Insert

Important information for people living with myelodysplastic syndromes

Fighting Bone Marrow Failure Diseases Through Patient Support and Research Since 1983www.AAMDS.org

100 Park Ave., Suite 108 | Rockville, MD 20850

Insert

continued

Interviews with the Experts

the ConnectionMDS

Fighting Bone Marrow Failure Diseases Through Patient Support and Research Since 1983 www.AAMDS.org

Understanding Prognosis in MDS

Richard Stone, MDDana-Farber Cancer Institute Richard Stone, MD is Director of the Adult Acute Leukemia Program at the Dana-Farber Cancer Institute and professor of medicine at Harvard Medical School, both in Boston, Massachusetts. He also serves as Chairman of the AA&MDSIF Medical Advisory Board. In this May 2011

interview, Dr. Stone responds to questions about prognosis in MDS—what it is, what MDS prognosis means, what prognostic tools are used, and how prognosis is used to predict and plan for potential outcomes.

Can you explain what myelodysplastic syndromes (MDS) are?

Myelodysplastic syndromes are a group of diseases that affect bone marrow output. It’s a heterogeneous group of diseases, meaning that one patient’s MDS is often different from another patient’s MDS. Some types are relatively mild, where a patient can live for years, and others are quite severe and dangerous, such that prospects for long-term survival can be poor. What they have in common is ineffective bone marrow function, where the marrow is trying to make the correct amount of all blood cells as evidenced by the high degree of cellular activity—red blood cells, white blood cells, and platelets—but is unable to do so.

What does prognosis mean?

Prognosis means a guess as to how a patient is going to fare in the future based on present conditions. Predicting the future is always diffi cult, but it is important to try to do this for patients with potentially serious diseases like MDS. We want to be able to tell patients what they may be able to in terms of upcoming problems as well as estimating survival duration. So-called prognostic features or ‘prognostic index’ represents a tool that doctors use to relatively accurately inform patients about expectations. The prognosis is also used to develop a treatment plan. For example, a poor prognosis may suggest a more risky therapy, such as a stem cell transplant, be tried. A better prognosis that indicates a longer life expectancy may infl uence a decision to stay with a safer approach.

Important Information for People Living with Myelodysplastic Syndromes

Can you discuss the International Prognostic Scoring System (IPSS)? How do doctors use it?

The IPSS was devised in 1998—after review of a large panel of MDS patients—which determined which specifi c disease features were important for prognosis The developers of the IPSS determined there were three things needed to help determine prognosis—the percentage of bone marrow blasts (immature white blood cells), the number of cytopenias (low blood counts), and the bone marrow karyotype (the chromosomal analysis of bone marrow that is made at the time of diagnosis). From these, a score is formulated that estimates the likelihood of disease and survival.

What are bone marrow blasts?

These are young cells that are immature, but have the capacity to eventually give rise to the functional blood cells—red, white, and platelets. It’s diffi cult to tell a cancerous immature cell or blast from a healthy normal one. So if there are too many immature cells in the bone marrow, that indicates a problem. Ideally, blasts should comprise less than 5% of all marrow cells.

Are there any problems with the IPSS?

There are a few problems. One is that it is relatively old, given the development of new more effective therapies since 1998 when it was introduced. It was developed based on patients in the 1980s and 1990s. New drug treatments have been introduced since then, like lenalidomide (Revlimid®) and the hypomethylating agents—azacitidine (Vidaza®) and decitabine (Dacogen®)—and IPSS doesn’t take the effect of these into account. Another drawback is that it is not dynamic; that is, it is determined at diagnosis and doesn’t take into account events and treatments that subsequently occur. Also, it doesn’t apply to patients with prior hematologic abnormalities or prior treatment for other cancers. Finally, it doesn’t include an assessment of overall health, marrow functional status, or other non-MDS

the ConnectionMDS

FREE PHONE SUPPORT FOR MDS PATIENTS, FAMILIES, AND CAREGIVERSDo you need to speak with someone directly

about myelodysplastic syndromes (MDS)?

Please contact Leigh Clark, our Patient Educator, at (800)747-2820, option 1, or by email at info@aamds.org. Leigh communicates with people all over the world, answering a wide range of questions about MDS, including information on treatment options, clinical trials, fi nancial resources, and more.

PEER SUPPORT NETWORKLet this AA&MDSIF resource help you!

The Peer Support Network is a national network of trained volunteers, including patients, caregivers, and family members, who offer information, personal experience, coping strategies, problem solving skills and informational resources to people just like themselves. Speaking with a Peer Support Network volunteer is a great way to gather information and receive emotional support from someone whose life has also been affected by bone marrow failure disease.

To connect with a Peer Support Network volunteer, call (800) 747-2820, option 1, and speak with our patient educator, Leigh Clark, who will match you with one of our volunteers. You can also email her at info@aamds.org.

Help is Here for MDS Patients

answerssupport&h pe

NO INTERNET ACCESS AT HOME? Internet-connected computers are found in many locations, including:

• Retirement homes • Apartment community rooms • Public libraries • Senior centers

And it’s almost certain you know someone (relatives, neighbors, friends) who is connected!

Connect with us at www.AAMDS.org!

Contact Ushelp@aamds.org (301) 279-7202 or (800) 747-2820

ISSUE 8, MARCH 2012

• Educational materials Read Your Guide to Understanding MDS, a free patient guide that explains in plain language what you need to know to understand MDS—why it happens, what to do about it, how to receive your best care—and tips for living well with MDS. Also available is What to Expect from Treatment: A Guide to Understanding FDA-Approved Drug Therapies for Myelodysplastic Syndromes (MDS).

• Print and electronic news Stay current with the latest information on areas relevant to

MDS through our newsletters: Insider (print) and eInsider (electronic).

• Patient connectionsConnect in person at a regional Community of Hope event (for more information, please contact crews@aamds.org).

• AA&MDSIF events Registration for 2012 AA&MDSIF Patient and Family Conferences is now open! Learn all the details and register at www.AAMDS.org/Conferences

www.AAMDS.org – Your best resource for medical and personal perspectives on myelodysplastic syndromes (MDS)

Aplastic Anemia & MDS International Foundation

Find Us on Facebook at www.facebook.com/aamds

Connect online with other MDS patients

Here’s what attendees of the 2011 conferences had to say about their experience:

Thank you for all your hard work in putting these conferences together for us. It was an amazing and wonderful experience for me and I will make sure to attend next year.Leslie, MDS patient

I really enjoyed your conference. It answered a lot of questions for my daughter and me. We left your conference feeling great. WE LOVED IT! Keep up the good work because you do make a difference.Diana, mother of aplastic anemia patient

Great job! Very supportive and hopeful atmosphere. Wonderful presentations of practical and meaningful information. Please receive our sincere thanks for your vital and life-giving work.Lon, PNH patient

I have attended many, many conferences in the last 50 years on many subjects, and this was the most organized and informative of them all!Wayne, MDS patient

I was impressed with the conference, the staff, volunteers, the warmth and friendliness of everyone. It was a well-thought out conference.Gayle, aplastic anemia patient

Meet more patients and families, take home more expert information!

