One of the Oldest HLHS Survivors – Stephanie’s Story

My name is Stephanie Romer and I am 30 years old. I was born and raised in Dayton, Ohio and born with a fun variety of congenital heart defects, summed up as Complex Congenital Heart Disease. I was born with Hypoplastic Left Heart Syndrome, Transposition of the Great Arteries, ASD, VSD and MVS. I now have Bradycardia and cardiac induced cirrhosis of the liver.

On November 16, 1985, my mother gave birth to what they would have assumed would be a healthy baby. However, life for my father and mother went from a Midwestern dream to extremely traumatizing within 30 seconds. After being told their perfect healthy pregnancy had turned into their daughter being born with a terminal heart condition, their lives got flipped upside down. The doctors had never seen anything like this before and had no idea what to do with me.

I then had three heart surgeries all before the age of four. The initial plan was to keep me alive long enough to receive a heart transplant. At 2.5 years old I was very blue and progressively getting worse. At a routine heart doctor’s appointment, they told my parents I was now in heart failure and they need to act fast. That’s when the doctors began researching other options, and started talking about a new surgery called The Fontan in place of a heart transplant.

My parents spent the next year taking multiple classes and meetings with doctors to teach them about my anatomy and how this procedure would help me. The doctors and surgeons also informed my parents that although there were some surviving Fontan patients with different heart defects, there were no known people with my combination of heart defects and it was unlikely I would survive to adulthood whether I got the Fontan or the Heart Transplant.

On June 26, 1989 I was one of the first babies to undergo the new Fenestrated Fontan procedure that had been done “a few times before” as my surgeon, Dr. Warren Bailey, said. My mother took a year off of work and my father drove back and forth over an hour every single day to go to work and visit me in the hospital. I am now 30 years old and have not had a heart surgery since my Fontan in 1988. I manage my heart and cardiac induced cirrhosis of the liver by monitoring what I put into my body, exercising, and keeping a strong spirit and mind by never neglecting my mental or physical health.

As a child, I played softball and was the best 3rd base player and undefeated pitcher. I worked out almost every day from age ten to fifteen. I was so focused on building my body strength so I could do what I wanted to do while I had a strong body. If I wasn’t doing homework, I was playing football, basketball, skateboarding and rollerblading with my brothers and all the neighborhood boys. If I wasn’t doing that, I was participating in my brothers weightlifting competitions, running on the treadmill, and doing sit ups, push-ups and all kinds of workouts alone in my room at my own pace.

The word determined doesn’t even explain it. I was on a mission to make my body indestructible. I refused to have this “heart problem” and as an adolescent I truly thought I could build my body so strong I wouldn’t have a bad heart anymore. I didn’t understand CHD until mid to late twenties. There was no internet and I had no way of getting to a library. I purely acted on instinct and listened to my intuition. I did struggle with body image issues pretty much my whole life. I was forced to learn and live two lives and it was beyond overwhelming. I just wanted it to end. How I dealt with it was working out and playing sports. I never cared as a teenager if I died trying to be normal – I just wanted to be normal so pushing myself was an understatement. Yet, in retrospect has now saved my life many times over because of strong I made my body. Little did I know, I could not “fix” myself of make my CHD better, this is a lifelong disease with no cure.

How I look at my diagnosis now is really hard to explain because it’s just the only life I know. I obviously never wanted this, I spent fifteen years on a mission to “get rid of it” and learned the harsh truth that I couldn’t. This took a huge toll on my mental health on and off throughout my whole life. I don’t know what it’s like to have the energy of a person with all four chambers of their heart and a steady stream of blood flowing from your feet to your brain and back again continuously without any struggle, sudden pains, shortness of breath, or surprise dizziness. I could never comprehend a life without being a living human experiment.

I am now one of the oldest living people with HLHS in the world and all I want to do is empower people. I believe God made me such a clear, articulate speaker and communicator from an abnormally young age to prepare me to articulate the needs for those who are unable to explain it.

After speaking at an event a few years ago, I was told by a family that they “truly believe you were born to be on the front lines of this battle” and it’s comments like that that keep me empowered to keep fighting. The fire inside of my veins is no longer an independent, adolescent motivation to somehow “cure” my own disease. It is fueled by you – the people who fight next to me, for me and with me in the trenches of a world that wasn’t built for the disabled to survive.

I will never stop investigating and unpeeling the giant CHD onion, and revealing our truths from the mouths of survivors. In turn this will slowly build a map for our doctors and loved ones, who will then teach the next generation more effective and safe ways of living their lives. As a result, together as a community of survivors, doctors, other professionals, and loved ones, we will create multiple medical advancements, resources and tools to continue unpeeling the mysteries of Congenital Heart Disease.As a result, we will finally create a world that is made for the disabled to not only survive but thrive for the first time in history.

And this is just the beginning.