“I am a survivor of a very rare disease called portopulmonary hypertension (PoPH). This is a disease classified under Pulmonary Arterial Hypertension (PAH) WHO Group 1. There is currently no cure for PAH. My flavor of the disease involves both the lungs and liver. It is often misdiagnosed as an upper respiratory problem and can continue for years without being detected and correctly diagnosed. When combined with end stage liver disease, which I also had, the condition can deteriorate quickly and become life threatening. My story has a happy ending. Many do not.
“On April 6, 2016, I was admitted to the Intensive Care Unit (ICU) with liver and kidney failure and was told that I might not make it out of the hospital alive. I spent seven days in ICU on IVs for hydration and nutrition. Upon my release, I was told that I had alcoholic cirrhosis and that I would make a full recovery provided I stopped drinking.
“My condition continued to worsen even though I had done everything doctors had told me. Local doctors were not sure what to do for me, so I was referred to two specialists at the University of South Alabama.
“My first appointment with both doctors was February 6, 2017. I met my hepatologist first that day. After some discussion, he told me, in the nicest way possible, that I needed a liver transplant. He also suspected that I might have PoPH and was glad that I was seeing a pulmonary specialist. Later that day, my PAH specialist confirmed that I did have PoPH, but my right heart pressure was too high to qualify me for the liver transplant list. My doctors recommended an aggressive treatment approach to try to lower my pressure so that I could get listed for transplant. I was put on a continuous IV medication called Veletri along with another PAH medication called Adcirca.
“In August 2017, seven months after my diagnosis, a new heart cath showed that my pressure had dropped low enough to get listed. A few weeks later, I was listed for transplant at the University of Alabama Birmingham (UAB).
“Three months later, on November 28, 2017, I received a call from the UAB telling me there was a liver for me. It was a high-risk liver, and I had the option to either take it or wait for another liver. Considering my declining health, I did not think twice about taking this liver.
“I received my life-saving transplant several hours later in the early morning hours of November 29, 2017. I had some complications, and a second surgery was required the day following my transplant. I went into atrial fibrillation and was placed in the Cardiac Care ICU with a heart rate of around 200. I spent the remainder of my stay with UAB PAH specialists taking care of my heart while my transplant team took care of my liver.
“Since my transplant, I’ve had a few complications related mostly to my anti-rejection medications. But, overall, I’m doing fabulous. The fantastic news is that my PAH has begun to reverse which is practically unheard of. I am basically a walking medical miracle.
“None of this would have been possible without the brave, caring person who was an organ donor. She died way too young at 36 years old. Because of her precious gift of life, I’m healthy again and getting to live my second chance at life. Not a lot of people get this opportunity, and I intend to make the most of it!”