When you attend one of our conferences, here is what you can expect: Answers to your medical and treatment questions The morning sessions include concurrent tracks on advances in research and treatment methods for each disease—aplastic anemia, MDS, and PNH. During these sessions, participants will have unprecedented access to the nation’s leading bone marrow failure disease experts, with plenty of time for everyone’s questions to be answered.

Sessions on Living WellThe afternoon will include a series of Living Well sessions (this will vary by location) on topics including managing fatigue, complementary and alternative medicine, nutrition, and exercise, coping skills for maintaining emotional health, and survivorship. Three sessions will be offered at each location.

New for 2012!Support forums to help you connect, share, and learn from others These professionally-facilitated support sessions will bring together patients and family members with similar issues and concerns. They provide plenty of time for talking, sharing, comparing, and hearing how others are coping with the challenges of living with a bone marrow failure disease. Sessions are broken out by disease type. A special session for caregivers only will also be included. Sessions for parents of pediatric patients will be provided at our programs in New York, Atlanta, and Los Angeles.

Hope for the futureEach conference concludes with a powerful and uplifting Celebration of Hope. This joyous gathering celebrates life and friendship. The program includes a survivor spotlight story, highlighting the struggles and triumphs of one of the many long-term bone marrow failure disease survivors who is successfully living with and beyond their illness. We also include a special presentation, including pictures, quotes, and comments provided by conference participants in the weeks leading up to each conference. Be sure to come and celebrate your life and your courage with us.

Make plans to attend…register now!Our 2012 conferences will provide answers, support, and hope for every patient, family member, and caregiver whose lives have been touched by bone marrow failure disease. If you’ve never attended one of our conferences, you won’t want to miss this informative, inspirational, and uplifting event.

ISSUE 8, MARCH 2012 • APLASTIC ANEMIA & MDS INTERNATIONAL FOUNDATIONSupported by an unrestricted education grant from Celgene Corporation.

Realistic Expectations and Faith Keep a Survivor Facing Forward

Understanding Prognosis in MDS continued from cover

I’m Arthur Henry, and I turn 71 in March 2012. I have MDS, specifi cally the subtype known as refractory anemia with ring sideroblasts (RARS). Over the years, I’ve told and updated my story on the AA&MDSIF Web site. Recently I was asked to retell it for The MDS Connection.

Diagnosis and reaction

About sixteen years ago, I felt that I was in great health. A routine checkup, followed by many tests and

then a bone marrow biopsy, yielded a RARS diagnosis, which at that time had a median survival of 2 to 5 years. That staggered me. I couldn’t believe it, because of how good I still felt and the healthy lifestyle I’d lived.

I worked at coming to terms with the likely brevity of my remaining life; one with abilities impaired beyond those from normal aging. I read self-help books and went to a psychologist. As a former research scientist, I had always considered the possibility that I would someday pay a price for years of chemical exposure (it is one possible condition that led to my disease); still, I was unprepared for the reality of it being true. One consoling factor was that despite there being myriad diseases that end our lives, there are only a few ways we die. That changed my distress from “Why me?” into “If not MDS, then something else” and helped me get a better grasp on my mortality.

Faith as foundational support

My most important aids, though, were not anything I read or thought about. First was and is my unshakeable belief in God. I believe that there is a reason for everything, including my illness, which I accepted as “His will”, but also wished I didn’t have. Belief does not cure anything—though it may by some miracle we cannot fathom—but it does lead to an attitude of acceptance and gratitude for what we do have. Both are keys to survival.

From that change in attitude, my relationship with my girlfriend, Suzy, was able to advance. We’d begun talking about marriage, but after my diagnosis, I sought to undo that. Suzy was a lot younger than I. If there were no future for me, there’d be none with me—and I didn’t want to deprive her of a normal life with a healthy spouse. Suzy, however, insisted on going forward. We were married a few months later and accepted what time we had. In January 2012, we celebrated our fi fteenth anniversary. I am most blessed!

The Patient Perspective

the ConnectionMDS

Facts and fi gures

Before addressing how we live with my MDS, I want tocover some practical issues regarding my condition and its treatment:

• My RARS is a slow-progressing MDS; it could have been worse.

• Surprisingly to me, things were little changed in how I felt for quite some time. Seven years post-diagnosis, though, treatment had to begin when my hematocrit (HCT) and hemoglobin (Hgb) counts got too low for safety.

• My hematologist tried erythropoietin (Procrit®), an erythropoiesis-stimulating agent (ESA) which works in only a small percentage of RARS patients, and then usually when taken in higher doses than I received – which were 20-60,000 units weekly, depending on my combined blood count (CBC). That kept my HCT in the mid 30s and my Hgb near 11.

• That worked for seven-and-a-half years, far longer than for most patients. Then, one-and-a-half years ago, I was switched to Aranesp® when Procrit® was recalled. A few months of juggling doses and timing got me stable again, if with a bit lower counts.

• That ESAs work so well for me is likely aided by my hemachromatosis—discovered after my MDS diagnosis. This life-threatening genetic disease places too much iron around my body and is managed well by phlebotomies—an “ironic” situation for someone with anemia!

Attitudes, activities, and adjustments

People have options as to how they will approach the disease. They can remain in a defeated state as I felt right after my diagnosis—dying in place, missing their remaining life. Some others may ignore their diagnosis, living an unchanged life. That is unwise because some concessions are necessary, such as avoidance of chemical exposure, avoidance of stress, and a greater avoidance of others’ illnesses. Others still may choose to make MDS the center of their existence by spending excessive time on researching treatments, becoming overly concerned with their illness. And then there’s the way I live. Barring an automobile accident, my MDS will likely take my life, and I am aware of that. It is a part of what makes me who I am, but it is not my identity.

There are compromises Suzy and I make, and most are no big deal in the overall scheme of things:

• My weekly treatments have no foreseeable end, and I must organize my life around those visits; long travel is diffi cult, often practically impossible.

Learn more about MDS at www.AAMDS.org/Learn• MDS Treatment: Options and Issues• Treating Lower-Risk MDS• Beating Fatigue• Survivorship• Living Well with MDS: A Focus on Emotional Health

diseases beyond the three defi ning criteria I mentioned earlier. For example, the need for red cells could infl uence prognosis for patients with a particular IPSS score.

What are some of the newer prognostic tools?

There are some newer, improved prognostic scoring systems that have been developed, including the World Health Organization Prognostic Scoring System (WPSS). The WPSS takes advantage of the fact that the classifi cation categories of MDS have changed. There have been refi nements in the different MDS subtypes based on the way the bone marrow looks—how many cell lines are dysplastic, that is, how odd they appear under the microscope. WPSS also takes into account whether the patient is having red cell transfusions, and the IPSS did not. A transfusion is an independent prognostic factor that matters—patients not needing transfusions are a little better off than those who do, even controlling for other known prognostic factors. Pathology, morphology, and the signifi cance of transfusions are all part of the changes in the WPSS. It may be able to deliver a more accurate estimate of prognosis than the IPSS. The IPSS has recently been refi ned as well to take into account a wider range of chromosomal abnormalities. Also, the MD Anderson Cancer Center has a prognostic scoring system that recently is becoming more widely used because it takes into account the patient’s performance status (overall health) that sometimes trumps the hematological issues.

When a patient gets diagnosed with MDS, what should they be asking their doctor about their prognosis?

