot, too dry, too humid, too stale. With extreme shortness of breath I seem un- der water never reaching the surface or running and not being able to stop and rest. This time is full of anxiety.

After I developed the body of this paper, I was struck by massive hemoptysis again. Time dulls the enormous frightfulness of that experience, so I am moved to tell about it. To watch the blood, my blood, literally pile up in a basin is--indescribable. Even in the hospital with rny doctor standing right there, there is nothing anyone can do. It helps immensely knowing someone is there to care; someone to w i p e my brow and neck of sweat because of the tremen- dous heat built up by that nonstop coughing. Even the conscious is totally divorced from the body. The autonomic system takes command; I am just a body out of control. I get so tired, and the stomach muscles hurt so bad; however, I just keep coughing for five or fifteen minutes without a stop. Wondering when the

blood will stop. And then when it is finished I never know when the blood and its grave consequences will strike again. It could be anytime, 8:14 P.M. and anyplace, the bench outside the college library. So to correct the problem this time, I had my fifth bron- chial artery embolization this past December, 1984. Perhaps the plumbing is fixed now?

There are other secondary manifestations of CF that can be excruciating when added up over time. My thinness is difficult. We searched all over to find a cushion I could sit on for more than an hour. Sleep- ing is not easy with knees and ankles pressed to- gether, and muscle alignment deformed by low weight. Eating can still be fun, but eating enough calories is an everyday chore. Sometimes the the- ophylline can interfere here. And of course coughing or just sitting can cause persistent muscle aches and arthritic kinds of pain. Virtually any sustained action from walking or cooking to bathing, writing, and sleeping places a severe time limit on me compared to normal people. All these perhaps nitpicking things add up. They are incessant and inexorable with, frustratingly, no end in sight.

Explaining CF is already acknowledged to be diffi- cult. What you say depends on whom you talk to. Telling people what I have has gone through an in- teresting metamorphosis. Originally no one knew except family and close friends. I did not tell any of my peers. When the compressor and nebulizer were out, I used to hide them fast if someone came to the front door. It took a while to master taking six cotazyme fairly unobtrusively at school. The aegis was "These are to help my digestion," which is still effective today. Occasionally somebody may com- ment snidely about "Smith's drugs." Over the years I let some good friends know, so by my senior year in high school about six knew. Some teachers knew, and I think more kids had a general idea I had some kind of disease. After all I could not cover up a lot of coughing and nose blowing.

In public places the best explanation is an allergy, asthma, or a breathing problem. Usually I get, "That's some cold," to which I answer, "Yeah." It can make people a little nervous when you bend over, hands on knees, and just hack some sputum up. Rightly so, I suppose. Once a lady told me I probably had pneumonia and should see a doctor. To which I replied, "It's OK; I'm seeing one." The basic idea is to tell people what they want to hear. Once I mention CF, the degree of detail I supply depends on the situation.

When I started college, I gradually began letting a small group know. I had to plan for potentially inca-

138 SMITH JOURNAL OF ADOLESCENT HEALTH CARE Vol. 7, No. 2

pacitating hemoptytic episodes, which made me tell a few more people. I wanted my friends to know why I had certain peculiar habits and limitations. Gradu- ally I widened the college group to whom I had ex- plained CF. I am sure some idea of my problems became generally known. During 19821 decided I did not care who knew because I had been sick and in the hospital so much. The CF had taken over and was an integral part of my life. Now I tell people whom I expect to be aquaintances early on if the atmosphere is right. This metamorphosis parallels my mental de- velopment. As I got stronger dealing with CF, more people knew. I do not trumpet the disease around, and I do not like being gossiped about. I believe in telling the truth--l ife is too short to bandy much else around.

Explaining CF's peculiar habits and limitations to friends is necessary and is like laying out the bound- aries of a baseball field. I am saying, "This is where I can play." To be responsible and disciplined in the disease is to have many short fences with still shorter ones being built. I mentioned staying up late in col- lege. I could almost feel the fence against my chest with my arms reaching over wanting to participate. I felt restrained from doing some things: outside, dif- ferent from other people. Sometimes though, I might make a quick foray over the wall. I know some spend much time over the wall. However the fence is there to keep me from falling into a chasm or pit. Some- times a crack yawns up where there is no fence. When I recover, then there is a new fence.

"How do you do it?" is a question often asked. That is a very good question. The process begins with conflict. Me versus the diagnosis; me versus a hospitalization; me versus a physical limitation like no running; me versus a pnuemothorax. The next step is to evaluate what is actually happening. This may begin with a visceral feeling or the observation of another, like the doctor. Expressing the feeling or problem in words happens next. Then I have a han- dle on it, and I can talk about it with others. Various people contribute ideas or a turn of phrase that cap- tures the feeling. Next these identified feelings get mulled over in my mind, and I find acceptance eventually. I may not agree; maybe I do not like it. Nonetheless that reality or viewpoint is what I have arrived at. Next I must live with this new view or new restriction. Always the biggest, worst thing is that really there is not a damn thing we can do about CF.

While growing up with the disease, this process happens more frequently and much more dramat-

ically. Maturing came by having many hospitaliza- tions and procedures. Each time one of these hap- pens, I still tear down part of my mental structure and rebuild or add a new part. I liken it to a centuries- old castle. Ramparts are breached and fixed up, first temporarily then permanently. New walls and tow- ers, higher and thicker, are built. New built on old. Some areas become obsolete, to be replaced with new ideas for new structure. I get to the acceptance stage faster now. The foundation of my castle, though, has to surround the idea that ultimately I get worse. I can be stable for a while, but I never go up a level--just gradually down. I have been in the hospi- tal eleven days and then back again 34 hours later with massive hemoptysis.

I always kind of went ahead and did not look or listen at either side. I did not think much of the castle building. It has been in this last year that people made me turn to look at my castle, which is now substantial. I have been surprised by how I have touched others. Many have helped to build it in the definition and discussion phases. I have affected oth- ers who just look at my castle. I think God held the castle plans all the time.

The maturing of disease and frame of mind exact their toll. I call it growing old early. It seems some symptoms now are much like old age. I have gone through the sadness of decaying physical abilities. I cannot be physically active; I walk slowly, have to sit clown a lot, take a nap every day. My mental abilities seemed to have faltered a little. Most of all I am facing the end of life. College was my working years before it ended abruptly. Now is my retirement. Retirement is hard when you are young and you cannot cross the fences. Projecting a life goal, living it, having the goal altered by luck and by fate, accomplishing that goal, and then reflecting on what has been done. That is life. That is a life compressed for us; CF is myopic. We cannot look through the long vista of life. That is disappointing, frustrating, and cursed at. We can live now. We can do the best we can. Set goals that fit on our playing field and accomplish them. Be joyful at what we have, or more importantly at who we have and who we touch.

Kermit the Frog used to sing a song on Sesame Street called "Its Not East Being Green." It's message seems appropriate especially after a hard time. The last line is "That's what I want to be." I would change it to "'That's what I am to be." To be like the toy cloth caterpillar a nurse gave me. When it is turned inside out, the caterpillar becomes a beautiful butterfly.