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A Report on Your Investment in Hope
Hope Cause for
Olivia, 2, treated for a single ventricle heart defect
A message from
Cardiac Center Leadership Dear Friends, The Cardiac Center at The Children’s Hospital of Philadelphia treats thousands of children every year and provides services for patients of all ages, from treating heart defects before birth to helping transition patients to adult care. Taking care of children with heart problems and achieving good outcomes for them is the ultimate team sport. The Cardiac Center’s team of more than 350 caregivers provides the entire spectrum of clinical services necessary for the care of congenital heart disease, including innovative therapies not found in most hospitals. The Cardiac Center’s Division of Cardiothoracic Surgery is a leader nationwide. More than 850 cardiothoracic surgeries are performed at CHOP each year, including 500 open heart procedures. Our surgical team members are experts in performing lung transplants and heart transplants, saving the lives of children who have no other option. They even handle simultaneous heartlung transplants, of which there are only three to five done each year in the United States. CHOP surgeons are among a small group of elite clinicians experienced in this complex procedure. The wait for a donor organ can be a year or more, and CHOP is one of the few children’s hospitals that has every tool available to help patients through this difficult period. The Cardiac Center
Thomas L. Spray, M.D. Chief, Division of Cardiothoracic Surgery Alice Langdon Warner Endowed Chair in Pediatric Cardiothoracic Surgery James M. Steven, M.D., S.M. Chief, Division of Cardiac Anesthesia
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has implanted complete artificial hearts as a bridge to transplant in two pediatric patients in the last year — an incredible treatment that is as rare as it sounds. Thanks to years of research on congenital heart disease, much of it done at CHOP, we are no longer asking “Will this child live?” but “How well will he live?” To answer that, we are looking at patients’ quality of life and their neurodevelopmental outcomes, as well as how they are doing long-term. We know children respond differently to the stress of heart disease and the stress of surgery, and we’re beginning to look at genetic factors with the goal of developing personalized medicine and individually customized treatment. This report will give you an idea of the breadth of the expertise at the Cardiac Center, both in our ability to provide cutting-edge clinical services around the clock and in our cutting-edge research that has the potential to transform lives. Thank you for joining with us to help make all of these things possible. Philanthropic support from you and the hundreds of other generous people who give to the Cardiac Center provides our surgeons, cardiologists, anesthesiologists, nurses, social workers and other caregivers the resources they need to give children facing serious heart problems the very best care.
Robert E. Shaddy, M.D. Chief, Division of Cardiology Jennifer Terker Endowed Chair in Pediatric Cardiology Alexis Perri, M.S.N., R.N., N.E.-B.C. Director, Cardiac Nursing
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Quinn, 3 months, treated for anomalous coronary artery
Tackling these problems involves researchers in cardiology, cardiothoracic surgery, cardiac anesthesia, intensive care, cardiac nursing, neurology, genetics, biostatistics and developmental pediatrics. The researchers are building a database of details about how heart disease and cardiac surgery cause these problems and why they occur in some children, but not in others. The research group asks children who meet certain criteria, such as having had particular procedures or being certain ages at the time of their surgeries, to come back to CHOP for annual or biannual tests to assess developmental benchmarks like speech, motor skills and executive function as they grow to adulthood. Some families also fill out regular questionnaires about the patient’s school performance, social life and other behaviors.
A Brighter Future Helping kids with congenital heart disease thrive
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s recently as 25 years ago, congenital heart defects (CHDs) were a death sentence for most newborns. Today, medical advances have upended those odds; most children treated for CHD survive. This survival comes at a cost, though: Evidence shows that CHD and heart surgery often affect children’s long-term development. CHOP’s internationally renowned Neurocardiac Research Group is studying how, why and what to do about it. Nearly 50 percent of children who have heart surgery at an early age have developmental issues such as delayed fine motor skills, learning
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disabilities or problems with the “executive functions” of planning and organizing, remembering details, paying attention, setting goals, learning from experience, and behaving appropriately. As many as one in three have attention deficit hyperactivity disorder (ADHD). “Brain development is not normal with these kids,” says J. William Gaynor, M.D., Daniel M. Tabas Endowed Chair in Pediatric Cardiothoracic Surgery and director of the Neurocardiac Research Group. “At birth, they’re more like premature babies. Their brains are more fragile and more prone to injury.”
