Last month, I had the opportunity to tell my story on the on local public radio (KVPR) of transitioning from pediatric to adult care. The story was picked up nationally on NPR – All Things Considered. I want readers to understand that complex health is not about one disease, not just congenital heart disease (CHD). I have two other life-threatening diseases that are secondary to my heart condition. That means that I need more than a cardiologists specializing in Adult Congenital Heart Disease (ACHD). For that, I go to four different hospitals; Stanford, Lucile Packard Children’s, Valley Children’s Hospital and UCLA Medical Center.
With only CHD, I wouldn’t need four hospitals. Science and technology are allowing us to live longer; however, we need more specialists that understand our underlying conditions. Often they are super-specialists – very rare and no single system has all of them. As heart kids, we are used to going to appointments, for me it was every three months with an echo and EKG. As a heart kid or a parent, you’re so worried about the present, not the future. Once you realize there will be a future, a whole set of different issues arise.
I was born with pulmonary atresia, which affected my lungs, but my lungs did not become a disabling problem until my late teens. Although, I saw a pulmonologist regularly just like the cardiologist. We only started thinking about what will happen and who would treat me as I transitioned when I started puberty. In 1993, I was the youngest to survive with my rare subtype of type of defects and the first stage of my surgeries. I was 9 months old. Today, they can do most surgeries within days of being born and in some cases they can do surgeries while still in utero, which shows how much science and technology has advanced. If science and technology has advanced so much, then is it so difficult congenital patients to transition? The fact is we didn’t live and now we are. The adult medical world is now faced with its first large bolus of us.
There are about 1.1 million pediatric congenital heart patients and 1.3 million adult CHD patients; many of whom have complex diseases and secondary illnesses. In the United States, there are about 2000 pediatric cardiologists and only about 200 boarded adult congenital Cardiologists. This alone demonstrates a huge gap in care. As we get older, we are faced with the same issues as our peers – adult health issues: if we can or should get pregnant, secondary issues, high blood pressure, obesity, diabetes, general conditions of aging, etc. I know that seeing a gynecologist was must, but my cardiologist was clueless on who are where to go. Most adult physicians have very little knowledge about childhood illnesses and vice versa. That is the problem and that is why at Stanford and UCLA I see both adult and pediatric physicians.
My transition remains difficult. When I was a teenager, I started to get sicker and we didn’t know why. As a teenager, I fell into a deep depression that I call the realization depression that I think anyone who grew up with complex disease suffers. Mine really hit when I turned 15 and lasted for two years. Something clicked one day and made me realize that this is reality for me and I will never be healthy. I never thought that I would be a sick adult. I realized my parents can’t fix this and there are responsibilities that my parents handled, that I will have for the rest of my life.
When I was diagnosed with CVID at 18, I was fed up with the pediatric world. CVID is usually diagnosed when you’re older and rarely when you’re still a child. Because of that, it was considered an adult illness. The same goes for my lung disease. Yes, I always had problems with my lungs, but when they found out that I had secondary pulmonary hypertension and COPD because of my physiology, they sent me to an adult pulmonologist. This makes coordinating care with all of my physicians – both pediatric and adult and across multiple hospitals near impossible. It also did not work.
In the fall of 2013, my health seriously deteriorated resulting in multiple ICU admissions less than a month apart and doctors “hot-potatoing me.” It became critical on January 1, 2014, when I was admitted to the PICU at Valley Children’s Hospital, stabilized and then transferred by plane to Stanford. These admissions were after 2-3 admission at UCLA for pulmonary issues – I couldn’t breath. At Valley Children’s they determined my pulmonary pressures exceeded my systemic pressure and I was suffering from steroid toxicity. At Stanford my condition continued to deteriorate and I was being evaluated for an emergent heart-lung transplant.
The entire time I was sick at UCLA, my pulmonologist did not contact my adult congenital cardiologist and I was seen by an adult cardiologist. The communication should have been non stop between my adult congenital cardiologist and my pulmonologist. There was a real disconnect between the coordination of my pediatric care and now with adult care. As written above, it almost killed me.
That hospital stay in 2014, I almost didn’t walk away alive. I was in the CVICU at Stanford for a month and saw doctor after doctor and even at another major teaching hospital, they could not figure out or agree on how to treat my diseases. I still have yet to receive a diagnosis for the 3rd lung condition I have. I am also having trouble coordinating with GI and Immunology for my CVID and chronic C.diff.
Even with my dad being a both a health care attorney / and a senior executive at a children’s healthcare system, a brother-in-law who is a pediatric cardiac intensivist, I still have trouble coordinating my care and timely receiving treatment.
Today, more than ever, we need system for adults with chronic diseases of pediatric origin. We will not survive if we can’t find medical care that has knowledge of our primary conditions.
On July 16, 2017 I will be exactly 16-years since my 4th open heart surgery. Surprisingly, my heart has been the most stable disease. Thank you Drs. Prochazka, Rompf and Hanley.