Patients should take into account the best possible prognosis, perhaps employing several classifi cation systems. Such an effort could aid a patient’s understanding of their particular disease state. This is a good opportunity for the doctor to remind the patient that these prognostic scoring systems are just a gross estimate of how a patient will do. A patient with a higher risk presentation of MDS and an adverse prognosis can still live a long time, since estimates are just that.

Compared to what may eventually exist in assessing MDS prognosis, what we have now is very rudimentary. Our increased understanding of the genetic underpinnings of MDS may allow a more accurate prognostic scoring system to be developed. While patients will understandably be concerned at the prospect of a shortened life expectancy, they should realize everyone’s situation will be different, especially given the heterogeneity of MDS. They should focus on learning everything they can about the disease, establish a good relationship with their doctor, and try to stay abreast of new developments in this disease. The AA&MDSIF Web site can be an excellent source of information.

If we have helped you or your loved ones…

please help us assist others!Join patients, families and friends

helping AA&MDSIF todayMake your gift online at www.AAMDS.org/DonateNow

or send your gift to AA&MDSIF at 100 Park Avenue, Suite 108, Rockville, MD 20850.Thank you for getting involved and giving back!

• A serious amateur artist, I gave up painting because of its chemical exposure… so I switched to needlepoint and computer graphics.

• We avoid unnecessary chemicals around the house—especially cleaners. I don’t put gas in our cars and our mower is electric.

• I sleep a lot more than I did, and I am honest about what I can’t do for it “taking too much out of me”—especially heavily stress-related activities; that’s hard to do for the type A personality I’d been all my life.

I begrudge none of these, grateful every morning to wake up able to do much of what I want. And as that diminishes—it does—I stay grateful for what I still can do, offering up to Him every morning (with Suzy, as the alarm sounds—she still works), all our actions that day in thanks.

Living life each day—from a new degree, to time on the track, and “happy regrets”

What does our “living with MDS” actually look like? I was already in ESA treatment when I began and completed my Master’s degree in theology. Today I lecture and write on current-event issues relating

to science and morality, among other topics that meld my science and spiritual training.

And I just drove—Suzy’s present for my 70th birthday—a NASCAR racer for 30 1.5-mile laps at speeds over 140mph! We also celebrated in July a 120-year party (my 70th, Suzy’s 50th birthdays) and also celebrated some “regrets.” Those regrets? When I was originally diagnosed, I made some fi nancial decisions based on an expected shorter life. I calculated that if I lived till 70, I would really regret those decisions… but would be around to celebrate my mistakes. And so, we celebrated.

Patient Perspective continued

March 24 WASHINGTON, DC April 21 LOS ANGELES, CA May 19 ATLANTA, GA July 28 NEW YORK CITY AREA September 22 DALLAS, TX October 20 CHICAGO, IL

June 16 SEATTLE, WAIn addition, AA&MDSIF is co-hosting a conference with the Fred Hutchinson Cancer Research Center on June 16 in Seattle, WA. This conference format will differ from the other six conferences. See Seattle conference Web site for agenda details.

2012 Regional Patient and Family Conferences

Building on the success and strong attendance at the 2011 Patient and Family Conferences, AA&MDSIF announces its conference series for 2012, now set for six metropolitan areas.

MARK YOUR CALENDAR NOW FOR THE LOCATION NEAREST YOU!

AA&MDSIF Patient and Family Conferences Have you ever met another person with aplastic anemia, MDS or PNH? Seven out of ten conference attendees had never met another person diagnosed with aplastic anemia, MDS or PNH until they connected with them at one of our conferences.

If you haven’t attended one of our Living with Aplastic Anemia, MDS or PNH patient and family conferences, mark your calendar now to attend! Patients will have the opportunity to attend a conference within just a few months of their diagnosis. The one-day format makes it easy for family members to participate at a time that’s convenient to school and work schedules.

Attendees will fi nd several enhancements to the 2012 conference program. These changes, based on input and suggestions received from attendees and presenters, are designed to increase the value of these conferences. Although the core program remains the same, our 2012 programs will focus on providing even more opportunity for participants to connect and share, along with some specifi c programming just for caregivers.

LIVING WITH APLASTIC ANEMIA, MDS OR PNH

• Hear from leading experts. Learn more about your diseases, current treatments and emerging therapies.

• Explore a variety of topics on living well and improving your quality of life.

• Get your questions answered. Plenty of time will be provided in every session.

• Learn skills and strategies that can help you stand up for your best medical care and become a more powerful patient.

• Meet other patients and family members and get connected to a support network.

• Participate in our Celebration of Hope and leave with a sense of hope and a positive outlook for the future.

• Understand how AA&MDSIF can help you through this life-changing experience.

• Like any AA&MDSIF live event, registration is free and includes meals and all materials. However, advance registration is required. For more information, and to register, visit www.AAMDS.org/Conferences.

Why Should You Attend One of Our Conferences?

For more informationwww.AAMDS.org/Conferences

ISSUE 8, MARCH 2012 • APLASTIC ANEMIA & MDS INTERNATIONAL FOUNDATIONSupported by an unrestricted education grant from Celgene Corporation.

Realistic Expectations and Faith Keep a Survivor Facing Forward

Understanding Prognosis in MDS continued from cover

I’m Arthur Henry, and I turn 71 in March 2012. I have MDS, specifi cally the subtype known as refractory anemia with ring sideroblasts (RARS). Over the years, I’ve told and updated my story on the AA&MDSIF Web site. Recently I was asked to retell it for The MDS Connection.

Diagnosis and reaction

About sixteen years ago, I felt that I was in great health. A routine checkup, followed by many tests and

then a bone marrow biopsy, yielded a RARS diagnosis, which at that time had a median survival of 2 to 5 years. That staggered me. I couldn’t believe it, because of how good I still felt and the healthy lifestyle I’d lived.

I worked at coming to terms with the likely brevity of my remaining life; one with abilities impaired beyond those from normal aging. I read self-help books and went to a psychologist. As a former research scientist, I had always considered the possibility that I would someday pay a price for years of chemical exposure (it is one possible condition that led to my disease); still, I was unprepared for the reality of it being true. One consoling factor was that despite there being myriad diseases that end our lives, there are only a few ways we die. That changed my distress from “Why me?” into “If not MDS, then something else” and helped me get a better grasp on my mortality.

Faith as foundational support

My most important aids, though, were not anything I read or thought about. First was and is my unshakeable belief in God. I believe that there is a reason for everything, including my illness, which I accepted as “His will”, but also wished I didn’t have. Belief does not cure anything—though it may by some miracle we cannot fathom—but it does lead to an attitude of acceptance and gratitude for what we do have. Both are keys to survival.

From that change in attitude, my relationship with my girlfriend, Suzy, was able to advance. We’d begun talking about marriage, but after my diagnosis, I sought to undo that. Suzy was a lot younger than I. If there were no future for me, there’d be none with me—and I didn’t want to deprive her of a normal life with a healthy spouse. Suzy, however, insisted on going forward. We were married a few months later and accepted what time we had. In January 2012, we celebrated our fi fteenth anniversary. I am most blessed!

The Patient Perspective

the ConnectionMDS

Facts and fi gures

Before addressing how we live with my MDS, I want tocover some practical issues regarding my condition and its treatment:

• My RARS is a slow-progressing MDS; it could have been worse.

