HLHS is something I’ll live with forever. In a way, it makes me who I am, but I don’t let it define me.
Photo by Jaylen Early
The American Heart Association states that congenital heart defects are present in 1 percent of live births. Within that 1 percent, HLHS ranges from 4.8 to 9 percent. I have lived my life with little to no limitations after surviving this rare defect.
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Hypoplastic Left Heart Syndrome, or HLHS, is a congenital heart defect where the left side of the baby’s heart does not form correctly in the womb, affecting normal blood flow to the heart. In a healthy baby, the left ventricle pumps oxygen-rich blood to the rest of the body, but in my case, the left ventricle is underdeveloped and too small and cannot perform properly. The Center for Disease Control states that one in 4,344 babies are born in the United States with this condition and the causes are unknown. Some heart defects are known to be caused by genes related to environmental factors the mother comes into contact with, her medications, or things she eats or drinks, but for this specific defect, the causes are unknown. We’ve rated the factors in our medical history and read article after article, but nothing adds up as to why or how I was born with this. As far as gender occurrence, HLHS is more common in girls and is hardly fatal. Complications of this defect later in life include growth restrictions, formations of blood clots that could lead to a pulmonary embolism or stroke, developmental problems in the brain or nervous system and additional surgeries, or even the need for a transplant. My parents were told I may never walk, would have speech trouble and be delayed in learning, but here I am, extremely high functioning and beating the odds of this congenital defect. Though most pregnant mothers today go through a series of prenatal tests, in 1997 there was no way of diagnosing this defect during pregnancy. There was no way of seeing it. After birth, the baby usually doesn’t show any signs of trouble for the first few days, however, my parents have told me that doctors immediately knew something was wrong. I don’t think I’ve ever asked my parents about this day. It’s a touchy subject and I don’t think they remember much, I’m sure it was mostly a blur. Eventually I’d like to ask them more about the day, maybe when I decide to have children of my own. I was born in at a small hospital in Hannibal, Missouri. It’s the largest hospital that is closest to my house, but they don’t have a Neonatal Intensive Care Unit or any sort of space to treat these types of high risk babies. As the doctors passed the information to my parents, I was airlifted to St. Louis Children’s Hospital, where my dad and other family members followed by car while my mom stayed in Hannibal to recover. I think we have a total of maybe five photos of the day I was born. My mom told me they didn’t want to remember those early anxious moments. I have only seen one photo of myself without the chest scar and I can’t wrap my head around the fact that it’s me. At the time, my mom worked at a smaller hospital, Pike County Memorial Hospital, and her colleagues encouraged her to deliver her baby there, but she refused. She knew the hospital wasn’t big enough and if something were to go wrong, they wouldn’t have the equipment or staff to treat the problem. She has later told me that if I had been delivered there, I wouldn’t have survived. To increase blood flow and make up for the poorly functioning left side of the heart, three surgeries are needed for survival. These surgeries do not cure the patient, but they help restore the heart for a hopeful chance of a normal life. Within the first week of my life, the first surgery, called the Norwood Procedure, was performed. Around 4 months to 6 months old, the Bi-directional Glenn Shunt Procedure was performed, and then at age 3, the final surgery, the Fontan Procedure, was performed. To read more about the procedures themselves, you can click here. As a child, and still today, I have little to no limitations in my everyday life, which is extremely rare in a congenital heart defect like this one. The most common and most obvious factor that I experience, as well as other cardiac patients, is shortness of breath and trouble retaining oxygen. The way I explain it to others, and the way my mom always explained it to me, is that a normal heart has one door through which blood enters the heart and another it uses to exit. My heart only has one door that the blood has to share for entering and exiting. So it takes longer for blood to flow through my heart, and because blood contains oxygen I have poor oxygen flow and lack of oxygen to my heart. This makes physical activity extremely hard, especially cardio. The best way I can describe the feeling is that it’s like an elephant is walking around on my chest while my heart is racing at the same time. Heavy pain, extreme shortness of breath and unable to handle extremely high or low temperatures. Other side effects include a normal irregular heartbeat, the occasional chest pain and irregular beats, and my lips and fingertips often turn blue when I’m really cold. These would never be considered serious or life-threatening unless they become regular or extremely painful. I’ve danced all my life, a heavy cardio-related sport. We are constantly jumping around for minutes at a time and we have to both conserve our breath to last the whole routine and not look tired at the same time. In school, P.E. was never my favorite class because of the running. You could ask me to do just about anything other than run and I’d say yes. That’s how much my body can’t tolerate it. The hardest concept to see is that I’ve never been an out-of-shape athlete, an overweight athlete or an athlete who didn’t eat well or exercise enough, but at the same time I’m always the last student to finish the lap around the track. I used to be hard on myself and think it wasn’t fair that I couldn’t keep up with everyone else. I used to tell myself that I should practice my running and maybe it would get better, but in reality no matter how much I practice, it won’t get better. There were times I remember holding back the tears because I just couldn’t do it and I couldn’t explain to my coaches or teachers the reasons why. Physically, I don’t appear any different, except for the scar down the middle of my chest. The scar runs from my collarbone to about an inch above my belly button, in total it’s about an inch wide, including the holes where it was sewn back together. It’s often called a “zipper” because, well, that’s exactly what it looks like. Up until this past summer, I had never seen a person my age with the exact same scar. In July, I attended Camp Rhythm, a camp for children and young adults with congenital heart defects, hosted by St. Louis Children’s Hospital. KSDK filmed a feature story on Camp Rhythm, you can watch that here. It never really bothered me, as a kid, when people would constantly point to or touch their own chest and ask me, “What happened?” I’d simply explain that when I was a baby, I had three heart surgeries, and then I’d move on. It only really shows in a swimsuit or when I was younger in a dance costume, and I’m so used to it that it doesn’t bother me. When I really sit and think about it, I know I’m lucky to be alive, but I don’t ever want people to think I use it as a crutch. I want people to be informed about this congenital defect because if anything were to go wrong with myself or another patient, people would need to act fast, in order to assure that we would be OK. Some sort of emergency response would have to happen, either an ambulance or helicopter to a St. Louis hospital. I say this to be cautious, because I’d rather be safe than sorry. My grandma calls me her miracle baby and it amazes me and my cardiologist that I’m able to dance as much as I do. Medication-wise, I take a blood thinner, just an 81 mg baby aspirin tablet a few times a week to prevent blood clots. Without the surgeons and doctors at St. Louis Children’s, I wouldn’t be alive today. I attend an annual checkup every summer where they perform both an electrocardiogram (ECG) and an echocardiogram (echo), which are basically sonograms of my heart, just to make sure things are sounding good. Day-to-day life is normal, I know my limitations and do what I can, but at the same time I know I’m beating the odds of this rare CHD. It’s not something that affects my daily life, but I love when I get to talk about it or inform someone on the topic. I think it will forever be a “why” question in my book. Why did it happen? Why to me specifically? I sometimes think I was born with this to inform others, or make a difference in the world. I take any opportunity I can to learn about the defect, educate others and learn about other heart defects. I often tell myself to not take life for granted, and to be thankful for every day. As cliche as it sounds, I am so lucky to be living as high functioning as I am, I often don’t even realize it. I want to encourage you to be grateful for your life, no matter the condition or state that you’re in. You were born the way you are for a reason, for a purpose and to help others any way you can.
Triston Buckley 
Emily Retzer