Family Spotlight

A New Heart … A New Start … A Miracle
Preparing for High School Graduation and Planning for College

Chandler West expected his junior year of high school to be tough.  He planned a year filled with challenging academics and a myriad of school activities.  But as he entered the school year, he suddenly began to tire easily and did not have the energy to participate in the activities he loved.  Many times he watched his friends from the sidelines, wondering why he was unable to be involved.  It was during his junior year that Chandler and his family learned the reason: the deadly diagnosis of Dilated Cardiomyopathy.  Chandler’s only hope was a heart transplant.

As the disease progressed, Chandler was hospitalized several times, including a lengthy stay in the intensive care unit of St. Louis Children’s Hospital in St. Louis, Missouri, to receive a pacemaker.  His energy level decreased tremendously, and his ability to live a normal high school teenager life became impossible.  As his condition worsened, the transplant team at St. Louis Children’s told him and his family the time had come:  They placed Chandler on the transplant waiting list. 

That’s when Chandler’s family called the Children’s Organ Transplant Association (COTA) for help with their mounting transplant-related expenses.  When asked about the impact COTA has had on her family, Maureen West, Chandler’s Aunt said, “Let’s face it, it’s painful asking for money but we now know the rewards far out weigh any discomfort.  My nephew went from being a 16-year-old boy waiting all alone for a heart transplant, to knowing that there was an army of family, friends and total strangers standing behind him.  Thank you COTA, for helping us put together our army.”

Chandler received his new heart, and his second chance at life, on December 4, 2007, at St. Louis Children’s Hospital.  Two weeks later, he was back home in Oklahoma City, and he returned to his high school to resume his classroom studies in January. 

Chandler admits he struggled to keep up with the rigors of his senior year studies, but his determination and drive kept him on top of his coursework and he was able to fulfill all graduation requirements.  In addition to his homework, Chandler’s pre- and post-transplant days were also filled with college applications, scholarship forms, interviews, and lots of paperwork.

His hard work and personal motivation paid off.

Chandler will graduate on time with his senior class later this month.  He has been accepted at Loyola University in Chicago where he will study science in hopes of one day becoming a doctor himself.

According to Chandler, “Each day I regain more and more strength, and I get closer to living a normal life.  Of course, none of this would have been possible without gift from a person whom I will never know – someone who gave his or her life so I could keep living mine.  Someone who gave me a new heart … and a new start.”

Chandler West … In His Own Words:
It was the last thing I remember … being wheeled into a bright, white room.  It was the first time I was truly scared.  My mind was racing, but there was nothing I could do. 

It all happened six months ago, on December 4, 2007, at 2:45 a.m., the exact time of my heart transplant surgery.  The team of surgeons and nurses all worked together and removed my diseased heart and gave me a new one – a heart from my unknown donor.  I am overwhelmed that a miracle has happened to me and I am struggling to figure out how to convey my feelings on paper.  However it was, and will likely always be, the most important thing that will ever happen to me. It’s when I got a new start … a new start that I want to tell the world about.

Over a year ago I was diagnosed with a form of congestive heart failure.  It all came on very suddenly with symptoms that I was ‘controlling’ with Tylenol and a day off school here and there.  But before I knew it, I was being rushed to the Intensive Care Unit at the University of Oklahoma Medical Center and then life-flighted to St. Louis Children’s Hospital.  That is when I first heard I would have to have a heart transplant.  I spent the next three months in St. Louis - virtually the entire time in intensive care.  Finally, I was put on the transplant list and sent back to Oklahoma with lots of medication and a new pacemaker.

Eventually, I got sick again.  I was told the time had come and I should start to prepare.  I kept the news to myself because my friends were really busy preparing for and taking college admission tests.  Goodbyes happened quickly, and we began our eight-hour drive to St. Louis.  I sat quietly for much of the car ride thinking that those moments could be the last time I would see my friends.  I also wondered if I would get the chance to apply to, and attend, college.

On December 4th I received my second chance at life. 

Now, each day I regain more and more strength, getting closer to leading a normal life.  None of this would have been possible without a person whom I will never know … someone whose decision to be an organ donor allows me to keep living.  Someone who gave me a new start and the chance to finish high school and start planning for college – just like my friends.

In retrospect, my high school experience has been anything but normal.  I started getting sick during my junior year and from that point on, many of my high school days were spent watching from the sidelines because I was always tired and had very little energy.  By that point my ability to participate in many normal high school activities was very limited. 

Within six weeks of my heart transplant, I returned to high school.  My energy level was much better.  I began gaining weight, and people constantly told me how much healthier and better I look.  But most importantly I was able to start doing the things I enjoy.  This winter I actually got to go snowboarding (one of my favorite things ever), and I did not have to sit and watch from the sidelines.  Each day I get closer to living a normal life.