• Surprisingly to me, things were little changed in how I felt for quite some time. Seven years post-diagnosis, though, treatment had to begin when my hematocrit (HCT) and hemoglobin (Hgb) counts got too low for safety.

• My hematologist tried erythropoietin (Procrit®), an erythropoiesis-stimulating agent (ESA) which works in only a small percentage of RARS patients, and then usually when taken in higher doses than I received – which were 20-60,000 units weekly, depending on my combined blood count (CBC). That kept my HCT in the mid 30s and my Hgb near 11.

• That worked for seven-and-a-half years, far longer than for most patients. Then, one-and-a-half years ago, I was switched to Aranesp® when Procrit® was recalled. A few months of juggling doses and timing got me stable again, if with a bit lower counts.

• That ESAs work so well for me is likely aided by my hemachromatosis—discovered after my MDS diagnosis. This life-threatening genetic disease places too much iron around my body and is managed well by phlebotomies—an “ironic” situation for someone with anemia!

Attitudes, activities, and adjustments

People have options as to how they will approach the disease. They can remain in a defeated state as I felt right after my diagnosis—dying in place, missing their remaining life. Some others may ignore their diagnosis, living an unchanged life. That is unwise because some concessions are necessary, such as avoidance of chemical exposure, avoidance of stress, and a greater avoidance of others’ illnesses. Others still may choose to make MDS the center of their existence by spending excessive time on researching treatments, becoming overly concerned with their illness. And then there’s the way I live. Barring an automobile accident, my MDS will likely take my life, and I am aware of that. It is a part of what makes me who I am, but it is not my identity.

There are compromises Suzy and I make, and most are no big deal in the overall scheme of things:

• My weekly treatments have no foreseeable end, and I must organize my life around those visits; long travel is diffi cult, often practically impossible.

Learn more about MDS at www.AAMDS.org/Learn• MDS Treatment: Options and Issues• Treating Lower-Risk MDS• Beating Fatigue• Survivorship• Living Well with MDS: A Focus on Emotional Health

diseases beyond the three defi ning criteria I mentioned earlier. For example, the need for red cells could infl uence prognosis for patients with a particular IPSS score.

What are some of the newer prognostic tools?

There are some newer, improved prognostic scoring systems that have been developed, including the World Health Organization Prognostic Scoring System (WPSS). The WPSS takes advantage of the fact that the classifi cation categories of MDS have changed. There have been refi nements in the different MDS subtypes based on the way the bone marrow looks—how many cell lines are dysplastic, that is, how odd they appear under the microscope. WPSS also takes into account whether the patient is having red cell transfusions, and the IPSS did not. A transfusion is an independent prognostic factor that matters—patients not needing transfusions are a little better off than those who do, even controlling for other known prognostic factors. Pathology, morphology, and the signifi cance of transfusions are all part of the changes in the WPSS. It may be able to deliver a more accurate estimate of prognosis than the IPSS. The IPSS has recently been refi ned as well to take into account a wider range of chromosomal abnormalities. Also, the MD Anderson Cancer Center has a prognostic scoring system that recently is becoming more widely used because it takes into account the patient’s performance status (overall health) that sometimes trumps the hematological issues.

When a patient gets diagnosed with MDS, what should they be asking their doctor about their prognosis?

Patients should take into account the best possible prognosis, perhaps employing several classifi cation systems. Such an effort could aid a patient’s understanding of their particular disease state. This is a good opportunity for the doctor to remind the patient that these prognostic scoring systems are just a gross estimate of how a patient will do. A patient with a higher risk presentation of MDS and an adverse prognosis can still live a long time, since estimates are just that.

Compared to what may eventually exist in assessing MDS prognosis, what we have now is very rudimentary. Our increased understanding of the genetic underpinnings of MDS may allow a more accurate prognostic scoring system to be developed. While patients will understandably be concerned at the prospect of a shortened life expectancy, they should realize everyone’s situation will be different, especially given the heterogeneity of MDS. They should focus on learning everything they can about the disease, establish a good relationship with their doctor, and try to stay abreast of new developments in this disease. The AA&MDSIF Web site can be an excellent source of information.

If we have helped you or your loved ones…

please help us assist others!Join patients, families and friends

helping AA&MDSIF todayMake your gift online at www.AAMDS.org/DonateNow

or send your gift to AA&MDSIF at 100 Park Avenue, Suite 108, Rockville, MD 20850.Thank you for getting involved and giving back!

• A serious amateur artist, I gave up painting because of its chemical exposure… so I switched to needlepoint and computer graphics.

• We avoid unnecessary chemicals around the house—especially cleaners. I don’t put gas in our cars and our mower is electric.

• I sleep a lot more than I did, and I am honest about what I can’t do for it “taking too much out of me”—especially heavily stress-related activities; that’s hard to do for the type A personality I’d been all my life.

I begrudge none of these, grateful every morning to wake up able to do much of what I want. And as that diminishes—it does—I stay grateful for what I still can do, offering up to Him every morning (with Suzy, as the alarm sounds—she still works), all our actions that day in thanks.

Living life each day—from a new degree, to time on the track, and “happy regrets”

What does our “living with MDS” actually look like? I was already in ESA treatment when I began and completed my Master’s degree in theology. Today I lecture and write on current-event issues relating

to science and morality, among other topics that meld my science and spiritual training.

And I just drove—Suzy’s present for my 70th birthday—a NASCAR racer for 30 1.5-mile laps at speeds over 140mph! We also celebrated in July a 120-year party (my 70th, Suzy’s 50th birthdays) and also celebrated some “regrets.” Those regrets? When I was originally diagnosed, I made some fi nancial decisions based on an expected shorter life. I calculated that if I lived till 70, I would really regret those decisions… but would be around to celebrate my mistakes. And so, we celebrated.

Patient Perspective continued

March 24 WASHINGTON, DC April 21 LOS ANGELES, CA May 19 ATLANTA, GA July 28 NEW YORK CITY AREA September 22 DALLAS, TX October 20 CHICAGO, IL

June 16 SEATTLE, WAIn addition, AA&MDSIF is co-hosting a conference with the Fred Hutchinson Cancer Research Center on June 16 in Seattle, WA. This conference format will differ from the other six conferences. See Seattle conference Web site for agenda details.

2012 Regional Patient and Family Conferences

Building on the success and strong attendance at the 2011 Patient and Family Conferences, AA&MDSIF announces its conference series for 2012, now set for six metropolitan areas.

MARK YOUR CALENDAR NOW FOR THE LOCATION NEAREST YOU!

AA&MDSIF Patient and Family Conferences Have you ever met another person with aplastic anemia, MDS or PNH? Seven out of ten conference attendees had never met another person diagnosed with aplastic anemia, MDS or PNH until they connected with them at one of our conferences.

If you haven’t attended one of our Living with Aplastic Anemia, MDS or PNH patient and family conferences, mark your calendar now to attend! Patients will have the opportunity to attend a conference within just a few months of their diagnosis. The one-day format makes it easy for family members to participate at a time that’s convenient to school and work schedules.

Attendees will fi nd several enhancements to the 2012 conference program. These changes, based on input and suggestions received from attendees and presenters, are designed to increase the value of these conferences. Although the core program remains the same, our 2012 programs will focus on providing even more opportunity for participants to connect and share, along with some specifi c programming just for caregivers.

