National Donate Life Month – Becoming A Donor

For the month of April, PCHA will be focusing on the theme of National Donate Life Month. In the second post of our series, Jennifer Weiner, an adult CHD patient, shares why she feels passionately about organ donation and how to sign up as a donor.

 

A while back, I got a letter from Jesse White, Illinois’ Secretary of State. It’s not like we’re pen pals, but when I renewed my driver’s license the state sent a friendly thank-you for preserving my organ donor status. Back in 2006, I signed up on the First Person Consent Registry, and I love getting that letter every four years. I love showing it to my family, saying, “Go to this web site and sign up” (Go to this website and sign up http://www.lifegoeson.com/)!  I love the reminder that I made an active, conscious decision to help someone else.

To me, signing up on the registry, promising a part of ourselves to a stranger, is one of the most amazing yet simplest ways of tying us all together. This ad, which caught my attention back in 2007, has stuck with me 10 years later and demonstrates that idea perfectly.

 

I remember when I turned 16, the First Person Consent list didn’t exist. I, no doubt about it, signed the back of that very first driver’s license. Even then, organ donation was something I felt strongly about. I wanted to make sure everyone knew my wishes; I insisted my friends and family follow through with donation under any circumstances.

When the registry came out, I read all the details and signed online immediately. The website explains that your status on the list isn’t available until after you pass, you can change your mind, and it’s legally binding, so even if your family disagrees with your decision, your wishes will be honored.

I’m sure by now you’re wondering if Jesse White’s letter asks for marketing help, but I swear it didn’t.  By my best estimation, this all started with me in 8th grade. My sister and I were prayer partners with Paige´ Wilsek – we went to Catholic School. I will never forget it. She was in third grade and suffering from cancer, which started as Leukemia and spread to her bones. Our church held a donor search to find a bone marrow match for her. The chances of finding one were pretty slim, because of her rare blood type. They never found a match. Paige´ died before she finished 4th grade. I remember how hard it was to go to her wake and funeral. I couldn’t stop thinking about all the things she would never get to do, the life she should have had. Her mother wound up comforting me instead of the other way around.

Typically, you’d think of an organ donation as a whole heart or a kidney, but, in reality, even one vital healthy piece can save someone’s life, like the bone marrow Paige´ never got. It stuck with me then, and hit closer to home when I was 17 and received a donation of my own.

In 1999, I received a pulmonary valve and conduit homograft. At first, I thought of it as some disembodied pulmonary artery sitting in a freezer somewhere. It wasn’t until someone asked me whether or not I was going to send a thank you letter to the family that it hit me. I was alive and healthy thanks to someone else’s final gift. I never did send a thank you, and still feel a bit guilty about that.  Perhaps the best way to say thank you, though, is to pay it forward. I want to give whatever I can in the end, in hopes that it will give someone else a second chance.

So for all of you that haven’t signed the First Person Consent Registry to become an organ donor, go to http://www.lifegoeson.com/ and sign up.

 

*Please note each State has its own policy/procedure for organ donation registration. Learn more about organ donation and the policy in your State,  or to register and learn more about  various types of donation, please check out Donate Life.

 

 

Jennifer is a graduate of DePaul University, with a degree in Elementary Education and an MA in English and Creative Writing from SNHU. She is a 35 year old adult congenital heart patient, born with Truncus Arteriosus, has had two repair surgeries, and is an ICD recipient. Jennifer volunteers for the Pediatric Congenital Heart Association, both nationally and locally, managing the PCHA Blog and IL Chapter Communications. She also serves on the steering committee of Chicagoland Cardiac Connections, an organization that provides support and resources for patients with cardiac devices, based out of Lurie Children’s Chicago.

National Donate Life Month – The Wait

For the month of April, PCHA will be focusing on the theme of National Donate Life Month. In the first post of our series, Bill Coon, professional author and speaker, shares thoughts on his wait for a multiple organ transplant.

 

I didn’t see the point in journaling yesterday. The day as a whole was very monotonous. My only visitor was my mom. She just sat in my room all day as I walked the halls of the CCU and worked on my homework.

 It seems the longer I wait for my transplants, the more I begin to think about the life of my donor.

 What are they doing right now? What will cause them to die? If they are driving in a car when they are killed, where were they going? Were they happy when they died? Will they find inner peace before they pass? Or did they just finish fighting with a loved one and will never get a chance to say they were sorry? Is my donor a good person? Do they have many regrets from their past? What are their future aspirations that they want to accomplish but will never get a chance to complete? Do they have a family? Are they alone? Will they be scared when they pass?

 My mind races with these questions on monotonous days. I find it shocking to believe that my donor’s life is so rich at the moment. They have no idea what is to come in the near future, nor do they have any idea as to whom I am and how horrendously awful my life has become in a matter of three and a half months.

 Like always, I am trying my hardest to push those thoughts aside. I try to look for the bright spots in my life, but I still can’t shake the thoughts.

 I hate monotonous days.