At the same time, the research group is searching DNA samples for an answer to the mystery of why cardiac treatments that work well for some children lead to neurological challenges for others. “This is not about the genetic roots of heart disease itself,” Gaynor explains. “It’s about finding the genetic factors to explain why two kids with the same heart problem and the same treatment respond differently to the stresses of the illness and the surgery.” The goal is to develop truly personalized medicine that tailors treatment to what’s best for each patient. Private donors have been the Neurocardiac Research Group’s primary source of support since its founding in 1998, with generous contributions from the Fannie E. Rippel Foundation, The Women’s Committee of The Children’s Hospital of Philadelphia and others.
Abby, 1 1/2, has had three open heart surgeries at CHOP.
Future philanthropy will help the group enroll more children in long-term outcome studies and support more detailed testing, including neuroimaging that actually lets researchers see brain activity. The group also wants to take a closer look at the ways heart disease damages the brain while it’s developing, with an eye toward monitoring and protecting the brains of children with CHD before they’re born as well as during and after surgery. “We don’t want kids just to survive, we want them to have a good life,” Gaynor says. “Ideally, we want them to grow up and for no one to be able to tell they ever had heart surgery apart from the scar on their chest.”
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Heart to Heart Special fund helps Cardiac Center families with travel, living expenses
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hen a child is diagnosed with heart disease, it is an extreme burden for young families — physically, emotionally and financially. Many come to CHOP from across the country and must deal with travel costs and hotel bills in addition to an uncertain future. The Penney Girls Foundation made the very first gift to establish and launch the Cardiac Center Families Fund, to provide families that have limited financial means with a stipend to cover travel and living expenses during the course of their children’s diagnosis and treatment. They have continued their support and have been joined by other generous donors, including Carroll and Ned Lewis, who have supported the development of this fund. Without this support, many families could not travel to CHOP for its excellent, specialized care. Since its launch, the fund has made over 90 distributions to families in need for emergency travel, hotel and gas expenses related to their children’s care at CHOP. Below are several examples of families that have been helped through the Cardiac Center Families Fund this past year. (To protect their privacy, all names have been changed.) The fund allows CHOP caregivers to provide lifesaving care and emotional support for families going through tremendous stress.
Ethan Lauren, 3, was cared for in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit while she waited for a heart transplant.
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Ethan was diagnosed with life-threatening heart failure at a hospital near his home when he was 14. He was rushed to CHOP, where he immediately received an artificial heart and later a heart transplant. He currently receives intensive rehabilitation. Ethan’s parents have been deeply involved in his care but have a very limited support system. To support their son, both
parents took unpaid leave from work, and Ethan’s mother ultimately had to resign. She has been traveling back and forth to their home every other weekend since her son was admitted to the Hospital.
Joshua After surviving premature birth, Joshua suffered an array of medical problems and underwent multiple open heart surgeries during the first five years of his life. When he was 11, Joshua traveled from his home three hours away to be evaluated and treated at CHOP’s Cardiac Center, where he is now awaiting a heart transplant. Joshua’s mom, a single parent, has been by his bedside since his admission to the Hospital and has been very involved in his care. But their family is under enormous financial strain, as Joshua’s mother is unemployed and is also caring for his siblings.
Tyler Six-year-old Tyler has been living at CHOP as he waits for a heart and kidney transplant. This will be his second heart transplant, the first having taken place when he was 3 months old. He has been in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit since he began to show rejection of his transplanted heart. Tyler’s family lives two hours from CHOP, but his parents commute to and from the Hospital daily, caring for other small children at home. By assisting with travel and other costs, CHOP has been able to ease these families’ stress and help them focus on their children. To contribute to the Cardiac Center Families Fund and help families in need get their children the care they deserve, mark the name of the fund on your check and return it in the envelope provided in this publication.
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Keeping Watch Over Children’s Hearts Renowned CHOP cardiologist leads effort to prevent sudden cardiac death in the young
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t was another significant milestone in a career filled with them: On Feb. 9, CHOP cardiologist Victoria L. Vetter, M.D., M.P.H., became the first woman — and the first pediatric cardiologist — to receive the American Heart Association of Southeastern Pennsylvania’s Edward S. Cooper M.D. Award, which recognizes medical professionals and researchers whose work has made a significant impact in the Philadelphia community. Vetter, an internationally recognized expert in pediatric electrophysiology, came to CHOP as a fellow in 1975. As chief of Cardiology from 1993 to 2007, she oversaw a period of tremendous growth for the division, during which the number
of faculty members increased from nine to 63 and the cardiac unit — once a small, 20-bed facility — was expanded to encompass the entire sixth floor of the Hospital. In 2005, she was awarded the Evelyn Rome Tabas Endowed Chair in Pediatric Cardiology. Today, Vetter devotes much of her time to Youth Heart Watch, a program she founded in 2006 with the goal of finding ways to prevent sudden cardiac death in the young. Her interest in the issue began very early in her career, when she cared for a young girl who suffered severe neurological damage from a sudden cardiac arrest (SCA) and she became convinced of a need to act as she met more and more families whose
Hundreds of students from 13 Philadelphia high schools showed off their lifesaving skills during the CPR/ AED Olympics, organized by Youth Heart Watch.