LIVING WITH APLASTIC ANEMIA, MDS OR PNH

• Hear from leading experts. Learn more about your diseases, current treatments and emerging therapies.

• Explore a variety of topics on living well and improving your quality of life.

• Get your questions answered. Plenty of time will be provided in every session.

• Learn skills and strategies that can help you stand up for your best medical care and become a more powerful patient.

• Meet other patients and family members and get connected to a support network.

• Participate in our Celebration of Hope and leave with a sense of hope and a positive outlook for the future.

• Understand how AA&MDSIF can help you through this life-changing experience.

• Like any AA&MDSIF live event, registration is free and includes meals and all materials. However, advance registration is required. For more information, and to register, visit www.AAMDS.org/Conferences.

Why Should You Attend One of Our Conferences?

For more informationwww.AAMDS.org/Conferences

ISSUE 8, MARCH 2012 • APLASTIC ANEMIA & MDS INTERNATIONAL FOUNDATIONSupported by an unrestricted education grant from Celgene Corporation.

Realistic Expectations and Faith Keep a Survivor Facing Forward

Understanding Prognosis in MDS continued from cover

I’m Arthur Henry, and I turn 71 in March 2012. I have MDS, specifi cally the subtype known as refractory anemia with ring sideroblasts (RARS). Over the years, I’ve told and updated my story on the AA&MDSIF Web site. Recently I was asked to retell it for The MDS Connection.

Diagnosis and reaction

About sixteen years ago, I felt that I was in great health. A routine checkup, followed by many tests and

then a bone marrow biopsy, yielded a RARS diagnosis, which at that time had a median survival of 2 to 5 years. That staggered me. I couldn’t believe it, because of how good I still felt and the healthy lifestyle I’d lived.

I worked at coming to terms with the likely brevity of my remaining life; one with abilities impaired beyond those from normal aging. I read self-help books and went to a psychologist. As a former research scientist, I had always considered the possibility that I would someday pay a price for years of chemical exposure (it is one possible condition that led to my disease); still, I was unprepared for the reality of it being true. One consoling factor was that despite there being myriad diseases that end our lives, there are only a few ways we die. That changed my distress from “Why me?” into “If not MDS, then something else” and helped me get a better grasp on my mortality.

Faith as foundational support

My most important aids, though, were not anything I read or thought about. First was and is my unshakeable belief in God. I believe that there is a reason for everything, including my illness, which I accepted as “His will”, but also wished I didn’t have. Belief does not cure anything—though it may by some miracle we cannot fathom—but it does lead to an attitude of acceptance and gratitude for what we do have. Both are keys to survival.

From that change in attitude, my relationship with my girlfriend, Suzy, was able to advance. We’d begun talking about marriage, but after my diagnosis, I sought to undo that. Suzy was a lot younger than I. If there were no future for me, there’d be none with me—and I didn’t want to deprive her of a normal life with a healthy spouse. Suzy, however, insisted on going forward. We were married a few months later and accepted what time we had. In January 2012, we celebrated our fi fteenth anniversary. I am most blessed!

The Patient Perspective

the ConnectionMDS

Facts and fi gures

Before addressing how we live with my MDS, I want tocover some practical issues regarding my condition and its treatment:

• My RARS is a slow-progressing MDS; it could have been worse.

• Surprisingly to me, things were little changed in how I felt for quite some time. Seven years post-diagnosis, though, treatment had to begin when my hematocrit (HCT) and hemoglobin (Hgb) counts got too low for safety.

• My hematologist tried erythropoietin (Procrit®), an erythropoiesis-stimulating agent (ESA) which works in only a small percentage of RARS patients, and then usually when taken in higher doses than I received – which were 20-60,000 units weekly, depending on my combined blood count (CBC). That kept my HCT in the mid 30s and my Hgb near 11.

• That worked for seven-and-a-half years, far longer than for most patients. Then, one-and-a-half years ago, I was switched to Aranesp® when Procrit® was recalled. A few months of juggling doses and timing got me stable again, if with a bit lower counts.

• That ESAs work so well for me is likely aided by my hemachromatosis—discovered after my MDS diagnosis. This life-threatening genetic disease places too much iron around my body and is managed well by phlebotomies—an “ironic” situation for someone with anemia!

Attitudes, activities, and adjustments

People have options as to how they will approach the disease. They can remain in a defeated state as I felt right after my diagnosis—dying in place, missing their remaining life. Some others may ignore their diagnosis, living an unchanged life. That is unwise because some concessions are necessary, such as avoidance of chemical exposure, avoidance of stress, and a greater avoidance of others’ illnesses. Others still may choose to make MDS the center of their existence by spending excessive time on researching treatments, becoming overly concerned with their illness. And then there’s the way I live. Barring an automobile accident, my MDS will likely take my life, and I am aware of that. It is a part of what makes me who I am, but it is not my identity.

There are compromises Suzy and I make, and most are no big deal in the overall scheme of things:

• My weekly treatments have no foreseeable end, and I must organize my life around those visits; long travel is diffi cult, often practically impossible.

Learn more about MDS at www.AAMDS.org/Learn• MDS Treatment: Options and Issues• Treating Lower-Risk MDS• Beating Fatigue• Survivorship• Living Well with MDS: A Focus on Emotional Health

diseases beyond the three defi ning criteria I mentioned earlier. For example, the need for red cells could infl uence prognosis for patients with a particular IPSS score.

What are some of the newer prognostic tools?

There are some newer, improved prognostic scoring systems that have been developed, including the World Health Organization Prognostic Scoring System (WPSS). The WPSS takes advantage of the fact that the classifi cation categories of MDS have changed. There have been refi nements in the different MDS subtypes based on the way the bone marrow looks—how many cell lines are dysplastic, that is, how odd they appear under the microscope. WPSS also takes into account whether the patient is having red cell transfusions, and the IPSS did not. A transfusion is an independent prognostic factor that matters—patients not needing transfusions are a little better off than those who do, even controlling for other known prognostic factors. Pathology, morphology, and the signifi cance of transfusions are all part of the changes in the WPSS. It may be able to deliver a more accurate estimate of prognosis than the IPSS. The IPSS has recently been refi ned as well to take into account a wider range of chromosomal abnormalities. Also, the MD Anderson Cancer Center has a prognostic scoring system that recently is becoming more widely used because it takes into account the patient’s performance status (overall health) that sometimes trumps the hematological issues.

When a patient gets diagnosed with MDS, what should they be asking their doctor about their prognosis?

Patients should take into account the best possible prognosis, perhaps employing several classifi cation systems. Such an effort could aid a patient’s understanding of their particular disease state. This is a good opportunity for the doctor to remind the patient that these prognostic scoring systems are just a gross estimate of how a patient will do. A patient with a higher risk presentation of MDS and an adverse prognosis can still live a long time, since estimates are just that.

Compared to what may eventually exist in assessing MDS prognosis, what we have now is very rudimentary. Our increased understanding of the genetic underpinnings of MDS may allow a more accurate prognostic scoring system to be developed. While patients will understandably be concerned at the prospect of a shortened life expectancy, they should realize everyone’s situation will be different, especially given the heterogeneity of MDS. They should focus on learning everything they can about the disease, establish a good relationship with their doctor, and try to stay abreast of new developments in this disease. The AA&MDSIF Web site can be an excellent source of information.