I wrote those words from my hospital bed in Chicago on Thursday, September 24, 2009. The very donor I referenced would save my life exactly twenty-seven days later when he or she would gift me their heart and one of their kidneys.

A photo from the hospital illustrating Bill’s neck post-procedure.

I truly believe that one of the largest misconceptions of organ donation is that the recipient’s life goes back to normal the second they exit the hospital with their clean bill of health. While the physical transition is incredibly quick, the mental transition from experiencing “the wait” is one that takes years to overcome. However, please know that I use the term “overcome” very lightly. For the realization that a stranger must die for you to live is more humbling than the realization of your own mortality. The realization causes you to answer a barrage of interpersonal questions. You spend your days in the hospital (and months, if not years) post-surgery questioning why you were saved. You ask yourself, time and time again, Why did the universe choose me over my donor? These questions, of course, can never be answered with any absolute certainty. However, they do force you to reevaluate your life and begin making decisions that not only honor the miraculous gift you received, but also the life of your donor.

The memories that you develop from “the wait” only deepen your new sense of responsibility to live a good life post-transplant. While I have many memories from “the wait” there are two that changed me the most. These two memories find a way to rise from my subconscious in the moments of my post-transplant life where I begin to stress about petty obstacles. It is as though my brain, in a beautiful way, reminds me that as long as I have my health and my loved ones, there are no problems that cannot be easily rectified and resolved.

The memory I would like to share with you is of a ritual that I had each night of my 70-day wait for a new heart and kidney. My doctors told me that I would be notified of a perfect match via a call to a tiny, white-yellow phone that rested on a nightstand at the head of my hospital bed. In turn, while I waited for the phone to ring, I began to obsess over the fantasy of hearing the tone. Consequently, each night I went out of my way to make sure I was ready to answer the phone at any hour of the night.

Prior to bed, I would take my food tray and set it next to the right side of my bed near my hand. I would then reach over the bed and grab the phone. After stretching the phone cord, I would neatly place the phone atop my food tray. Next, I would place a pillow on the left side of my body because I developed a tendency of banging my left arm against the bed rail while I slept. I would then turn the lights off and I would begin to pray. I would begin to beg for God, the universe, for anybody or anything to save me. I would then transition to the final part of my ritual where I would lie in bed for hours feeling absolutely terrible that I had, technically, just prayed for someone else to die.

This memory is a perfect microcosm of “the wait”. Though you spend your days in organ failure feeling miserable, for the most part, you are rarely left alone. The time spent with others is typically discussion surrounding your medications, upcoming appointments, procedures and what life will be like once you receive your organs. In the rare hours when you are left alone, you spend those hours thinking of the donor. The donor never leaves you before and after they become a part of you.

It is with this in mind that I would like to encourage everyone to become an organ donor. In doing so, not only will you save a life, but also you will be loved and honored each day after your passing by the recipient of your generosity.

Bill just moments prior to his second heart transplant.

 

 

Please use this link to find your state’s donor registry and become an organ donor today.

 

 

Bill Coon is a two-time heart transplant recipient, kidney recipient, HLHS survivor, author and professional speaker. His book, SWIM: A Memoir of Survivor has been read by thousands internationally and has aided countless individuals in overcoming the mental battle of a chronic illness. Click here to learn more about Bill.

National Reading Month: You’re Here for a Reason

For the month of March, PCHA will be focusing on the theme of National Reading Month. In the 3rd post of our series, PCHA’s Jessica Chenevert talks with us about one special book. Read on to hear why You’re Here for a Reason holds a special place in her heart.

Barrett checking out his book

When I bought this children’s book, I had no idea that the first time I would read it to my son, Bear, I would be brought to tears. Never in my life has a book made me cry. The book is titled, “You’re Here for a Reason,” and it’s written and illustrated by Nancy Tillman. I decided to buy it, because I loved another one of her books, “On the Night You Were Born,” which Bear had received as a gift for his first Christmas. Not only does the book have a beautiful rhyming phrases, but it is written in a way that floats off the pages and can truly relate itself to your life. In my case, the words reached through the pages and spoke to me about Barrett’s journey with Congenital Heart Disease. The book, as far as I know, has no relation to heart disease and wasn’t written with a heart kid in mind, but every word I read applied to it perfectly.

Barrett is here for a reason. Every one of us is. Nancy shows her readers how each of us fits into life’s big picture and how this world would simply be incomplete without each of us in it. As we sat there after reading the book, I held my sweet, strong warrior and rocked him back and forth and thought it was such a perfect gift for children and families living with CHD.

One of the fun things I am able to do at PCHA is pick a quote and pair it with photo to create an inspirational design of sorts. Naturally, I created one using a passage from one of these great books and posted it on social media. To my great surprise, the author herself engaged in the post. I immediately reached out to her on my personal account about my love for her books and sent her a picture of Barrett.

Of course, she responded to those also, and I was overjoyed. A small interaction, but one that would stay with me. Little did I know it would not end there. Last September, I had the privilege of being honored at the Congenital Heart Gala in Milwaukee with the Heart Hero of the Year Award. Our Director of Programs, Amy, knowing my love for Nancy Tillman books, reached out to the New York Times best-selling author so that when I walked up to accept the award I would also have a copy of “You’re Here for a Reason” made out to Barrett, complete with a signature.