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children had died of SCA. Hearing their stories had a profound impact on Vetter: “I realized we really needed to do something to prevent these deaths.”
A Pioneering Program Youth Heart Watch works to prevent sudden cardiac death through two community-based initiatives: a screening program to identify children with serious heart conditions and a program that places automated external defibrillators (AEDs) in public schools and helps schools implement CPR/AED programs. The program has received support from the American Heart Association, Pfizer, the Laerdal Foundation for Acute Medicine and Simon’s Fund, created by Darren and Phyllis Sudman after they lost their son to sudden cardiac death. Individual donors are crucial to the program, including grateful parents Max and Elyse Berger, who are strong advocates for Youth Heart Watch and its mission of saving children’s lives. Youth Heart Watch initiatives have made a tremendous difference for children in the tristate area. The program has screened about 3,500 children and found that nearly 1 percent of them had a significant cardiac abnormality — which has, in turn, helped to identify many others at risk for SCA. “These are inherited diseases,” says Vetter. “If we identify one child with a condition, there are studies that show there are eight other family members who would also be identified.” In addition, the program has provided AED consultations to 250 schools, helped directly train nearly 100 school staff in CPR and AED use, and provided more than 100 training grants to schools implementing AED programs, resulting in the training of 894 staff and students with an additional 44 adults trained as CPR/AED instructors. Vetter and her team, which includes research nurse coordinator Noreen Dugan, R.N., and program coordinator Danielle Main Haley, M.P.H., also conduct extensive research related to AED use and SCA. One study involved students from Philadelphia high schools who were asked to
“Youth Heart Watch saves lives and spares families the kind of tragedies we have seen too often.” — Victoria L. Vetter, M.D., M.P.H. Medical Director, Youth Heart Watch
develop creative ways to teach CPR and AED skills to their peers. The students showcased their teaching curricula — which included plays, cheers, websites and board games — during an outdoor event called the “CPR/AED Olympics,” where they also had the opportunity to test their skills in AED relays, mock codes, “chest compression challenges” and other activities. In a follow-up study, the research team found that these teens had an 89 percent retention rate for their CPR/AED skills — a truly phenomenal result, unprecedented in earlier studies. “Our hope is to obtain additional funding, so we can take this program into more schools and develop a website where other people can access these activities,” says Vetter. “Our goal is to have every kid who graduates from high school be trained in CPR and AED use. There’s no reason Philadelphia can’t be the leader in the nation for this — and we have the model to support it.”
If you would like to support Youth Heart Watch’s lifesaving work, please mark your donation for Youth Heart Watch and return it in the envelope provided in this publication.
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With All Our Hearts Families learn about congenital heart disease — and connect with each other — at annual Cardiac Center education day
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owhere is the Cardiac Center’s commitment to caring for patients from before birth through adulthood more clear than at Heart and Mind Education Day, held each summer on CHOP’s Main Campus. Throughout the event, which has received philanthropic support from the Edna G. Kynett Memorial Foundation and Mr. and Mrs. Robert N. Spahr, parents of the center’s tiniest patients chat with older children who have been cared for at the Cardiac Center since infancy. Surgeons, child life specialists, physicians, nurses and other staff talk about every aspect of the Cardiac Center
experience, from the Fetal Heart Program to transitioning to adult care. And adult patients share what it’s like to live, and thrive, with congenital heart disease (CHD). For David Drajpuch, M.S.N., C.R.N.P., a nurse practitioner with the Philadelphia Adult Congenital Heart Center, a joint program of the Cardiac Center and Penn Medicine, one of the most inspiring things about the day is seeing so many parents of infants in sessions intended for adult patients. “They’re so focused on getting through the surgeries and procedures their child needs as a
Good friends Stacie Jackley (left) and Stacey Lihn are among the many parents who attended Heart and Mind Education Day in 2012.
