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.