If we have helped you or your loved ones…

please help us assist others!Join patients, families and friends

helping AA&MDSIF todayMake your gift online at www.AAMDS.org/DonateNow

or send your gift to AA&MDSIF at 100 Park Avenue, Suite 108, Rockville, MD 20850.Thank you for getting involved and giving back!

• A serious amateur artist, I gave up painting because of its chemical exposure… so I switched to needlepoint and computer graphics.

• We avoid unnecessary chemicals around the house—especially cleaners. I don’t put gas in our cars and our mower is electric.

• I sleep a lot more than I did, and I am honest about what I can’t do for it “taking too much out of me”—especially heavily stress-related activities; that’s hard to do for the type A personality I’d been all my life.

I begrudge none of these, grateful every morning to wake up able to do much of what I want. And as that diminishes—it does—I stay grateful for what I still can do, offering up to Him every morning (with Suzy, as the alarm sounds—she still works), all our actions that day in thanks.

Living life each day—from a new degree, to time on the track, and “happy regrets”

What does our “living with MDS” actually look like? I was already in ESA treatment when I began and completed my Master’s degree in theology. Today I lecture and write on current-event issues relating

to science and morality, among other topics that meld my science and spiritual training.

And I just drove—Suzy’s present for my 70th birthday—a NASCAR racer for 30 1.5-mile laps at speeds over 140mph! We also celebrated in July a 120-year party (my 70th, Suzy’s 50th birthdays) and also celebrated some “regrets.” Those regrets? When I was originally diagnosed, I made some fi nancial decisions based on an expected shorter life. I calculated that if I lived till 70, I would really regret those decisions… but would be around to celebrate my mistakes. And so, we celebrated.

Patient Perspective continued

March 24 WASHINGTON, DC April 21 LOS ANGELES, CA May 19 ATLANTA, GA July 28 NEW YORK CITY AREA September 22 DALLAS, TX October 20 CHICAGO, IL

June 16 SEATTLE, WAIn addition, AA&MDSIF is co-hosting a conference with the Fred Hutchinson Cancer Research Center on June 16 in Seattle, WA. This conference format will differ from the other six conferences. See Seattle conference Web site for agenda details.

2012 Regional Patient and Family Conferences

Building on the success and strong attendance at the 2011 Patient and Family Conferences, AA&MDSIF announces its conference series for 2012, now set for six metropolitan areas.

MARK YOUR CALENDAR NOW FOR THE LOCATION NEAREST YOU!

AA&MDSIF Patient and Family Conferences Have you ever met another person with aplastic anemia, MDS or PNH? Seven out of ten conference attendees had never met another person diagnosed with aplastic anemia, MDS or PNH until they connected with them at one of our conferences.

If you haven’t attended one of our Living with Aplastic Anemia, MDS or PNH patient and family conferences, mark your calendar now to attend! Patients will have the opportunity to attend a conference within just a few months of their diagnosis. The one-day format makes it easy for family members to participate at a time that’s convenient to school and work schedules.

Attendees will fi nd several enhancements to the 2012 conference program. These changes, based on input and suggestions received from attendees and presenters, are designed to increase the value of these conferences. Although the core program remains the same, our 2012 programs will focus on providing even more opportunity for participants to connect and share, along with some specifi c programming just for caregivers.

LIVING WITH APLASTIC ANEMIA, MDS OR PNH

• Hear from leading experts. Learn more about your diseases, current treatments and emerging therapies.

• Explore a variety of topics on living well and improving your quality of life.

• Get your questions answered. Plenty of time will be provided in every session.

• Learn skills and strategies that can help you stand up for your best medical care and become a more powerful patient.

• Meet other patients and family members and get connected to a support network.

• Participate in our Celebration of Hope and leave with a sense of hope and a positive outlook for the future.

• Understand how AA&MDSIF can help you through this life-changing experience.

• Like any AA&MDSIF live event, registration is free and includes meals and all materials. However, advance registration is required. For more information, and to register, visit www.AAMDS.org/Conferences.

Why Should You Attend One of Our Conferences?

For more informationwww.AAMDS.org/Conferences

Insert

Important information for people living with myelodysplastic syndromes

Fighting Bone Marrow Failure Diseases Through Patient Support and Research Since 1983www.AAMDS.org

100 Park Ave., Suite 108 | Rockville, MD 20850

Insert

continued

Interviews with the Experts

the ConnectionMDS

Fighting Bone Marrow Failure Diseases Through Patient Support and Research Since 1983 www.AAMDS.org

Understanding Prognosis in MDS

Richard Stone, MDDana-Farber Cancer Institute Richard Stone, MD is Director of the Adult Acute Leukemia Program at the Dana-Farber Cancer Institute and professor of medicine at Harvard Medical School, both in Boston, Massachusetts. He also serves as Chairman of the AA&MDSIF Medical Advisory Board. In this May 2011

interview, Dr. Stone responds to questions about prognosis in MDS—what it is, what MDS prognosis means, what prognostic tools are used, and how prognosis is used to predict and plan for potential outcomes.

Can you explain what myelodysplastic syndromes (MDS) are?

Myelodysplastic syndromes are a group of diseases that affect bone marrow output. It’s a heterogeneous group of diseases, meaning that one patient’s MDS is often different from another patient’s MDS. Some types are relatively mild, where a patient can live for years, and others are quite severe and dangerous, such that prospects for long-term survival can be poor. What they have in common is ineffective bone marrow function, where the marrow is trying to make the correct amount of all blood cells as evidenced by the high degree of cellular activity—red blood cells, white blood cells, and platelets—but is unable to do so.

What does prognosis mean?

Prognosis means a guess as to how a patient is going to fare in the future based on present conditions. Predicting the future is always diffi cult, but it is important to try to do this for patients with potentially serious diseases like MDS. We want to be able to tell patients what they may be able to in terms of upcoming problems as well as estimating survival duration. So-called prognostic features or ‘prognostic index’ represents a tool that doctors use to relatively accurately inform patients about expectations. The prognosis is also used to develop a treatment plan. For example, a poor prognosis may suggest a more risky therapy, such as a stem cell transplant, be tried. A better prognosis that indicates a longer life expectancy may infl uence a decision to stay with a safer approach.

Important Information for People Living with Myelodysplastic Syndromes

Can you discuss the International Prognostic Scoring System (IPSS)? How do doctors use it?

The IPSS was devised in 1998—after review of a large panel of MDS patients—which determined which specifi c disease features were important for prognosis The developers of the IPSS determined there were three things needed to help determine prognosis—the percentage of bone marrow blasts (immature white blood cells), the number of cytopenias (low blood counts), and the bone marrow karyotype (the chromosomal analysis of bone marrow that is made at the time of diagnosis). From these, a score is formulated that estimates the likelihood of disease and survival.

What are bone marrow blasts?

These are young cells that are immature, but have the capacity to eventually give rise to the functional blood cells—red, white, and platelets. It’s diffi cult to tell a cancerous immature cell or blast from a healthy normal one. So if there are too many immature cells in the bone marrow, that indicates a problem. Ideally, blasts should comprise less than 5% of all marrow cells.

Are there any problems with the IPSS?