baby,” he says, “that to have this group come in and say, ‘We take care of them once they’re adults’ — it completely blows their minds.” Stacey Lihn, of Phoenix, Ariz., is one of those parents. Lihn’s daughter Zoe, now 3, was born in the Garbose Family Special Delivery Unit and has had two open heart surgeries at CHOP. At Heart and Mind Education Day in 2012, Stacey and her husband, Caleb, enjoyed learning about everything the Cardiac Center has to offer their daughter as she grows up. “CHOP is so willing to educate parents,” she says. “We have all this weight on our backs, and it’s such a relief to know that CHOP is with us, carrying it.” Throughout the day, the Lihns also connected with other parents of kids with CHD. “It’s a relaxing feeling to be around like minds,” says Lihn. “It’s amazing to have such a strong connection to other families.” Clinicians, too, appreciate the opportunity to form stronger connections with patients and
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Friends Zoe and Kellen are treated for hypoplastic left heart syndrome at CHOP. Their moms (see opposite page) are also close friends. their families — to answer any questions they have about the Cardiac Center, to hear their concerns, and to learn more about their lives and the challenges they face. “The best part of the day is having time to sit and talk with patients and their families,” says Drajpuch. “It’s nice to get to know them on a more personal level and to spend time with them. It’s a wonderful experience.”
Save the Date The next Heart and Mind Education Day will be held on Saturday, July 27, 2013. For more information, visit heart.chop.edu/heartandmind.
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A Helping Hand for Failing Hearts Advances in mechanical support make life better for patients
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ne size fits all” isn’t true in the dressing room, so why should it be true in the operating room? The Children’s Hospital of Philadelphia boasts multiple mechanical devices to replace or support hearts with many different problems. These devices can keep children healthier and stronger while they await a heart transplant or recover from illness or surgery — and even let them wait or recuperate at home instead of in the Hospital. “We’re one of just a handful of hospitals in the country to have so many devices to help patients with so many different diseases for so many different lengths of time,” says Joseph Rossano, M.D., CHOP’s medical director of heart transplantation. “Patients come from around the world for these therapies. We can help them in ways that weren’t possible as recently as five years ago.”
Zion, 17 months, relies on a mechanical support device called the Berlin heart.
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The mechanical circulatory support program recently acquired the SynCardia, a true artificial heart that replaces a failing heart muscle entirely. The first patient to receive it at CHOP was a boy in his mid-teens who had already had multiple surgeries for a hard-to-treat cardiac defect. When the boy arrived at CHOP, Rossano says, he probably had only hours to live. After surgery, though, he was able to eat and even walk a bit — and he has now had a transplant and returned home.
surgery in the catheterization lab to support hearts recovering from short-term illnesses like a post-transplant rejection episode or acute myocarditis, which is inflammation of the heart muscle. The Cardiac Center began using the Tandem heart in mid-February. Another acquisition for 2013 is the HeartMate II, a small, durable, battery-powered pump worn in a belt, bag or vest. Because this device can provide a failing heart with months or even years of support, Rossano predicts some children who would otherwise be confined to a hospital until a transplant is available will be able to go home instead and live more active, healthy lives while they wait. These devices are all designed and approved for use in adult-sized bodies, which makes using them with smaller patients a challenge, Rossano points out. Some can be adapted, but others can’t. To help address this problem, CHOP hopes to participate in an upcoming National Institutes of Health study that will study heart pumps specifically designed for children and infants. “The mechanical circulatory support program is a joint effort by a lot of different skilled experts,” Rossano says. “It takes multiple departments to make these devices work, from surgeons and transplant specialists to nursing and rehab, but it’s a major advance in our field. We’re optimistic about our goal of supporting every type of failing circulation in patients of every size.”
The Hospital has also acquired a Tandem heart, which can be implanted and removed without
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Committed to Excellence Nurse practitioners bring years of specialized training to the front lines of patient care
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aced with work-hour restrictions and other new policies over the last two years that have meant fewer residents were available to care for patients, the Cardiac Center helped maintain a low patientprovider ratio by adding five nurse practitioners
Nurse practitioner Katie Dodds, R.N., M.S.N., C.R.N.P., with Tassos, 10, treated for hypoplastic left heart syndrome
(NPs) to its staff. They have advanced degrees and years of specialized training. They have the knowledge and skills necessary to care for critically ill patients. And there’s another benefit: Unlike residents, who complete several rotations in different areas and may not have
a specific interest in cardiology, nurse practitioners are with the Cardiac Center for the long term. “This is what we love,” says Sara Sheils, M.S.N., C.P.N.P.-A.C., a nurse practitioner who has worked in the Cardiac Care Unit for five years and is the team lead for the center’s inpatient nurse practitioners. “We’re all very interested in cardiology.” The NPs’ passion for pediatric cardiology comes through every day, as they monitor and assess patients, conduct rounds with physicians, work on quality improvement projects on the unit, develop care plans, and interact with families, who appreciate seeing familiar faces who know their children’s history. That’s especially important in a place as large as CHOP, where patients see so many providers. “The NPs are here every week,” says Sheils. “We’re continuity.” The Philadelphia Adult Congenital Heart Center, a joint program of the Cardiac Center and Penn Medicine, has also put NPs front and center. In January, the center opened a clinic staffed by NPs as part of an effort to expand access to the program. The NPs get to know each patient well and are typically the first point of contact when patients call with questions. “They’re always talking to someone who’s familiar with their case,” says David Drajpuch, M.S.N., C.R.N.P., one of the Philadelphia Adult Congenital Heart Center’s two NPs. “And well over 80 or 90 percent of the time we’re able to take care of whatever it is they need.”
