Christy Sillman is the nurse coordinator for the Adult Congenital Heart Program at Stanford, a joint program with Lucile Packard Children’s Hospital Stanford and Stanford University Medical Center. Christy was born with a type of congenital heart disease known as tetralogy of Fallot with pulmonary atresia, and she works to bring awareness to the lifelong care needs for all congenital heart disease patients.
If you haven’t seen Jimmy Kimmel’s gut wrenching monologue where he told the story of his son’s birth, surprise congenital heart disease diagnosis, and first open heart surgery, you can watch it here: https://youtu.be/MmWWoMcGmo0
Thank you, Jimmy Kimmel! My entire life I’ve struggled to explain to people what my congenital heart disease (CHD) is and tell them that, no, I didn’t have a heart attack at the tender age of 1 day old. It is now much easier to say to non-medical people, and sometimes even medical people, “I have what Jimmy Kimmel’s son has.”
The awareness you’ve brought to the indiscernible nature of CHD — that it can truly happen to anyone regardless of color, class or creed — has been invaluable. CHD is the most prevalent birth defect, affecting 1 in every 100 babies born. When I was born in 1980, the outcomes were not good for children born with CHD. They have vastly improved since then, and today more than 90 percent of CHD patients now live into adulthood. We’ve done such a great job saving the lives of those born with CHD that we now have more adults than children living with CHD. This is a true testament to the advances in surgical techniques and medical therapies used to treat this disease.
Yet, despite the many advances we’ve made, there continues to be one major misunderstanding about CHD that persists today, to the detriment of many: that CHD can be cured or fixed through surgery.
I, along with many of my patients, have been told this myth multiple times throughout my life, and I still occasionally encounter both laypeople and medical professionals who believe it. How can the heart, an organ that relies on efficient movement for function, be “normal” after enduring incisions, sutures, devices and hardware?
Once you cut through a heart, it will never pump normally, even if the blood flows in a normal pattern.
Many of the surgical techniques that have been developed for moderately or highly complex CHD cases are “out of the box” and take strange and twisted routes through the heart. These surgical techniques are palliative in nature, and we’re now discovering that our human-made approaches don’t necessarily cooperate well with the rest of the body’s organs.
Even CHD patients who haven’t had surgery experience long-term consequences from the abnormal blood flow and heart pressures caused by CHD-related defects. Some of my sickest patients have the simplest of defects.
The truth is that all CHD patients need lifelong care with specialized providers, and more than half of them will require other interventions later in life. Adult congenital cardiology, the newest medical specialty in 30 years, focuses on advanced cardiology training in the unique long-term needs of CHD patients.
The Adult Congenital Heart Association’s (ACHA) accreditation for adult congenital heart disease (ACHD) programs is changing the landscape of ACHD care in the United States by ensuring care centers have the resources and provisions necessary to provide high-quality, appropriate care. That means patients like me will have a much easier time identifying facilities where we can receive care.
The Adult Congenital Heart Disease Program at Stanford was one of the first programs in the nation to become accredited as a comprehensive care center by the ACHA. Despite the national recognition of our program, we still have patients who are referred to us or who arrive in our emergency department after detrimental lapses in care. Some of them have gone as long as 30 or 40 years without any cardiology care or have received inappropriate care with cardiologists who are untrained in treating CHD.
Even I had a decade-long lapse in care that lasted until I was seven months pregnant with my son and going into heart failure. My journey back to CHD care and my introduction to the field of adult congenital cardiology was extremely stressful and scary, but it ultimately helped me discover my passion in life — to ensure that other CHD patients will not “fall through the cracks” or experience lapses in CHD care.
Sadly, these lapses in care can have disastrous consequences for CHD patients. By the time some of them return to care, they may have irreversible damage that could have been prevented by earlier intervention and medical management.
The great news is that many adults who receive appropriate care for their CHD lead amazing lives. I’ve enjoyed seeing my patients start a range of careers, including as lawyers, medical professionals and even professional athletes. I’ve also gotten to help our patients start families of their own. Their perseverance is often awe-inspiring, as they’ve overcome unbelievable odds and have proven to the world that they will thrive in the face of adversity.
Jimmy Kimmel’s son Billy has already become a beacon of hope for other families whose infants have been diagnosed with CHD. My hope for Billy Kimmel and all babies born with CHD is that they will have long, enjoyable lives full of astonishing adventures and joyous triumphs.
But we must refute the myth that open heart surgery cures congenital heart disease and make sure that Billy Kimmel and all congenital heart disease patients out there receive high-quality congenital cardiology care throughout their entire lives.
Authors
- Christy Sillman
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- Stanford Medicine Childrens Health
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Fantastic article on such an important topic!
Hi Megan. Thank you for your comment! We’re so pleased Christy was willing to share her story about congenital heart disease with the Stanford Children’s Health community.
I am Christy’s dad and remember the night she was born like it was yesterday. She was transferred by a critical care ambulance from Kaiser hospital in San Diego to UCSD San Diego for a catherization. After 2 hours of anxious waiting, the cardiologists came back in the conference room and explained, mostly with flow diagrams, what Tetralogy of Fallot with pulmonary atresia was, and how it would be a long, hard road and my wife, Susan, and I could just opt to leave Christy on comfort care and let nature take its course. No way I told him would we do that with our beatiful little girl, so we left it in God’s hands and the rest is history.
Hi Dr. Car. A dedication to healthcare must run in the family! Thank you for sharing this part of Christy’s experience with us. We are inspired by her story and her efforts to bring awareness to the lifelong care needs for all congenital heart disease patients.
I am also an adult w congenital heart disease, working in a PICU that is a huge centre for cardiac surgery. I was appalled when I was pregnant at the misinformation amongst medical staff treating me, and the level of panic held about how I would respond to the changes- particularly after birth. The fights I had with the anaesthetic staff trying explain why I needed my arterial line in my right arm! I hope for all our sakes that the expertise catches up to us all growing older!
Nothing gives parents of children with CHD more hope than hearing about adults with CHD who are leading full and happy lives. Thank you for sharing this story, and thank you for letting people know babies with CHD will require lifelong care. What an inspiration and comfort Christy must be for her patients.
My son was born with TAPVR and is doing beautifully. I’m so grateful to see how much the CHD community has grown since he was born. We are all in this together!
My daughter was born with the same as Jimmy Kimmels son. She is now going to be 26 in December and is having her aortic valve replaced with a mechanical valve and also having a pigs valve put in as she was born with no pulmonary valve. These procedures will be done at the end of November in Philadelphia. She has oboma care with a high deductible. She will be out of work for at least 2 months. And their is nothing she can collect as a income during that time in the state of Pa. Her and her boyfriend recently bought a home. I don’t understand how they expect at such young ages to have a large bank account to cover all these bills while being out of work and not being able to collect anything. I can only imagine the cost of her insurance after the end of this year as she will no longer be on our insurance thru oboma care As with having our own business and to get insurance was impossible due to her defect and other issues with my husband. And yet she will as with others need follow up Heath care the rest of her life. In the end if we had to sell our home to get her the care she needs that is what we would do. A home we could rebuild a child you cannot replace.