Ezra on his 2nd Birthday

Giving parents words that can match the feelings they have for their children is Nancy Tillman’s goal in creating these books. Before I even knew about that, I had felt it. I had already decided it would be the book I wanted to gift to other amazing little warriors and their parents. Just one month earlier, I had given it as part of a gift from Barrett to his buddy Ezra on his 2nd birthday. Now, Barrett and Ezra were born roughly two weeks apart, they had all the same newborn clothes, underwent surgery at the same hospital, and were both living with CHD. Ezra’s battle had been particularly rough, but Ezzie’s mom and I liked to call them twins, because even with different defects, different struggles, and ultimately different outcomes…they were brothers on this journey.

This book has a special place on Barrett’s book shelf and in my heart. Each time we read it, I hope he knows how truly amazing and strong he is and how much he is loved.

 

 

 

Note from the Author:

Dear loving parents,

My own grandchild was born with a hole in her heart, so I know much of the struggle that you are going through.  She is well and happy now and that is what I wish for all your precious children.  I wrote You’re Here for a Reason to speak from your heart. I so dearly hope it does.  Your child is miraculous.

Blessings,

Nancy Tillman

 

 

 

 

Jess lives up the North Shore of Minnesota with her husband Nolan and their son Barrett “Bear” and their dog Bailey. Barrett was born with Transposition of the Great Arteries & Coarctation of the Aorta. With a background in several aspects of the medical field Jess. Joined PCHA shortly after Barrett’s Birth and subsequent diagnosis. Now a stay at home heart mom she strives to be an active volunteer as an advocate, committee member, and Social Media Coordinator for PCHA.

National Reading Month: A Q&A on The Love of Reading with Members of the CHD Community

For the month of March, PCHA will be focusing on the theme of National Reading Month. In the 2nd post of our series, PCHA asked members from across the spectrum of our CHD Community to tell us what they love about reading. Sharing with us this week, about what reading has brought to their lives, are Megan Setzer from the perspective of her son Caleb, born with Hypoplastic Left Heart Syndrome, Alison Connors, mother of two heart warriors, McKenzie and Archer, and Carol Raimondi, an Adult CHD patient with Congenitally Corrected Transposition of the Great Arteries.

 

PCHA:  Why do you love to read?

Megan + Caleb: I’m still learning to read. I love being read to. I like feeling like I’m part of the story.

Alison: Reading is a great escape for me. When I read I am able take time to myself to decompress. Life can be stressful and having that outlet is so important. Reading is probably the most important skill you learn besides math.

Carol:  I love to read because it relaxes me. It allows my mind to focus on the story, and take me to wherever it is set. I can be in a place where the characters are, and that lets me temporarily forget any stresses or worries I have going in my life. 

 

PCHA: Where’s the best place to settle in with a good book?

Megan + Caleb: Anywhere. My favorite place is to snuggle into my bed and look at books before I go to sleep at night.

Alison: The best place to read a book is anywhere! A good book can be read in the loudest place imaginable, because once you start reading you are taken to a different place anyway!

Carol: I love stretching out in bed when I read. Being somewhere with little distraction is key!

 

PCHA: What’s the best part of your favorite book?

Megan + Caleb: Everything! I don’t really have a favorite.

Alison: For me, I like to read historical biographies of famous figures, like Abraham Lincoln, Che, Jackie O and Benjamin Franklin. Reading about the lives of historical figures and about our past customs in American history is a favorite of mine.

Carol:  I really don’t have one favorite book. Anytime I can relax and read for an extended period of time makes me happy!

 

PCHA: What character, from any book, do you most identify with?

Megan + Caleb: There is a small part of all the characters that I can identify with. I am strong like the Super Heroes that I read about and I’m silly like the funny creatures in the Dr. Seuss books.

Alison: When reading historical biographies I don’t necessarily identify with the characters I read about, but one thing about reading about someone’s history is that the past does sometimes repeat itself so I can see how America and the world hasn’t really changed as much as we all assume it has. Technology has changed but as human beings we are still fighting over the same things, fighting for the same things and uniting about the same things. It’s very interesting.

Carol: I tend to identify with any female lead character. Someone who has obstacles to overcome time and time again, but stays strong and shows perseverance. 

 

PCHA: Do you have a favorite author or genre? What do you like about that style?

Megan + Caleb: Dr. Seuss is my favorite author, because his books are funny.

Alison: Historical biographies are pretty much all I read. I figure I’d like to learn something while I am making the time to read. Abraham Lincoln is probably one of the most interesting people I have ever read about. How he ran the country, how he united with his rival, his views on leadership, and work ethic really impressed me. As a person you are always evolving, so whenever I can learn something from reading, I feel accomplished.

