Marla Jan DeFusco recounts her experience growing up with tetralogy of Fallot and how the passion of the nurses at The Children's Hospital of Philadelphia inspired her own career in nursing.
I remember looking back and waving to my parents as I was being wheeled into the operating room for surgery to correct a congenital heart defect called tetralogy of Fallot. I had my favorite childhood doll by my side and was the picture of bravery.
No tears for me, this was old hat! So what finally caused me to throw a fit? The anesthesiologist told me he would have to take off my pink nail polish! To calm me down, he promised he would buy me a new bottle.
Growing up with a heart defect was just a part of my life; I didn’t know any different.
I was used to the trips to The Children’s Hospital of Philadelphia, taking medications and being smaller than my classmates. I grew tired quickly in gym class (when I could participate at all) and I constantly had to explain how I got my scar.
While I couldn’t play competitive sports (things are much different today), I excelled in music. I participated in school and community theater, high school shows and choir concerts, and the All South Jersey and All State New Jersey choirs.
A trip through memory lane
My mother Olga remembers way before choirs and theater to the days following my birth. The doctors in Connecticut, where I was born, told my parents I had a ventricular septal defect, a hole in the tissue separating the two sides of the heart. They said it was small and already starting to spontaneously close. When my father’s job relocated us to Philadelphia, our pediatrician referred us to CHOP.
In September 1981 my mom brought me to meet with my first CHOP cardiologist, the late Sidney Friedman, MD. He discussed tetralogy of Fallot that afternoon, and after an echocardiogram, I was admitted to the hospital. A cardiac catheterization the next day confirmed the diagnosis.
My mom recalls going from what she was told was a minor heart murmur to a serious, life-threatening condition.
“I thought I was going to lose you,” she tells me. “Everything changed.”
Open heart surgery
In February of 1982, when I was 6 months of age, I had my first open heart surgery, a Blalock-Taussig shunt, performed by cardiothoracic surgeon Larry Stephenson, MD.
Over the next five years, I was on multiple cardiac medications, had numerous catheterizations and three more heart surgeries, all performed by William Norwood, MD.
I remember so much about my last surgery, from the breathing tube down my nose, tubes in my chest, and IV and lines in my arm. I remember that first painful time getting out of bed, and it getting easier each day.
I would hang out at the nurses’ station with my favorite nurse, Maria. She stayed with me when my mom needed to step out, she drew hearts and flowers on my bedpans, and she played the biggest role in making sure I got better.
Transitioning to adult care
Over the years following the last surgery, my visits to CHOP grew less frequent. At 16, my cardiologist explained the anatomy of tetralogy telling me that I needed to understand my defect and start taking responsibility for my own medical care. He also explained that the transition to adult care is vital in congenital heart disease because patients’ cardiac problems differ from those that adults develop as they age.
He facilitated my smooth transition to the Philadelphia Adult Congenital Heart Center, a program that unites the resources of CHOP and the University of Pennsylvania Health System. Richard Donner, MD, is now my cardiologist, and I couldn’t be happier with my care. I truly believe I am in the best hands.
Beating the odds
Was it always easy? No, of course not. Though I usually took everything in stride, I was still a kid, and didn’t like being seen as different from my peers.
As a young child, I hated wearing a Holter monitor, (in the 80s they were 5x7 inches, today, smaller than my cell phone!) and the only way my parents could keep it on me was by calling it my “Michael Jackson Tape Recorder.”
I went through a phase where I disliked my Medic-Alert bracelet. I hid it wherever I could, thinking my mom would never find it, but she always did. Even though I knew the answer, I begged my mom to let me play soccer or be on the swim team, and would make her feel guilty when she said no. (Sorry Mom!)
As a kid, I was a proud member of the “Zipper Club,” but as an adolescent, I did everything I could to cover my scar. I’m sure I drove my poor mom crazy searching for bathing suits that kept it covered (love you, Mom!).
Over time, I stopped trying to hide it, and eventually learned to embrace my scar. It’s my battle wound, a symbol of the odds I beat, and what I survived.
There were definitely some struggles throughout young adulthood, and like everything else, I was able to overcome them. I lived away from home, had the college experience and graduated magna cum laude.
I am now 29 years old and happily married. While there is the possibility of future heart surgeries, I take each day as it comes. I’ve been a CHOP nurse for almost six years, and in 2010 I joined the nursing team in the Cardiac Intensive Care Unit.
Sharing my experience
I feel my experiences make me a better nurse. I never bring up what I went through, unless a patient or parent notices my scar and asks.
I was recently taking care of a patient, who, a few days prior, underwent heart surgery himself. He noticed my scar, and asked if I also had open heart surgery. After I said yes, I was no longer just his nurse, but someone who personally knew what he was going through.
When I said that he needed to start taking deep breaths, (who really wants to take deep breaths after heart surgery?) and that it really does get easier, he listened. He asked me lots of questions about what I had gone through, and when I tried to steer him away from talking about me, he said he loved hearing what I had to say and it helped calm him down.
When I left for the day, he gave me a high-five. Though my own experiences help me relate to the patients I’m always careful to never take away from what they are going through.
There is no better feeling than giving back to the place that saved my life. It is the care from the nurses at CHOP that truly stands out clearly in my memory and inspired my own career in nursing.
I knew before I started nursing school I was meant to work at CHOP. Where else would the nurses draw flowers on your bedpans and the anesthesiologist keep his promise and a few days after he made you cry before your surgery, bring you a brand new bottle of pink nail polish?
By Marla Jan DeFusco, February 2011