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Cardiac Nursing Education Fund Nurses play a very important role in caring for children with cardiac problems — and philanthropic support is needed to ensure these important caregivers have all of the knowledge, skills and resources they need to continue to provide excellent care. The Cardiac Nursing Education Fund provides support for nursing education, such as attending conferences and trainings on best practices in pediatric cardiac care. If you would like to support the continuing education of the Cardiac Center’s nursing staff, including nurse practitioners, please mark your donation for the Cardiac Nursing Education Fund and return it in the envelope provided in this publication.
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Creating New Standards of Care CHOP cardiologist spearheads effort to improve care for kids at high risk for sudden cardiac death
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t’s the second most common cause of sudden cardiac death from structural heart disease among children and young adults — but despite its prevalence, most people have never heard of it. And there’s no consensus among cardiologists on the best way to care for children who have it. CHOP cardiologist Julie Brothers, M.D., has spent years studying the congenital heart defect anomalous aortic origin of a coronary artery (AAOCA), which occurs when one or both coronary arteries arise from the wrong location on the aorta. Brothers and her colleagues at the Cardiac Center hope they will be able to establish standards of care for AAOCA and develop the ability to give affected children the information they need to not only avoid sudden cardiac death, but to live full lives despite the condition.
Srishti, 4, treated for tricuspid atresia
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In 2009, Brothers brought together a group of cardiologists and surgeons to establish the Registry of Anomalous Aortic Origin of the Coronary Artery. Through the registry, the team has collected data on more than 200 patients from the United States and Canada to date — the largest cohort of patients with AAOCA being studied anywhere in the world. With additional donor support, they hope to enroll even more patients in the future. The team’s goals are to establish evidence-based guidelines for managing this condition and to
develop models that will help clinicians stratify kids with AAOCA according to risk. Some kids with AAOCA have no symptoms and are only diagnosed because they have an echocardiogram done for another reason — because they have a heart murmur, for instance, or a family history of heart disease. Should those children be exerciserestricted? What about surgery or medication? Right now, the right way to manage such cases simply isn’t clear, and that can have big consequences for children. If a young person on a sports scholarship is suddenly found to have AAOCA and, in the absence of evidence-based guidelines, is told to limit exercise, his life will be turned upside down. “We struggle with this every day,” says Brothers. “It’s very difficult because these decisions affect kids for the rest of their lives.” Donor support — including gifts from Valerie B. Bowman, the Michael H. Ludwig Memorial Foundation, the Cardiac Center, the Children’s Heart Foundation and others — has helped Brothers expand her work and conduct additional research on this devastating condition. “This is such an important anomaly,” she says. “It’s something we don’t know enough about, and we need to make sure we’re treating kids appropriately so they have the best quality of life in the safest environment.”
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Care for Life CHOP-Penn team provides expert care for adults with congenital heart disease
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hirty-year-old Joanna Stevenson has been a patient of The Children’s Hospital of Philadelphia all her life. Diagnosed with transposition of the great arteries as an infant, she had four surgeries and countless other procedures at CHOP, returning to the Hospital for care even after she moved to Alaska, where she works as an elementary school teacher. Recently, she turned to her CHOP team once again as she prepared for a major event in her life: her first pregnancy.
An Experienced Team Joanna transitioned her care to the Philadelphia Adult Congenital Heart Center, a joint program of CHOP and Penn Medicine that provides expert, multidisciplinary care for adults with congenital heart disease (CHD). The center’s team includes cardiovascular surgeons, cardiac anesthesiologists, and specialists in catheterization, electrophysiology, noninvasive imaging, heart failure/transplantation, genetics and reproductive services, all of whom have vast experience treating adults with CHD, who require ongoing subspecialty care throughout their lives.