There are a few problems. One is that it is relatively old, given the development of new more effective therapies since 1998 when it was introduced. It was developed based on patients in the 1980s and 1990s. New drug treatments have been introduced since then, like lenalidomide (Revlimid®) and the hypomethylating agents—azacitidine (Vidaza®) and decitabine (Dacogen®)—and IPSS doesn’t take the effect of these into account. Another drawback is that it is not dynamic; that is, it is determined at diagnosis and doesn’t take into account events and treatments that subsequently occur. Also, it doesn’t apply to patients with prior hematologic abnormalities or prior treatment for other cancers. Finally, it doesn’t include an assessment of overall health, marrow functional status, or other non-MDS

the ConnectionMDS

FREE PHONE SUPPORT FOR MDS PATIENTS, FAMILIES, AND CAREGIVERSDo you need to speak with someone directly

about myelodysplastic syndromes (MDS)?

Please contact Leigh Clark, our Patient Educator, at (800)747-2820, option 1, or by email at info@aamds.org. Leigh communicates with people all over the world, answering a wide range of questions about MDS, including information on treatment options, clinical trials, fi nancial resources, and more.

PEER SUPPORT NETWORKLet this AA&MDSIF resource help you!

The Peer Support Network is a national network of trained volunteers, including patients, caregivers, and family members, who offer information, personal experience, coping strategies, problem solving skills and informational resources to people just like themselves. Speaking with a Peer Support Network volunteer is a great way to gather information and receive emotional support from someone whose life has also been affected by bone marrow failure disease.

To connect with a Peer Support Network volunteer, call (800) 747-2820, option 1, and speak with our patient educator, Leigh Clark, who will match you with one of our volunteers. You can also email her at info@aamds.org.

Help is Here for MDS Patients

answerssupport&h pe

NO INTERNET ACCESS AT HOME? Internet-connected computers are found in many locations, including:

• Retirement homes • Apartment community rooms • Public libraries • Senior centers

And it’s almost certain you know someone (relatives, neighbors, friends) who is connected!

Connect with us at www.AAMDS.org!

Contact Ushelp@aamds.org (301) 279-7202 or (800) 747-2820

ISSUE 8, MARCH 2012

• Educational materials Read Your Guide to Understanding MDS, a free patient guide that explains in plain language what you need to know to understand MDS—why it happens, what to do about it, how to receive your best care—and tips for living well with MDS. Also available is What to Expect from Treatment: A Guide to Understanding FDA-Approved Drug Therapies for Myelodysplastic Syndromes (MDS).

• Print and electronic news Stay current with the latest information on areas relevant to

MDS through our newsletters: Insider (print) and eInsider (electronic).

• Patient connectionsConnect in person at a regional Community of Hope event (for more information, please contact crews@aamds.org).

• AA&MDSIF events Registration for 2012 AA&MDSIF Patient and Family Conferences is now open! Learn all the details and register at www.AAMDS.org/Conferences

www.AAMDS.org – Your best resource for medical and personal perspectives on myelodysplastic syndromes (MDS)

Aplastic Anemia & MDS International Foundation

Find Us on Facebook at www.facebook.com/aamds

Connect online with other MDS patients

Here’s what attendees of the 2011 conferences had to say about their experience:

Thank you for all your hard work in putting these conferences together for us. It was an amazing and wonderful experience for me and I will make sure to attend next year.Leslie, MDS patient

I really enjoyed your conference. It answered a lot of questions for my daughter and me. We left your conference feeling great. WE LOVED IT! Keep up the good work because you do make a difference.Diana, mother of aplastic anemia patient

Great job! Very supportive and hopeful atmosphere. Wonderful presentations of practical and meaningful information. Please receive our sincere thanks for your vital and life-giving work.Lon, PNH patient

I have attended many, many conferences in the last 50 years on many subjects, and this was the most organized and informative of them all!Wayne, MDS patient

I was impressed with the conference, the staff, volunteers, the warmth and friendliness of everyone. It was a well-thought out conference.Gayle, aplastic anemia patient

Meet more patients and families, take home more expert information!

When you attend one of our conferences, here is what you can expect: Answers to your medical and treatment questions The morning sessions include concurrent tracks on advances in research and treatment methods for each disease—aplastic anemia, MDS, and PNH. During these sessions, participants will have unprecedented access to the nation’s leading bone marrow failure disease experts, with plenty of time for everyone’s questions to be answered.

Sessions on Living WellThe afternoon will include a series of Living Well sessions (this will vary by location) on topics including managing fatigue, complementary and alternative medicine, nutrition, and exercise, coping skills for maintaining emotional health, and survivorship. Three sessions will be offered at each location.

New for 2012!Support forums to help you connect, share, and learn from others These professionally-facilitated support sessions will bring together patients and family members with similar issues and concerns. They provide plenty of time for talking, sharing, comparing, and hearing how others are coping with the challenges of living with a bone marrow failure disease. Sessions are broken out by disease type. A special session for caregivers only will also be included. Sessions for parents of pediatric patients will be provided at our programs in New York, Atlanta, and Los Angeles.

Hope for the futureEach conference concludes with a powerful and uplifting Celebration of Hope. This joyous gathering celebrates life and friendship. The program includes a survivor spotlight story, highlighting the struggles and triumphs of one of the many long-term bone marrow failure disease survivors who is successfully living with and beyond their illness. We also include a special presentation, including pictures, quotes, and comments provided by conference participants in the weeks leading up to each conference. Be sure to come and celebrate your life and your courage with us.

Make plans to attend…register now!Our 2012 conferences will provide answers, support, and hope for every patient, family member, and caregiver whose lives have been touched by bone marrow failure disease. If you’ve never attended one of our conferences, you won’t want to miss this informative, inspirational, and uplifting event.

Insert

Important information for people living with myelodysplastic syndromes

Fighting Bone Marrow Failure Diseases Through Patient Support and Research Since 1983www.AAMDS.org

100 Park Ave., Suite 108 | Rockville, MD 20850

Insert

continued

Interviews with the Experts

the ConnectionMDS

Fighting Bone Marrow Failure Diseases Through Patient Support and Research Since 1983 www.AAMDS.org

Understanding Prognosis in MDS

Richard Stone, MDDana-Farber Cancer Institute Richard Stone, MD is Director of the Adult Acute Leukemia Program at the Dana-Farber Cancer Institute and professor of medicine at Harvard Medical School, both in Boston, Massachusetts. He also serves as Chairman of the AA&MDSIF Medical Advisory Board. In this May 2011

interview, Dr. Stone responds to questions about prognosis in MDS—what it is, what MDS prognosis means, what prognostic tools are used, and how prognosis is used to predict and plan for potential outcomes.

Can you explain what myelodysplastic syndromes (MDS) are?

Myelodysplastic syndromes are a group of diseases that affect bone marrow output. It’s a heterogeneous group of diseases, meaning that one patient’s MDS is often different from another patient’s MDS. Some types are relatively mild, where a patient can live for years, and others are quite severe and dangerous, such that prospects for long-term survival can be poor. What they have in common is ineffective bone marrow function, where the marrow is trying to make the correct amount of all blood cells as evidenced by the high degree of cellular activity—red blood cells, white blood cells, and platelets—but is unable to do so.

What does prognosis mean?