Since getting sick, the hardest part of high school has been trying to keep up with my school work. It is amazing how quickly you get behind when you are repeatedly hospitalized, and how long it takes to catch up.  My teachers were very helpful.  With their guidance and my drive to succeed, I am scheduled to graduate with my friends later this spring.

College has always been my goal; specifically attending Loyola University in Chicago because of its outstanding reputation for its science curriculum.  After much help and clarification of my transcripts (that clearly show a student who was absent for more than half of his senior year), I received a call on my cell phone from Loyola’s Dean of Admissions who told me I had been accepted.  I was shocked.  I always had an interest in medical science, but this whole transplant experience has given me a new appreciation for it.  I owe everything to my team of transplant surgeons, and I want to become a doctor.  I want to be able to help children in the way my doctors helped me.  My ultimate goal is going to medical school to become a pediatrician so I can help children get the same miracle I got – a second chance at life.

My current challenge is figuring out how to pay for college and to that end, I have a commercial photography website (www.ChandlerWest.com) with profits helping to offset my tuition bills.  I will do anything necessary to make sure my new start … my miracle … will help another child also get a second chance.

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Transplants Today

Refinements in the Risk Classification for Acute Myeloid Leukemia

Both studies, "add clinically interesting chapters to the unfolding story of the dazzling molecular heterogeneity of AML," wrote Bob Löwenberg, MD, PhD, from Erasmus University, in Rotterdam, the Netherlands, in an accompanying editorial.  “They add to the accumulating knowledge about the way genetic disruptions in AML can affect prognosis.”

Cytogenetic abnormalities are already useful in guiding treatment for AML.  One of the crucial questions in the treatment of AML is whether or not to go for an allogeneic stem cell transplant, Dr. Löwenberg said.  Transplantation can be a successful treatment for AML, but it does carry risks.  Currently, it is usually avoided when the AML has a cytogenetic pattern that is associated with a relatively favorable prognosis, and considered when the cytogenetic abnormalities foretell a high risk for relapse after chemotherapy, according to Dr. Löwenberg.

However, about half the patients with AML have no detectable cytogenetic abnormality in the leukemic cell.  At present, all of these patients are categorized as being at intermediate risk, but there is a lot of variation in this group.  This is where the latest studies show promise; they describe new ways of differentiating these patients.

The study of gene mutations, reported by Richard Schlenk, MD, from the University of Ulm, in Germany, analyzed data on 872 patients who had taken part in four treatment trials conducted by the German-Austrian Acute Myeloid Leukemia Study Group.  The researchers found that certain genotypes are associated with clinically significant differences in response to therapy and in survival.

The Effect of Genotype on Survival in Cytogenetically Normal AML

For example, the genotype of mutant NPMI without FLT3-ITD was associated with a complete remission after conventional anthracycline and cytarabine-based induction therapy.  These patients might not benefit from related-donor transplantation as a first-line treatment, the researchers said.  The mutant CEBPA genotype was also associated with a complete remission, a correlation that has not been reported previously, they added.

In contrast, the genotype of FLT3-ITD and the triple-negative genotype of wild-type NPMI and CEBPA without FLT3-ITD had poor outcomes.  Notably, only these two groups benefited from an allogeneic transplant performed during the first complete remission.  In both of these groups of patients, transplantation involving a related donor — and possibly an unrelated donor — should be explored, at least while there are no successful targeted therapies available, according to the findings.

However, in his editorial, Dr. Löwenberg states that the study does not provide direct evidence to support such a treatment recommendation.  He also suggests that decision making about transplantation should take into account not only leukemia-specific risk factors, but also the risk for death from transplantation itself, which depends on factors such as age and coexisting medical conditions.

"Our data provide a basis for refining the risk classification of AML," Dr. Schlenk and colleagues said.  They propose that patients with cytogenetically normal AML and the favorable genotypes of either mutant NPM1 without FLT3-ITD or mutant CEBPA should no longer be classified as having intermediate-risk leukemia, but should rather be classified as having favorable-risk leukemia, together with the core-binding AMLs.

"We recommend that screening for NPM1, FLT3, and CEBPA mutations be part of the initial workup for newly diagnosed AML.”

The other study examined microRNA signatures but was smaller in size.  The team, headed by Guido Marcucci, MD, from Ohio State University, in Columbus, Ohio, analyzed data collected during the Cancer and Leukemia Group B (CALGB) 19808 study from 64 patients in the training group and 55 patients in the validation group.

This team derived a set of 12 microRNA sequences that could distinguish two subgroups of cytogenetically normal AML patients: one with an 11% probability of event-free survival and the other with a 36% probability of event-free survival.  The researchers acknowledge the small number of patients on which their survival estimates are based (i.e. 28 and 27 patients), and that their results require confirmation, the editorial noted.

