The Feisty Fontan

There are several reasons why I ended up in the hospital: the medical reason, the poop reason and the dumbass reason.

The medical reason may actually be the easiest to explain and maybe even to understand. Thirty-two years ago, I had open heart surgery to correct an congenital heart defect. The specific type of surgery I had is called a Fontan. Fontan’s were considered controversial because of the low survival rate. When I had it in 1986 the procedure had only been performed about 700 times in 20 years and only about 30% had survived. It was a last-ditch effort to keep me alive; with it I stood a chance, without I wasn’t predicted to see many more birthdays.

But I did survive. I even thrived. I had a few hiccups along the way; an arrhythmia there, a pacemaker here, but considering how complex my congenital heart defect was, I was “healthy for a heart kid.” I became a member of the first generation of heart kids to live into adulthood.

Being the first generation of anything, means you’re the guinea pig, you’re the one they are working the kinks out on. The Fontan had some long-term complications that we started to keep an eye out for. But other than adding a liver doctor to my day of bi-yearly appointments, nothing really changed with my health or my life.

Until it did change. Thirty-two years after my Fontan. I developed a Fontan complication called Protein Losing Enteropathy (PLE). PLE can be caused by a variety of health issues. There is still a lot of research trying to determine the link between the Fontan procedure and PLE (again, part of the fun of being part of the first generation of something). Most of the time it develops a few months or years after surgery; it is pretty unusual for it to happen so long after the procedure.

So, why did I get it 32 years later? Well, because if it is a weird, unusual, rare, complex, never before seen medical issue, complication, problem, then I am going to get it. I am a head scratcher to even the best doctors. Every doctors’ visit ends with me being told “You’re pretty unique.”

While having a new diagnosis and an additional medical condition to manage is never fun knowing what’s causing my symptoms and having a plan to alleviate them is actually welcomed news. At this point in my life, I already take more medications than most peoples’ grandparents, so what’s one or two more?