The Feisty Fontan

Hello! I’m Liz and welcome to The Feisty Fontan!

I was born with multiple heart defects; the most complex ones being a single ventricle, a single atrium and isolated dextrocardia (only my heart is backwards.) At the age of 6 I had open heart surgery, a modified Fontan, to build some walls and conduits to help with my blood flow and oxygenation. At the time (1986) it was considered a pretty controversial procedure because the survival rates were pretty low.

But I did survive. I had some minor complications over the years. I got my first pacemaker at the age of 9 and am currently on pacemaker #5 (Johnny 5 and a high five to you if you get the reference.) Other than being advised not to run any marathons or carry children (neither of which was surprising news), I didn’t have many limitations. I went on to do all the boring life stuff…graduate college, get married (and divorced), work all the excessive hours that come with a grown up corporate job.

In June of 2018 I was diagnosed with a common Fontan complication, Protein Losing Enteropathy (PLE). While it may be a common complication, it is not common to get it thirty two years later. Developing PLE is a sign that my Fontan may need revised and after my most recent heart cath I was told that my Fontan is “not energy efficient.” So, after all these years and making it this far as one of the few Fontan patients who has NOT needed a revision, my Fontan is failing and causing cardiac cirrhosis in my liver.

Since my PLE diagnosis, I have exited the workforce due to my health issues. I have also met and married the absolute love of life who is steadfast in his support and love for me. Managing a life full of health challenges is made easier with him holding my hand thru it all. I became a full time 24/7 step-mom to two amazing, fascinating and hysterical kids. I also learned that I would need a heart and liver transplant. I was recently approved for listing at Vanderbilt Medical Center in Nashville, TN.

The one thing I have always used to get thru the hospitalizations and the everyday struggles of dealing with a chronic medical condition is humor. Especially the self-deprecating variety. The phrase “If you don’t laugh, you’ll cry” is never more true than when you are dealing with the physical pain of illness. Finding a giggle in the situation is the only way I know how to deal with the medical mayhem that is my life.