All of this emerging new information needs to be integrated into a comprehensive and clinically useful algorithm, Dr. Löwenberg concluded.  However, there are still many unresolved issues.  He predicts that, "we will probably not have a single, universally accepted system for stratification of AML prognosis for some time."

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Low Socioeconomic Status a Risk Factor for Heart Graft Failure

Low socioeconomic position remained an independent risk factor for graft failure in children even after controlling for multiple clinical and demographic factors, said Tajinder P. Singh, MD, MBBS, from the Department of Pediatric Cardiology at Children's Hospital Boston, and an assistant professor of pediatrics at Harvard Medical School.

"This is really the first time, in children, that this has been looked at from a socioeconomic as opposed to a racial standpoint," said Robert J. Boucek, MD, Chief of the Division of Cardiology at Seattle Children's Hospital and Regional Medical Center, during an interview with Medscape Transplantation.

"Several studies previously demonstrated there is an association between race, so that nonwhites — specifically, black patients — have worse long-term survival," Dr. Singh said. "They seem to have more rejections of the heart transplant, and there is a lot of emphasis on genetic and immunologic studies to differentiate different races and how their biology is different."

Dr. Singh continued, "Based on some clinical observations, I wondered whether somebody had systematically looked at the socioeconomic data in these people.  The problem is we in medical records do not record what the patient or family income is, what the parents education is, what occupation they are in — maybe occupation, but it is not systematically and consistently written down."

Zip codes, which some investigators use to identify patient demographic, social, and financial characteristics, cast too wide a net and may skew averages too heavily in one direction or another, Dr. Singh said.  To get around this problem, he and his colleagues used block groups — the smallest units available in U.S. census databases that also contain socioeconomic data.

"All data on block groups are reported every 10 years by the U.S. census bureau; it's meant to be permanent, and it's meant to be relatively homogeneous with regards to people's socioeconomic status — it's literally your neighborhood," he said.

The investigators looked at U.S. census data from 2000, extracting data on six variables of wealth, income, education, and occupation for each transplant recipient.  They then calculated a summed socioeconomic z-score for 126 heart transplant recipients who survived to discharge at Children's Hospital Boston from 1990 to 2005.

They also created multivariate Cox proportional hazards models to determine the risk of graft failure, defined as time to death or to retransplantation, for the children in the lowest tertile of socioeconomic status compared with those in the middle and upper tertiles, who served as control patients.

The patients ranged in age from six days to 23 years (median, 8.9 years) at the time of transplant.  The distribution of age, sex, diagnosis, and treatment year was similar among the groups.  Whites accounted for significantly fewer children on the lowest rung of the socioeconomic ladder, at 67% vs. 90% of control patients (P < .01).

In all, there were 37 graft failures.  The authors found in a univariate analysis that being in the low-socioeconomic group was associated with a more than twp-fold risk for failure (hazard ratio [HR], 2.3; 95% confidence interval [CI],1.2 – 4.4), and being a member of a nonwhite race was associated with a nearly three-fold increase in risk (HR, 2.9; 95% CI,1.4 – 5.9).

In multivariate models controlling for patient age, diagnosis (i.e. congenital heart disease vs. dilated cardiomyopathy), transplantation era, and race, the effect of race on graft failure was slightly attenuated but still significant (HR, 2.5; 95% CI,1.2 – 5.4), and the effect of low socioeconomic position remained relatively unchanged (HR, 2.1; 95% CI, 1.0 – 4.3; P = .04).

All of the children in the study, both rich and poor, were insured, Dr. Boucek said, suggesting that, "it's not an issue of access, but it may be care before leading to transplant that accounts for the differences."

The Children's Hospital Boston researchers will be expanding this study to look at a larger population, with centers in New York City, Atlanta, and Loma Linda, California, and to look at data on both children and adults transplanted in Boston hospitals over the last decade.

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COTA News

www.cota.org – An Online Information Source for Transplant Professionals

The Children’s Organ Transplant Association is striving to make learning about COTA and obtaining COTA materials easier than ever before. Our website, www.cota.org, has been designed with an entire section devoted solely to Transplant Professionals. There, you can:

• Learn more about COTA … Catch up on previous editions of our monthly enewsletters, read patient stories and read the latest news about COTA.
• Visit our Online Forum … Communicate with others about transplantation.
• Request materials … Materials are available both for your use and your patients’ and families’ use.

Join COTA’s mailing list … Throughout each year we send numerous mailings to help keep you informed of our service to your patients.

Please visit, and bookmark, www.cota.org today.

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COTA Fast Fact

The Children’s Organ Transplant Association (COTA) provides Management of the Funds Raised. Funds may be used for transplant-related expenses. The Children’s Organ Transplant Association does not charge for its services and provides families and donors the assurance that funds will be used appropriately. COTA’s program is flexible -- COTA assists families regardless of their need, or the type of support they require.

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