Carol: I tend to vary on the genre I read. Sometimes I will read medical non-fiction, as I enjoy learning about disease management and how others have handled living with chronic illness. Other times, I prefer to go the exact opposite and stay light by reading fantasy books. Anything with a vampire or werewolf will suffice! It is nice to escape reality and let my imagination go wild!

 

PCHA: Have you ready any books involving characters with CHD? How’s they hold up to your own experience?

Megan + Caleb: I have read the book, Zipline…It’s about a girl who had a heart surgery, and she is all better now.  Unlike this character, I have had multiple heart surgeries.  I also have a hard time keeping up with my friends when they are running around the playground.

Alison: I have not yet read any books involving characters with CHD! I will have to look into that! Every CHD story is different, which probably why CHD is not as well-known as I would like. Having over 35 different types, so many different outcomes, and sometimes having multiple CHDs really makes every Heart Family’s perspective completely unique, but I think each of us, whether your child’s ASD closed by itself or whether you child is on his/her 4th surgery, we’re all the same. We’re shocked, worried parents who strive to give our children the best life possible, and we’re not alone in this CHD heart journey. Heart parents and patients are pretty passionate people, so I think I would definitely relate to a book with a CHD character.

Carol: I have read several books with CHD patients as the main character. In some cases, I have identified completely with the feelings they have shared, to the point where it hits too close to home, and I need to put the book down for a few days. In other circumstances, I felt like the CHD patient did not give a good representation of what life is like with heart defects. It was either overdramatized or made to seem like it wasn’t a big deal. I prefer a happy medium between the two.

 

PCHA: What’s missing from bookstores? What book would you like to see on the shelves?

Megan + Caleb: I don’t know what’s missing from the bookstores, because most of my books come from the school Book Fair, in the mail, or are on my tablet. I love it when the Book Fair comes to my school and seeing all the different types of books that they have!

Alison: We lost my husband’s Mother, Nicky, suddenly last year. She was a fiction writer. She worked hard for her kids, and, when they were grown, she worked hard for herself. She went to Columbia College in Chicago and worked there as well. She was the coolest person to have as a Mother-in-Law; every time I talked to her she challenged my thinking, inspired me to be a strong woman, and always gave my husband a hard time, which was fun to see! I always thought we would have more time with her. I would love to see her works put into a book. She always talked about having so many works, pages and pages of unfinished work, a life unfinished. Maybe one day, as a family, we can make that happen. I would love to sit one day, open a book by Nicky Chakalis, and read it to the next generation of our family. For me, that’s what is missing on the bookshelves. 

Carol: I would love to see more books geared towards teens on living with chronic illness, whether CHD or not. I think this an important age group that needs as much information as they can get.  What book would I like to see on the shelf? A manual for life with CHD, from pre-natal diagnosis through adulthood, with chapters specifically for family members, friends, spouses and health care providers. That’s not asking too much, right?

 

Megan Setzer is a mother of a child with Hypoplastic Left Heart Syndrome. With a background in Social Work, it has become her passion to provide support and resources to those impacted by CHD.  Shortly after her son’s birth in 2009, Megan chartered Mended Little Hearts of Winchester as a way to connect area families and to offer support and resources. Under her leadership, the group was honored with the 2012 MLH Group Excellence Award and it continued to grow its network. In 2013, her group expanded geographically and became Mended Little Hearts of the Shenandoah Valley, serving families in the Shenandoah Valley of Virginia and Panhandle of West Virginia. She was elected as the Mended Little Hearts National Board Director in 2013, where she served until December 2016. She is currently serving as a Board Director for the Pediatric Congenital Heart Association.Megan has a Bachelor’s of Social Work from James Madison University, graduating in 1999.  She worked for almost 10 years as a Medical Social Worker, and is now employed in the health technology field as a Clinical Advisor, specializing in care transitions. When she is not volunteering, she is kept busy by her husband and two boys. She loves to read and crochet.

Alison Connors is a mother of three children: McKenzie, Jackson and Archer. Her oldest McKenzie and youngest Archer both had open heart surgery for congenital heart defects. McKenzie and Archer have been in the care of the PSHU team at Advocate Children’s Hospital in Oak Lawn, Illinois since birth. Alison has been married since 2009 to her best friend Christopher, and she recently went back to work as a 1:1 teacher’s aide for children with special needs. She and her family have a busy life, but she has a passion for volunteering and believes that there is healing power in taking part in something that is bigger than yourself. Having two children with CHD threw Alison onto a path she never expected to be on, but a path her family is very grateful to be on. It’s an honor for Alison and her family to share resources, give hope, and support to others who face the same situations that they have.

 

Carol Raimondi is an adult CHD patient and nurse, living with Congenitally Corrected Transposition of the Great Arteries.  She has had 4 open heart surgeries in her 40 years, as well as a pacemaker since the age of 6. After spending a large part of her childhood in and out of hospitals, she developed a passion for nursing. She went to school to become a cardiac nurse. Carol’s many hospital experiences helped her as a nurse to better understand what her patients were going through and  to care for them with that much more compassion and empathy.