Joanna, shown here with her daughter, Anja, is a patient of the Philadelphia Adult Congenital Heart Center.
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adult. As survivors pass through adolescence and into young adulthood, they have to learn to understand their disease and advocate for themselves, especially since some adult physicians may not fully understand CHD. They also have to understand their personal responsibility for their health, such as avoiding high-risk behavior that many teens and young adults wouldn’t think twice about. Their primary CHOP cardiologists and nurse practitioners discuss these issues with the kids as they prepare to hand them over to adult care.
babies — face. For some women with CHD, Kim says, pregnancy is clearly something they should not consider. But for most, it’s an option. In any case, these are intense and emotional discussions.
The center also has a strong research program, which was especially appealing to one significant donor to its work, Big Hearts to Little Hearts, a group of grateful parents of Cardiac Center patients. Their children are truly pioneers, many of them having survived heart defects that would have been fatal just a few decades ago, and there is still much to learn about the best ways to care for them as they grow up.
Kim worked with a multidisciplinary team on regular screening and care to ensure that Joanna’s pregnancy and delivery were as uneventful as possible. A fetal echocardiogram performed at CHOP showed no problems with the baby girl’s heart — and the pregnancy, Joanna says, was stress-free. “I felt so good and was doing so well,” she says. “Once I knew her heart was OK, I really wasn’t worried.”
“Our mission is to lengthen and improve the lives of the littlest heart patients,” says Lynne Accisano, the organization’s president. “We want to do everything we can to help find medications and treatments that will help these children.”
On Sept. 21, baby Anja was born at the Hospital of the University of Pennsylvania at full term.
A CHD Survivor
Thanks to advances in caring for children with CHD, there are now more adults living with CHD than children, opening up a new field of medicine for which adult cardiologists need to learn from pediatric cardiology. For now, specialists at the center care for these patients and build the field for future survivors.
Joanna is one of the many patients who have benefited from research advances made over the past few decades. During her appointments with Yuli Kim, M.D., director of the Philadelphia Adult Congenital Heart Center, Joanna learned that though she was considered to be at very high risk — she has a severe heart arrhythmia and had a pacemaker implanted at CHOP in early 2012 — pregnancy was definitely something she could consider.
The center starts its work as survivors become teenagers, involving them in their own transition to living with and finding care for CHD as an
Kim speaks to all of her patients of childbearing age about family planning, outlining the risks pregnant women with CHD — and their
“We’re not just talking about a woman’s heart — we’re talking about her ability to have a family,” says Kim. “And having a family is the ultimate declaration of life — that you are a survivor, that you’ve made it.”
A Special Birth
Joanna’s successful pregnancy is a testament to the extraordinary level of care CHOP provides — and to how far care for patients with CHD has come. Patients who once would have faced terrible odds can now have full lives — and the Cardiac Center team is right there with them, every step of the way. “The care we provide doesn’t stop when a patient turns 18,” says Kim. “It continues on and on.”
In addition to Big Hearts to Little Hearts, funding for the Philadelphia Adult Congenital Heart Center comes from the Children’s Heart Foundation, the Adult Congenital Heart Disease Coalition and others.
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Ahead of the Curve Pioneering anesthesiologist to join Cardiac Center
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n anesthesiologist whose groundbreaking research promises to buy precious time for patients starving for oxygen is joining The Children’s Hospital of Philadelphia in 2013. Five years ago, Frank McGowan, M.D., first began investigating ways to get more oxygen into the blood of patients who were breathing poorly or not at all. He collaborated with a group of scientists to discover whether it was possible to bypass the lungs completely. It took several years, but the team was eventually able to wrap oxygen in nontoxic lipids, creating microbubbles tinier than the smallest blood vessel. These microparticles can be injected directly into the bloodstream to deliver oxygen, then collapse so the body can eliminate them safely. The research made the cover of Science Translational Medicine in June 2012 for its successful use in animals and was lauded in the December 2012 issue of Scientific American as one of the year’s top 10 “world changing ideas.”
Nico had his first open heart surgery at CHOP when he was 11 days old. He had another open heart surgery at 3 1/2 months of age.
