The Connection Between My Congenital Heart Defect and Tummy Troubles

Being born with a congenital heart defect, I’ve spent my life consciously aware of every heartbeat. I know when a fast heart rate is just from exertion and when its tachycardia in need of medical attention. I am familiar with all the other cardiac symptoms; shortness of breath, chest pain and dizziness that appear on a regular basis. I am expert on my condition and the myriad of specific symptoms my body gets. I can tell the difference between just having a bad day and needing to go the emergency room.

As I entered my late 30s, I started to experience some of the physical quirks of adulthood; the need to hibernate after a long week a work, a severe headache if I didn’t get my morning caffeine fix, heartburn after spicy Thai food and a whole range of symptoms that could be politely described as “tummy troubles.”

The last one, tummy troubles, begin to plague me on a regular basis. It seemed like no matter what I ate it caused me intense pain and quickly exited my body in a fit of diarrhea. I alternated between spells of nausea so severe that the mere thought of food made me gag and being so ravenously hungry that I wasn’t full no matter what I ate. As a working professional with a stressful and demanding job, I wrote it off as stress and getting older.

I tried ways to self-treat my unpleasant digestive related symptoms; I ate more yogurt, took a probiotic, and drank kombucha. I tracked what I ate in the hopes of identifying a trigger. At times I thought my efforts were working and I would have some relief until my symptoms would magically re-appear more intense than they were before.

Over the years I had heard about Protein Losing Enteropathy or PLE, a complication that could arise from the Fontan surgery I had as a kid. The adult congenital cardiologist I saw for over a decade once casually mentioned that since I had my Fontan over 20 years ago if I didn’t have PLE by now, then I would probably never get it. He thought that maybe I had irritable bowel syndrome. When I started to see a gastroenterologist for routine liver monitoring, I mentioned the symptoms to him, and he agreed that IBS was a possibility but didn’t recommend further testing.

In late 2016, I moved from the Midwest to the Florida panhandle. I had to transfer my care to an adult congenital heart clinic that was closest to my new home and chose a place in New Orleans. On my first visit with my new adult congenital cardiologist, she asked me if I had ever had my stool tested and sent me home with a stool collection kit. I recently started a new job that required long hours and frequent travel, so taking the time to collect a stool sample and take it to a local lab was the last thing on my mind. Besides, my tummy troubles were calm at that point and I accepted the issues as just the way my digestive tract functioned. And, to be honest, the idea totally grossed me out.

In early 2018, I started to experience an overall general “blah” feeling and a weird combination of symptoms; my feet were swelling, my weight kept fluctuating, I seemed to be losing more hair in the shower. Plus, no matter how much I slept I was exhausted all the time and getting out of bed in the morning seemed to be getting harder and harder. I was still working my demanding job and was having serious marital issues, so once again, I wrote it off as stress. I did take the time to schedule an appointment with my primary care doctor to see if there was anything he could do to help me manage my symptoms until my next cardiologist appointment.

Shortly after my appointment, my doctor’s nurse called me with a slight panic in her voice. My routine blood work showed that I was anemic, severely vitamin D deficient and that a protein in my blood, albumin, was much lower that it should me. The nurse actually asked me if I had an eating disorder. I was prescribed iron and vitamin D supplements and told to eat more protein.

Looking back, I think I knew something significant was going on with me. My feet and legs got more swollen by the day. But going to my cardiologist was an all-day ordeal for me because my adult congenital cardiologist was three hours away and the company I was working for was going through a pretty serious crisis so I was worried that my boss would flip out if I asked for a day off work. I had a cardiologist appointment scheduled for a few months down the road; I kept thinking that I just needed to make it to that appointment, get my meds tweaked and I would be fine.

Two weeks before that appointment, in June 2018, I passed out in the shower. My then husband took me to the local ER, where I was admitted for heart failure. As soon as they got me stable, they arranged for me to be transferred to the adult congenital heart clinic.

When I arrived there, they immediately began working on pumping me full of diuretics to reduce the amount of fluid I was holding. When my doctor came around to see me, she asked me again for a stool sample. She was almost positive I had Protein Losing Enteropathy.

Her hunch was right. Thirty plus years after my Fontan I had developed PLE.

In laymen’s terms, protein losing enteropathy causes proteins that should be in your blood and carried through your body to leak into your digestive tract. Every single symptom I had experienced over the previous six months were caused by PLE. All my tummy troubles; the pain, the diarrhea, the nausea, are all typical PLE symptoms. When my PLE was left untreated for so long, it caused me to become nutritionally deficient, which explained the hair loss and my blood test results. Even the fluid retention and swollen feet, which are classic heart failure signs, were a direct result of my new GI disorder. Low albumin causes swelling.

After 10 total days in the hospital and massive amounts of Lasix, I was pounds of fluid lighter. I was prescribed a corticosteroid and instructed to follow a high protein, high medium chain triglycerides, low fat, reduced sodium diet.

On my first post hospital visit, my cardiologist told me she thought I probably had PLE for years. I looked back over my years of tummy troubles, and armed with my new knowledge of PLE, concluded that she was probably right. Never in a million years did I think that a cardiologist would be the one to solve my tummy troubles.

The worse part of this whole story was that I could have spared myself six months of misery and an expensive hospital if I had just done the stool sample test when she asked me to. It could have been diagnosed and treated well before it made me as deathly ill as it did. Being too busy and too grossed out to collect my own poop almost killed me.

Follow this journey on The Feisty Fontan.