Due to worsening medical issues, Carol had to give up the profession she loved. That did not stop her from being a patient advocate, however. She joined her local hospital’s’ Patient Family Advisory Council, which she now co-chairs, and shortly thereafter she joined Mended Little Hearts Chicago(MLHC) as an adult CHD liaison. She then expanded her work in the CHD community by starting an adult and teen CHD group within MLHC and became an  Ambassador for the Adult Congenital Heart Association. Currently she sits as the Pediatric Congenital Heart Association of IL(PCHA-IL) Vice President and State officer, after a recent transition from MLHC. Her proudest moments are when she is advocating and raising awareness for the CHD community, both on Capitol Hill in D.C. and locally.

National Reading Month: It’s My Heart Book

For the month of March, PCHA will be focusing on the theme of National Reading Month. In the first post of our series, the Children’s Heart Foundation shares an overview of the It’s My Heart book, a resource for families affected by CHD. PCHA and the Children’s Heart Foundation are partnering to include the It’s My Heart book in every Conquering CHD Kit. 

 

 

 

Receiving a congenital heart defect (CHD) diagnosis is often an emotional, confusing and overwhelming time.

In 2003, The Children’s Heart Foundation (CHF) – along with a dedicated group of doctors, nurses and parents – set-out to address a dire need noted by patients with CHDs and their loved ones. Although doctors and nurses often explained things well, written materials were either too simple (pamphlets on how the normal heart works) or too complex (medical journals). There was a need for a resource guide with clear writing and illustrations on the most common kinds of heart defects, tests, equipment, surgical procedures and medications.

Published in 2004, It’s My Heart, was written in plain, understandable language. It provides descriptions of the types of CHDs, explanations of the various tests, surgical procedures and treatments for CHDs. This book also contains helpful “before and after” diagrams, a glossary of medical terms, and much, much more.

“Hours after my son was first diagnosed with a CHD at 2 days old, our nurse handed me a copy of It’s My Heart in the hospital. I remember being so scared and confused at that time; it felt as if everything was broken,” said Sally Powers, CHF Executive Board Member & Heart Mom to Sam.

“But that book was one of the first resources I had to help lift me up and move me forward on our journey with CHD,” Sally continues. “And that made me feel hopeful.”

Today, in its’ second edition, It’s My Heart is still a widely used resource book. CHF distributes this book to patients, parents, family members, healthcare professionals and friends of congenital heart defect patients worldwide.

It has always been a high priority for CHF to offer the book free-of-charge. Thanks to support from Medtronic Foundation, Northwestern University Dance Marathon 2011 and various grants, CHF has been able to continue to do so.

“I’ll always be thankful that The Children’s Heart Foundation knew the need for newly diagnosed parents to have that kind of easy-to-understand resource at such a critical time,” Sally said. “My son is 8 now and I still refer to It’s My Heart often.”

To order your free copy of It’s My Heart, please visit: http://weblink.donorperfect.com/Itsmyheartbookorder.
If you have any questions or to place an international order, please contact Rosemary at RWheeler@childrensheartfoundation.org.

 

 

About The Children’s Heart Foundation

The mission of The Children’s Heart Foundation is to fund the most promising research to advance the diagnosis, treatment and prevention of congenital heart defects. For more information, please visit:www.childrensheartfoundation.org. Follow us on Facebook and Twitter.

 

Research Blog Series Recap

For the month of October, PCHA recognized the importance of Research. All different kinds of research are vital to finding a treatment and an eventual cure for CHD and we thank all the clinicians, scientists, and patient volunteers for their important contributions to CHD research. The fight to conquer CHD is strengthened by the involvement of all of those who share our mission. It takes a community coming together around education, research and awareness to make an impact. Volunteer, join our advocacy network today, and connect with us on Facebook! In case you missed a post, below is the full Research Blog series:

The Importance of CHD Surveillance

Psychosocial Functioning of Adolescents with D-Transposition of the Great Arteries

Genetics and Genomics Research – Why It Matters

Genetic Link Between CHD and Neurodevelopmental Disorders

Why Should My Child Participate in Clinical Research?

Research Matters: Why Should My Child Participate in Clinical Research?

research matters

As we continue with our theme of research for the month of October, PCHA welcomes back NIH medical officer and pediatric cardiologist Dr. Kristin Burns. Dr. Burns answers some frequently asked questions about clinical research and provides information about ongoing clinical research studies.

 

 

Have you ever noticed flyers posted in the waiting room of your child’s healthcare provider’s office advertising clinical research studies? Has your child’s healthcare provider ever invited you to learn more about a research study or asked if you want your child to participate in clinical research? If so, have you wondered, “What is clinical research and why should my child participate?”