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The technique won’t be ready for clinical use with humans for another five to 10 years; the research team still needs to determine the best delivery system and prove its safety. However, the potential alone was enough for James M. Steven, M.D., S.M., chief of the Division of Cardiac Anesthesia and a member of the Cardiac Center Executive Committee, to recruit McGowan to CHOP from his current position at the Medical University of South Carolina Children’s Hospital. “The original intent of this work was short-term rescue in crisis situations,” Steven says. “However, it has potential for longer-term treatment of patients awaiting surgery whose heart disease
Frank McGowan, M.D. results in inadequate delivery of oxygen to the body. This work is an extension of his career interest in advancing our understanding of the mechanisms of cell injury with congenital heart disease and treatments such as cardiopulmonary bypass.” Indeed, McGowan’s other passion is developing innovative treatments to prevent or reverse reduced cardiac function and heart failure in patients with congenital heart diseases, especially those that affect the right ventricle. His group is currently examining therapies based upon cell transplantation or drugs targeted at novel biochemical mechanisms. He is particularly excited by preliminary results suggesting that they can isolate and manipulate the individual patient’s own cardiac cells in the laboratory and then return them to the patient to improve cardiac performance. In addition to his research, McGowan maintains a clinical practice in pediatric and neonatal cardiac anesthesia — something he will continue to do as a member of the CHOP Cardiac Center team. “We recruited a gem,” says William J. Greeley, M.D., M.B.A., anesthesiologist-in-chief and chair of CHOP’s Department of Anesthesiology and Critical Care Medicine. A R e p o r t o n Yo u r I n v e s t m e n t i n H o p e
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News Briefs Cardiac Center Research Helps Mothers Facing Prenatal Diagnosis of Heart Problems A CHOP study published in the September 2012 issue of The Journal of Pediatrics showed that mothers whose children were diagnosed with a congenital heart defect prenatally were at risk for post-traumatic stress disorder, depression and anxiety. The study also identified possible protective factors. Cardiac Center researchers expected these mothers would be at higher risk for mental health problems — and found more than 39 percent of the women in the study experienced clinically important traumatic stress, 22 percent experienced depression, and 31 percent experienced various states of anxiety. But prior to this work, the risk had not been studied and proven. Even better than scientifically demonstrating the risks were the additional findings that satisfaction with their partners and use of positive coping mechanisms, such as acceptance of the diagnosis and use of support networks, reduced those risks. The research was partially funded by a gift from Anne Bazik and was led by Jack Rychik, M.D., medical director of the Cardiac Center’s Fetal Heart Program and the Robert and Dolores Harrington Endowed Chair in Pediatric Cardiology.
CHOP Innovates Valve Replacement Procedure Children with the complex heart defect tetralogy of Fallot undergo their first open heart surgery in infancy, with more to come throughout life. But over the past several years, interventional 22
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cardiologists at CHOP have been performing a noninvasive procedure to replace a child’s pulmonary valve through a small incision in the groin, potentially reducing the number of open heart procedures the child will need over his or her lifetime. CHOP offers this procedure combined with an advanced imaging technique called XMR, which combines X-ray and MRI images to create a detailed 3D picture of the child’s heart. “The more anatomic detail we can see, the better and the safer the procedure will be,” says CHOP interventional cardiologist Matthew Gillespie, M.D. “Before we even begin the invasive portion of the procedure, we have a complete understanding of the complex anatomy from the MRI, which provides exquisite anatomic detail without additional ionizing radiation. This is different than most centers in the world.” CHOP currently performs 25 to 30 transcatheter valve replacements each year, and is the only pediatric center in the region to offer this procedure. Gillespie and his team are researching how to apply this technique to other types of pulmonary and mitral valve replacement, making noninvasive heart surgery available to more children.
Kiona Allen, M.D., Named 2013 Ann Newman Fellow This spring, Kiona Y. Allen, M.D., was named the Ann Newman Fellow for 2013, earning the distinguished award for her exemplary work and the promise she shows for future outstanding performance in the area of clinical care.
Now in its 24th year, the fellowship is awarded annually to a second-year fellow and was named for Ann Newman, whose estate created the endowment supporting the fellowship. Ann’s mother, Ida Newman, continues to give generously to support the Cardiac Center in other ways. The Ann Newman Fellowship has helped form the careers of gifted clinicians and researchers who have gone on to productive careers at CHOP and across the country. Endowed fellowships ensure that funds will always be available for young investigators to carry out their research studies, complete their training, and continue to enhance the clinical care of pediatric patients with heart disease.