Prognosis means a guess as to how a patient is going to fare in the future based on present conditions. Predicting the future is always diffi cult, but it is important to try to do this for patients with potentially serious diseases like MDS. We want to be able to tell patients what they may be able to in terms of upcoming problems as well as estimating survival duration. So-called prognostic features or ‘prognostic index’ represents a tool that doctors use to relatively accurately inform patients about expectations. The prognosis is also used to develop a treatment plan. For example, a poor prognosis may suggest a more risky therapy, such as a stem cell transplant, be tried. A better prognosis that indicates a longer life expectancy may infl uence a decision to stay with a safer approach.

Important Information for People Living with Myelodysplastic Syndromes

Can you discuss the International Prognostic Scoring System (IPSS)? How do doctors use it?

The IPSS was devised in 1998—after review of a large panel of MDS patients—which determined which specifi c disease features were important for prognosis The developers of the IPSS determined there were three things needed to help determine prognosis—the percentage of bone marrow blasts (immature white blood cells), the number of cytopenias (low blood counts), and the bone marrow karyotype (the chromosomal analysis of bone marrow that is made at the time of diagnosis). From these, a score is formulated that estimates the likelihood of disease and survival.

What are bone marrow blasts?

These are young cells that are immature, but have the capacity to eventually give rise to the functional blood cells—red, white, and platelets. It’s diffi cult to tell a cancerous immature cell or blast from a healthy normal one. So if there are too many immature cells in the bone marrow, that indicates a problem. Ideally, blasts should comprise less than 5% of all marrow cells.

Are there any problems with the IPSS?

There are a few problems. One is that it is relatively old, given the development of new more effective therapies since 1998 when it was introduced. It was developed based on patients in the 1980s and 1990s. New drug treatments have been introduced since then, like lenalidomide (Revlimid®) and the hypomethylating agents—azacitidine (Vidaza®) and decitabine (Dacogen®)—and IPSS doesn’t take the effect of these into account. Another drawback is that it is not dynamic; that is, it is determined at diagnosis and doesn’t take into account events and treatments that subsequently occur. Also, it doesn’t apply to patients with prior hematologic abnormalities or prior treatment for other cancers. Finally, it doesn’t include an assessment of overall health, marrow functional status, or other non-MDS

the ConnectionMDS

FREE PHONE SUPPORT FOR MDS PATIENTS, FAMILIES, AND CAREGIVERSDo you need to speak with someone directly

about myelodysplastic syndromes (MDS)?

Please contact Leigh Clark, our Patient Educator, at (800)747-2820, option 1, or by email at info@aamds.org. Leigh communicates with people all over the world, answering a wide range of questions about MDS, including information on treatment options, clinical trials, fi nancial resources, and more.

PEER SUPPORT NETWORKLet this AA&MDSIF resource help you!

The Peer Support Network is a national network of trained volunteers, including patients, caregivers, and family members, who offer information, personal experience, coping strategies, problem solving skills and informational resources to people just like themselves. Speaking with a Peer Support Network volunteer is a great way to gather information and receive emotional support from someone whose life has also been affected by bone marrow failure disease.

To connect with a Peer Support Network volunteer, call (800) 747-2820, option 1, and speak with our patient educator, Leigh Clark, who will match you with one of our volunteers. You can also email her at info@aamds.org.

Help is Here for MDS Patients

answerssupport&h pe

NO INTERNET ACCESS AT HOME? Internet-connected computers are found in many locations, including:

• Retirement homes • Apartment community rooms • Public libraries • Senior centers

And it’s almost certain you know someone (relatives, neighbors, friends) who is connected!

Connect with us at www.AAMDS.org!

Contact Ushelp@aamds.org (301) 279-7202 or (800) 747-2820

ISSUE 8, MARCH 2012

• Educational materials Read Your Guide to Understanding MDS, a free patient guide that explains in plain language what you need to know to understand MDS—why it happens, what to do about it, how to receive your best care—and tips for living well with MDS. Also available is What to Expect from Treatment: A Guide to Understanding FDA-Approved Drug Therapies for Myelodysplastic Syndromes (MDS).

• Print and electronic news Stay current with the latest information on areas relevant to

MDS through our newsletters: Insider (print) and eInsider (electronic).

• Patient connectionsConnect in person at a regional Community of Hope event (for more information, please contact crews@aamds.org).

• AA&MDSIF events Registration for 2012 AA&MDSIF Patient and Family Conferences is now open! Learn all the details and register at www.AAMDS.org/Conferences

www.AAMDS.org – Your best resource for medical and personal perspectives on myelodysplastic syndromes (MDS)

Aplastic Anemia & MDS International Foundation

Find Us on Facebook at www.facebook.com/aamds

Connect online with other MDS patients

Here’s what attendees of the 2011 conferences had to say about their experience:

Thank you for all your hard work in putting these conferences together for us. It was an amazing and wonderful experience for me and I will make sure to attend next year.Leslie, MDS patient

I really enjoyed your conference. It answered a lot of questions for my daughter and me. We left your conference feeling great. WE LOVED IT! Keep up the good work because you do make a difference.Diana, mother of aplastic anemia patient

Great job! Very supportive and hopeful atmosphere. Wonderful presentations of practical and meaningful information. Please receive our sincere thanks for your vital and life-giving work.Lon, PNH patient

I have attended many, many conferences in the last 50 years on many subjects, and this was the most organized and informative of them all!Wayne, MDS patient

I was impressed with the conference, the staff, volunteers, the warmth and friendliness of everyone. It was a well-thought out conference.Gayle, aplastic anemia patient

Meet more patients and families, take home more expert information!

When you attend one of our conferences, here is what you can expect: Answers to your medical and treatment questions The morning sessions include concurrent tracks on advances in research and treatment methods for each disease—aplastic anemia, MDS, and PNH. During these sessions, participants will have unprecedented access to the nation’s leading bone marrow failure disease experts, with plenty of time for everyone’s questions to be answered.

Sessions on Living WellThe afternoon will include a series of Living Well sessions (this will vary by location) on topics including managing fatigue, complementary and alternative medicine, nutrition, and exercise, coping skills for maintaining emotional health, and survivorship. Three sessions will be offered at each location.

New for 2012!Support forums to help you connect, share, and learn from others These professionally-facilitated support sessions will bring together patients and family members with similar issues and concerns. They provide plenty of time for talking, sharing, comparing, and hearing how others are coping with the challenges of living with a bone marrow failure disease. Sessions are broken out by disease type. A special session for caregivers only will also be included. Sessions for parents of pediatric patients will be provided at our programs in New York, Atlanta, and Los Angeles.

Hope for the futureEach conference concludes with a powerful and uplifting Celebration of Hope. This joyous gathering celebrates life and friendship. The program includes a survivor spotlight story, highlighting the struggles and triumphs of one of the many long-term bone marrow failure disease survivors who is successfully living with and beyond their illness. We also include a special presentation, including pictures, quotes, and comments provided by conference participants in the weeks leading up to each conference. Be sure to come and celebrate your life and your courage with us.

Make plans to attend…register now!Our 2012 conferences will provide answers, support, and hope for every patient, family member, and caregiver whose lives have been touched by bone marrow failure disease. If you’ve never attended one of our conferences, you won’t want to miss this informative, inspirational, and uplifting event.