What is clinical research?
  • Clinical research is a series of tests or observations that help scientists learn about how safe or effective medications, devices, and treatments are in humans or how diseases progress over time.
  • A clinical trial is a specific type of clinical research study that compares treatments against each other. Participants are often assigned randomly (like a coin flip) to one treatment or another and their outcomes are compared.
  • Clinical research is different than the medical care your child receives from their healthcare provider. Research tries to understand whether a treatment may help a group of people with a certain condition in the future. Medical care focuses on the individual needs of a single person at the present time.
Why is it important for children to be in clinical research studies?
  • Many medicines used in children have not been tested in children to see if they are safe or if they work well. Because children are not just small adults and are still growing and developing, their bodies may work differently than adults, their health conditions may be different from adults, and medicines that work for adults may not work well or may be unsafe for children.
  • Therefore, it is important to do research studies involving children to test treatments and learn about pediatric diseases.
How does it benefit my child to be in a clinical research study?
  • By being in a research study, it is possible that your child might get access to newer drugs or treatments. Whether your child is assigned to get the experimental treatment, an existing treatment or a placebo (a sugar pill), your child is likely to have closer monitoring during a study, and you may learn more about your child’s condition by being in a research study.
  • It is possible that your child’s condition may improve by taking an experimental treatment. But it is also possible that an experimental treatment might not work better than existing treatments.
  • Your child’s participation may help other children with the same condition in the future. It may lead to the development of new treatments that work better, or it may prevent children from receiving a treatment that was proven in a research study to be unsafe or to not work well.
Is it safe for my child to participate in clinical research?
  • In addition to the doctors and nurses who will be monitoring the children in the research study, independent review boards, ethics committees, and safety monitoring boards have reviewed and approved the design of each study and will be monitoring its progress for safety.

It is your choice whether you want your child to participate in clinical research. Whether or not you decide to participate, your child’s medical care will not be affected.

 

What clinical research studies are going on now for children with congenital heart disease?
  • The Pediatric Heart Network, funded by the National Heart, Lung, and Blood Institute at the National Institutes of Health (NIH), performs clinical research studies for individuals with congenital heart disease.
  • The Pediatric Heart Network is currently enrolling participants in the FUEL Trial (Fontan Exercise Longitudinal Assessment). Teens who have had a Fontan operation and who are 12 to 18 years of age will be randomly assigned to 6 months of treatment with either a medication called Udenafil or placebo pills (that don’t contain any medication). The study will test whether treatment with Udenafil improves the ability to exercise. Previous studies have shown that, in people who have had a Fontan operation, decreasing ability to exercise over time is associated with worsening heart failure and increasing hospitalizations. This study hopes to identify a possible preventative treatment that could improve Fontan function over time and delay the development of heart failure. More information about the FUEL Trial can be found here.
  • Other clinical research studies may also be going on in your area or for your child’s condition. Ask your healthcare provider about other research studies that are available to your child.
Where can I learn more about clinical research?

Burns Formal PhotoKristin M. Burns, M.D. is a medical officer in the Heart Development and Structural Diseases Branch in the Division of Cardiovascular Sciences at the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health (NIH) and a pediatric cardiologist at the Children’s National Medical Center. Dr. Burns received her B.A. in Biochemistry and German from Wellesley College and her M.D. from the University of Massachusetts Medical School.

Research Matters: Genetic Link Between CHD and Neurodevelopmental Disorders

research matters

As we continue with our focus on genetics and genomics research, PCHA welcomes NIH medical officer Dr. Jonathan Kaltman. Dr. Kaltman provides an overview of a recent study that uses genomic data to establish a genetic link between congenital heart disease (CHD) and neurodevelopmental disorders. This study provides an important example of how genetics and genomics research can help us understand the genetic causes of CHD and other congenital anomalies.

 

The journal Science recently published a study performed by the Pediatric Cardiac Genomics Consortium evaluating the genetic cause of congenital heart disease (CHD). The investigators also tried to determine if genetics can explain why many children with CHD also have other medical conditions, including neurodevelopmental disorders and other congenital problems. You can find the complete study here.

About this Study:
  • The purpose of this study was to determine the genetic cause of severe CHD and its related medical problems.
  • Genetic sequencing was performed on 1,213 children with CHD and their parents and compared to families who did not have CHD.
  • Participants with CHD were also evaluated for neurodevelopmental disorders, such as learning disabilities or attention deficit/hyperactivity disorder, and other congenital problems, such as cleft lip.
Main Findings:
  • Children with severe CHD have a high number of spontaneous mutations.
  • The finding of a spontaneous mutation was especially strong in patients with CHD and another structural birth defect and/or neurodevelopmental disorders suggesting that these medical conditions happening together is likely due to a genetic cause.
    • Spontaneous mutations occurred in 20% of subjects with CHD, neurodevelopmental disorders, and another birth defect. They occurred in 5-10% of subjects with CHD and either a neurodevelopmental disorder or another birth defect. They occurred in only 2% of subjects with only CHD.
  • Many of the genes with mutations work in early development in both the heart and the brain, suggesting that a single mutation may cause both CHD and neurodevelopmental disorders.
  • Defects in certain genes result in a very high risk for developing neurodevelopment disorders associated with the CHD.
What this Means:
  • Neurodevelopmental disorders in children with CHD have often been thought to be caused by abnormal circulation and/or stresses associated with surgery and post-operative care. The findings from this study suggest that underlying genetics may also play an important role.
  • If these findings are repeated in other experiments, clinical genetic tests might be developed that can identify patients at high risk for developing neurodevelopmental abnormalities, enabling clinicians to target these patients for early therapy with the ultimate goal of improving their outcome.