Single Ventricle Survivorship Program Establishes New Research Database Led by Jack Rychik, M.D., the Single Ventricle Survivorship Program has revealed ways treatment can be optimized at every stage to maximize both survival and quality of life for children with single ventricle conditions, but much is still unknown about the long-term outcomes for these patients. For the past two years, the program has been building the infrastructure to support research into this area to better understand what patients can expect and how their long-term health could be improved. Philanthropy from Mark and Stacy Harrington, Robert Klimasewski, The Children’s Heart Foundation — Nevada, and others supported development of a database that will include the experience of every single ventricle patient Children’s Hospital has treated since 1984. Data collection is scheduled to begin this year and will allow CHOP researchers to conduct
many studies, from establishing a baseline of survivors’ typical experience as they age to exploring what factors early in treatment predict better quality of life later on. That could help identify potential areas for treatment advances for children with single ventricle problems in the future.
Philanthropy Supports Drug Development Many of the pharmaceuticals used for cardiovascular disease have very challenging chemical properties that make injections difficult or render impossible novel delivery systems designed to enhance therapeutic effects, such as nanoparticles. With support from the Erin Beatty Endowed Fund for Cardiology Research, Robert Levy, M.D., who holds the William J. Rashkind Endowed Chair in Pediatric Cardiology, Michael Chorny, Ph.D., and Ivan Alferiev, Ph.D., are addressing this problem by creating new drug fusion compounds, either combining two active drugs or attaching medications to inactive agents. The goal of both techniques is to help make the drugs both more effective and more compatible with the various routes of administration. Research is also under way on existing drugs that could inhibit the progression of aortic or mitral valve disease. The Kibel Foundation supported a published DNA-microarray study using heart valve cells from pigs that showed increased amounts of certain serotonin receptors, indicating these were potential drug targets for new therapies. Ongoing studies have shown these same receptors appear to be involved in human heart valve cells. These studies have led to a recent NIH grant application, and experiments in the next program year will focus on these discoveries to identify potential new treatments for heart valve disease.
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Endowed Chairs Robert and Dolores Harrington Endowed Chair in Pediatric Cardiology Jack Rychik, M.D. William J. Rashkind Endowed Chair in Pediatric Cardiology Robert J. Levy, M.D. Daniel M. Tabas Endowed Chair in Pediatric Cardiothoracic Surgery J. William Gaynor, M.D. Evelyn Rome Tabas Endowed Chair in Pediatric Cardiology Victoria L. Vetter, M.D. Josephine J. Templeton Endowed Chair in Pediatric Anesthesiology Clinical Education Susan C. Nicolson, M.D. Jennifer Terker Endowed Chair in Pediatric Cardiology Robert E. Shaddy, M.D. Alice Langdon Warner Endowed Chair in Pediatric Cardiothoracic Surgery Thomas L. Spray, M.D.
Endowed Funds Erin Beatty Endowed Fund for Cardiology Research William Ash Gallant Fund for Cardiac Research Benjamin Kirschner Cardiovascular Research Fund Tommy Martin Memorial Fund Aaron Morton Endowed Fellowship in Pediatric Cardiology Ann Newman Fund Lauren Nowicki Endowed Fund Thomas Garrett Rauch Memorial Fund Christine Yerkes Social Work Emergency Relief Fund for the Cardiac Center Maya, 3, treated for hypoplastic left heart syndrome
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Let Your Legacy Be the Gift of Childhood Evett, 15, heart transplant recipient
On the cover: Stosh, 8 months, treated for hypoplastic left heart syndrome
Thank You The children you see throughout this report are
Our care, our research, our teaching. They all rely on gifts from Children’s Hospital supporters, now and in the future.
our patients. It is for them, and for the many others in our care, that we relentlessly pursue the next advances in treatment and research. Your support is vital to the work of the Cardiac Center.
Many of the most important and enduring gifts to CHOP have been legacy gifts. Including Children’s Hospital in your will, or as a beneficiary of a retirement plan, is one of the easiest ways to invest in the health and well-being of future generations of children. These gifts won’t affect your current cash flow and may even help reduce taxes for loved ones. If you have already made a planned gift to CHOP, thank you, and please let us know. We can help you designate your gift to a specific Hospital program, and we’ll invite you to join the Lewis Society, a select group of supporters who have included Children’s Hospital in their plans. Visit us at giftofchildhood.org/plannedgiving to learn more, or contact Tom Yates, director of planned giving, at 267-426-6472 or yatestg@email.chop.edu.
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To learn more about how you can help, please contact Meghan Tumolo at 267-426-6498 or TumoloM1@email.chop.edu.
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The Children’s Hospital of Philadelphia is ranked No. 1 on Parents magazine’s list of Best Children’s Hospitals in the nation and No. 1 on the U.S.News & World Report Honor Roll. The Cardiac Center shares Parents’ No. 1 ranking for Heart Care.