Everyone always jokes with doctors, “What happens if you get sick?” Some of us laugh off the question. The busy schedule of health care often means our own health comes last. However, what happens when it’s a chronic disease that requires treatment? Then the answer to this question is a little different.
I am a family medicine physician dealing with congenital heart disease (CHD). Saying that someone has congenital heart disease is a broad statement. CHD affects 40,000 babies born each year, but the severity and complications have a broad range. Some infants may only have a small hole between the two ventricles; other infants can have entire parts of their heart missing.
I happen to fall into the second category. I was born with right ventricular hypoplasia, which means my right ventricle is just a small flap unable to pump blood adequately. The diagnosis of CHD for me meant multiple surgeries in my infancy followed by a lifetime of “being careful.”
Realistically, I’m lucky. I’m blessed with wonderful parents who happen to be doctors. Their comprehensive care of me as a child taught me how to take care of myself. They wanted me to have as normal of a life as I possibly could. With their focus on how to live with congenital heart disease, I was able to complete medical school, get married, and own my own home. This has made it easy to ignore my ominous heart condition; my life is, for the most part, is happy.
As a child, the seriousness of my condition almost never bothered me. My parents refrained from explaining the depth of my condition to me until I was a teenager, and even then, I barely understood it. The most annoying thing to me was that I couldn’t participate freely in gym class or go on roller coasters with my friends.
As I got older new questions arose. How much alcohol was I allowed to have? How much heat was too much? Nobody knew the answer, but the cardiologist always said: “Be careful.” For the most part, these small things never interfered in my life.
Until the bad days.
In 2014, a young family member with CHD just dropped dead. I say it plainly because that’s exactly how it happened. The night before, he was totally fine, but in the morning, he was found in the bathroom, dead. Unfortunately, cause of death can be difficult to determine in cases of CHD: maybe he wasn’t taking his medication, maybe he overdid it with exercise or alcohol. Research on congenital heart disorders is still determining if sudden mortality is truly preventable or if these guidelines are simply band-aids meant to help us feel better.
For me, being in the doctor’s seat and the patient’s seat can be unsettling. I don’t pretend to be an expert; I am not a cardiologist. However, I know enough about the research to know that studies have only a small number of participants. Why, you ask? There just aren’t that many of us out there. Only recently have doctors been able to do surgeries to “fix” the condition. Do these surgeries hold out in the long term? We honestly don’t know. The oldest individuals who have received these surgeries are only about fifty.
As a matter of fact, due to the complexity of this disease, physicians who see patients with congenital heart disease regularly have to complete special fellowships in congenital issues. Only recently has “adult congenital heart disease” become something physicians even try to understand, because all of us congenital heart disease kids just became adults! Before us, kids born with these diseases just died. Today, physicians have to grapple with the unique patients who underwent experimental surgeries over thirty years ago.
As patients and as doctors, we have even more to deal with in terms of congenital heart disease and adulthood. What if these patients want a family? Can their hearts deal with the extra volume load pregnancy places on a woman’s body? Being both the patient and the doctor puts you at a strange impasse. Do I do what I want because I think my body can handle it or do I err on the side of caution because that’s what I would tell my patients to do? Truly, the answer is nonexistent.
At times, my husband will hear a story about someone with CHD dropping dead. I can see on his face that it scares him, but he will say “Well we don’t know if that person was really taking care of themselves.” It’s true, we don’t know, but we also don’t know if any of those wellness guidelines are going to make the difference between my own life and death. That mortality is, for the most part, too much to bear. When my husband becomes worried, I want to comfort him, but my doctor side thinks that I should be preparing him. What kind of wife and doctor would I be if I didn’t tell him that I too could drop dead out of nowhere?
Part of the problem is that those of us with CHD are everywhere, but it’s hard for us to find each other. Sure, I have support from my family and very close friends, but when I start talking about the grim possibilities, everyone’s eyes glaze over. It feels as if they don’t want to deal with the uncertainty I deal with every day. I don’t want to be the one to bring everyone down, so most of the time, I ignore it and move on.
Individuals with CHD look like everyone else, so it’s hard to see us as different. I write this as a doctor, but also as a patient, to freely express my feelings and to remind others that if you suffer from congenital heart disease, you aren’t alone. It is my hope that the coming decades will bring more attention that will quiet the difficult questions of mortality in our different but beating hearts.
Sweta Shah is a family physician.
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