These findings are helping to identify new molecular pathways that are important to heart and brain development improving basic knowledge of how the human body develops and providing understanding of the causes of various birth defects.


Jon KaltmanJonathan R. Kaltman, M.D., is Chief of the Heart Development and Structural Diseases Branch in the Division of Cardiovascular Sciences at the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health (NIH). Dr. Kaltman provides leadership for the Pediatric Cardiac Genomics Consortium and also helps oversee the Pediatric Heart Network. Prior to joining the NHLBI, Dr. Kaltman served as an assistant professor at the Children’s Hospital of Philadelphia at the University of Pennsylvania School of Medicine. He then joined the Children’s National Medical Center in D.C. as an assistant clinical professor where he continues to hold a part-time position. Dr. Kaltman received his B.S. in Molecular Biophysics and Biochemistry from Yale University and his M.D. from Emory University School of Medicine. 

Research Matters: Psychosocial Functioning of Adolescents with D-Transposition of the Great Arteries

research matters

Continuing our theme of Research, PCHA welcomes back pediatric psychologist Dr. Erica Sood. Dr. Sood provides an overview of research studies that highlight the importance of monitoring children and adolescents with CHD for psychosocial issues and that further reveal how parent/caregiver stress can affect the emotional and behavioral functioning of a child with CHD.

 

Psychosocial functioning of adolescents with d-transposition of the great arteries

By Erica Sood, PhD, Pediatric Psychologist

The Journal of Pediatrics published a study examining rates of emotional and behavioral disorders and overall psychosocial functioning among adolescents with d-transposition of the great arteries (d-TGA).* Findings highlight the importance of monitoring and attending to the psychosocial health of children and adolescents with CHD in addition to their physical health. You can find the complete study here.

About this Study:
  • This study compared adolescents with d-TGA to healthy adolescents with respect to rates of emotional and behavioral disorders and overall psychosocial functioning.
  • The d-TGA group consisted of 139 adolescents who were enrolled in the Boston Circulatory Arrest Study during infancy and have since been assessed at 1, 4, 8, and 16 years of age. Learn more about the Boston Circulatory Arrest Study here. The comparison group consisted of 61 healthy adolescents.
  • Adolescents and their parents completed psychiatric interviews and questionnaires evaluating diagnoses and symptoms of mood, anxiety and disruptive behavior disorders. Parents also reported on symptoms of post-traumatic stress related to raising a child with d-TGA. Stress within the parent-child relationship and cognitive functioning were previously assessed at age 8.
Main Findings:
  • Adolescents with d-TGA had higher rates of attention-deficit/hyperactivity disorder (ADHD) compared with healthy adolescents (16% versus 3%).
  • Rates of mood and anxiety disorders were similar between the two groups based on psychiatric interview. Adolescents with d-TGA reported more symptoms of depression and anxiety on questionnaires, although these were still considered within the “normal” range for their age.
  • Adolescents with impaired cognitive functioning had worse psychosocial functioning.
  • Parental stress, but not severity of illness, was associated with adolescent psychosocial functioning.
    • Parents who reported more stress within the parent-child relationship and more post-traumatic stress symptoms had adolescents with worse psychosocial functioning.
    • Severity of illness (for example, length of hospitalization, subsequent operations, seizures in the hospital) was not associated with adolescent psychosocial functioning
What this Means:
  • The psychosocial health of children and adolescents with CHD should be monitored in addition to physical health.
    • Children and adolescents with CHD are at higher risk for ADHD, as demonstrated in this study as well as many prior studies.1,2
    • Although adolescents with d-TGA did not exhibit higher rates of mood or anxiety disorders, they did report more symptoms of depression and anxiety. These symptoms could potentially increase their risk for a mood or anxiety disorder as they face new challenges during the transition to adulthood.
    • Periodic surveillance, screening and evaluation of emotional and behavioral functioning should be standard of care for children and adolescents with CHD,3 as recommended by the American Heart Association and the American Academy of Pediatrics.
  • It is important for parents of children with CHD to care for themselves and manage their own stress.
    • This study and several prior CHD studies 4,5 have found a relationship between parental stress and child emotional and behavioral functioning.
    • Raising a child with CHD comes with more than its fair share of stress. While it is certainly not easy to prioritize self-care, taking care of yourself is an important aspect of caring for your child and family.

If you have concerns about your child’s emotional or behavioral functioning, we encourage you to discuss these concerns with your child’s healthcare providers.

* DeMaso DR, Labella M, Taylor GA, Forbes PW, Stopp C, Bellinger DC, Rivkin MJ, Wypij D, Newburger JW. Psychiatric disorders and function in adolescents with d-transposition of the great arteries. J Pediatr. 2014;165:760-766.

References:

  1. Shillingford AJ, Glanzman MM, Ittenbach RF, Clancy RR, Gaynor JW, Wernovsky G. Inattention, hyperactivity, and school performance in a population of school-age children with complex congenital heart disease. Pediatrics. 2008;121:e759–e767.
  1. Hövels-Gürich HH, Konrad K, Skorzenski D, Herpertz-Dahlmann B, Messmer BJ, Seghaye MC. Attentional dysfunction in children after corrective cardiac surgery in infancy. Ann Thorac Surg. 2007;83:1425–1430.
  1. Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: Evaluation and management: A scientific statement from the American Heart Association. Circulation. 2012;126:1143-1172.
  1. Visconti KJ, Saudino KJ, Rappaport LA, Newburger JW, Bellinger DC. Influence of parental stress and social support on the behavioral adjustment of children with transposition of the great arteries. J Dev Behav Pediatr. 2002;23:314-321.
  1. Goldberg S, Janus M, Washington J, Simmons RJ, MacLusky I, Fowler RS. Prediction of preschool behavioral problems in healthy and pediatric samples. J Dev Behav Pediatr. 1997;18:304-313.

 

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Dr. Sood is a pediatric psychologist in the Nemours Cardiac Center and Assistant Professor of Pediatrics at Sidney Kimmel Medical College at Thomas Jefferson University. She received her PhD in Clinical Psychology from Temple University and completed residency and fellowship in Pediatric Psychology at Nemours/duPont Hospital for Children. She directs the Nemours Cardiac Learning and Early Development (LEAD) Program and provides psychological consultation and therapy for children with congenital heart disease and their families. Dr. Sood also conducts research on neurodevelopmental outcomes, developmental care and family psychosocial interventions for this patient population. She serves on the editorial board for Clinical Practice in Pediatric Psychology and is an active member of the Society of Pediatric Psychology’s Cardiology Special Interest Group and the Cardiac Neurodevelopmental Outcomes Collaborative. Dr. Sood provides supervision and mentorship to psychology fellows working within the Nemours Cardiac Center to promote psychologist involvement in the field of pediatric cardiology.

Research Matters: The Importance of CHD Surveillance

research matters

For the month of October, PCHA will be focusing on the theme of Research. In the first post of our series, Dr. Matt Oster provides an overview of a recent study that estimates the prevalence of CHD across all age groups in the United States and highlights the importance of surveillance in improving outcomes for CHD across the lifespan.

 

Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010

By Matt Oster, MD, MPH

Congenital heart disease (CHD) is the most common and critical birth defect. Medical research has led to groundbreaking advances in identification and treatment of CHD. While we have learned enough to improve the survival rate to where most babies born with CHD will live to adulthood, there is still so much we don’t know.

Despite how common, critical, and costly CHD is, the understanding of the public health impact of CHD is surprisingly limited. In fact, we cannot accurately answer the basic question of “How many people with CHD are currently living in the U.S.?”

The American Heart Association, in their journal Circulation, recently published a study, Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010, that mapped Canadian CHD statistics onto the U.S. population. The study’s main findings as outlined in the Centers for Disease Control and Prevention (CDC) Key Findings summary included:

  • Approximately 2.4 million people were estimated to be living with a CHD in the United States in 2010. About 1 million of those were children under the age of 18 years and about 1.4 million were adults age 18 years and older.
    • About 12% (289,000 people) were estimated to have a severe CHD.
  • There were slightly more women (1,260,000) than men (1,163,000) living with a CHD in the United States.

However, the authors of the study also draw these additional conclusions –

Our estimates highlight the need for two important efforts:

  • Planning for health services delivery to meet the needs of the growing population of adults with CHD.
  • The development of surveillance data across the lifespan to provide empirical estimates of the prevalence of CHD across all age groups in the US.

First authorized in 2010 by the Congenital Heart Futures Act, the Centers for Disease Control and Prevention and the National Institutes of Health have begun to take steps to address this burden, needing additional resources to continue and expand their efforts. Continued federal investment is necessary to provide rigorous epidemiological and longitudinal public health surveillance and public health research on infants, children, adolescents and adults to better understand CHD at every age, improve outcomes and reduce costs.

Efforts by patient advocacy groups such as the Pediatric Congenital Heart Association are essential to ensure the further development of systems to provide surveillance data to better understand CHD across the lifespan.

Oster_0811_largeDr. Oster is a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta. He holds Emory appointments of Assistant Professor of Pediatrics in the School of Medicine and Assistant Professor of Epidemiology in the School of Public Health as well as an appointment as a medical officer at the CDC’s National Center on Birth Defects and Developmental Disabilities. He earned his MD at the University of Pennsylvania School of Medicine and his MPH in epidemiology at Emory University Rollins School of Public Health. He completed residency training in pediatrics at the University of California-San Francisco and fellowship training in pediatric cardiology at Emory University. When not seeing patients, he serves as director of the Children’s Cardiac Outcomes Research Program at Sibley Heart Center. His primary research interests include the epidemiology of CHD and long-term outcomes for patients with CHD.