The Feisty Fontan

So, I’m realizing that I’m not the most consistent blogger. One would think that sitting at a computer typing would be a nice sedentary activity for someone in heart failure. But with heart failure comes less oxygen and some days my poor under oxygenated brain can barely remember that word for the white stuff that comes in a jug that my step-kids drink faster than we can buy it.  Despite my inability to remember the word “milk,” I did manage to conjure up enough oxygenated brain cells to help my step-kids with their virtual schooling and became the preferred grown up to help them with assignments involving writing. Needless to say, having enough brain power left over to string together words into a coherent sentence and those sentences into a coherent blog post has been a challenge most days.

With that being said, I wanted to provide an update on my transplant journey. As of last week, I have officially been approved for listing for a new heart. By the end of this week, I will most likely be approved for listing for a new liver. And when I move to Nashville on March 1^st, I will officially go on the UNOS list.

It has taken a while to get to this point. I was referred to the advanced heart failure team in April. I had my first appointment in June where I was told that I definitely needed to be evaluated for a heart and liver transplant. After months of consultations, insurance approvals and figuring out the scheduling puzzle, in October I spent a week at Ochsner being poked and prodded in crevices I didn’t even know I had. I had thirty-eight appointments over three days. Between multiple blood draws and IV’s for contrast, both of my arms were covered in black bruises that didn’t fade until Christmas.

And after all that testing, they gave me the answer of no answer. They didn’t approve or deny me. They decided to try a new medication (you may know it by its marketed name of Viagra). They wanted to do another cath. Basically, they wanted to try everything possible to keep me off the list for as long as possible, which I wasn’t mad about. It’s not like I was super eager to be sliced from stem to stern to have two organs switched out.

Plus, over the course of evaluation I learned that I would be only the second combined heart and liver transplant performed there and potentially the very first adult Fontan heart and liver transplant to ever be done there. I wasn’t confident that I really wanted to be the first and I got the feeling they didn’t really want to be the first either. I started thinking that maybe Ochsner, despite providing me with some of the best care I’ve ever received, was not the best place for me to get my transplant and I began to research other centers.

During the months long period it took to get my evaluation testing scheduled, my Adult Congenital Cardiologist left Ochsner to go start another ACHD clinic because she is a badass with a passion for congenital patients. I was devastated when she left; we had a great rapport, and she saved my life by diagnosing my PLE. I was not thrilled about getting a new Adult Congenital Cardiologist right before my transplant evaluation.

Of course, my new doctor ended up being awesome, like every doctor I’ve ever had at Ochsner. And my new doctor happened to be basically BFF’s with a doctor at Vanderbilt that specializes in heart transplants in congenital patients, especially Fontans. And my new doctor was very honest and direct and told me that Vanderbilt was really my best hope and probably my only hope.

At the end of January, I had two appointments scheduled: one with the Dr. Rockstar heart transplant doctor and one with an adult congenital cardiologist. My husband I headed to Nashville with the expectation that we would meet with these doctors, have a conversation, have them agree that a heart and liver transplant was in my future and that we would probably be back in the next few weeks/months for more tests and appointments.

My first appointment began with talking to two Nurse Practitioners who knew my medical history better than I did. Then the doctor came in and we had talked about one of my test results from Ochsner that was overly concerning. A test result that produces a lower number in regular heart failure patients, so the heart failure team at Ochsner wasn’t concerned because they’ve seen worse. A test result that in Fontan patients was downright abysmal. The team at Vanderbilt talked about how important it is to get the timing right because they want to transplant us “before we fall over the cliff.” This test result meant I was getting close to the edge of the cliff. The appointment concluded with these two super nurses jumping into action to get their transplant evaluation testing done in the next three days.

One of the first tests was to draw another like 40 vials of blood. In my head I was wondering what blood tests they could possibly be drawing that Ochsner didn’t. I had been tested for every virus, antibody, and illegal substance known to man. They drew levels of every possible substance floating around my body. Or so I thought.

Amongst the many vials of blood, they ran two tests that I have never had checked before. They checked for two proteins that are biomarkers for inflammation. Biomarkers that increase in congenital related heart failure. On one of them, the really high level that is like super bad is 10; my levels were in the 50’s. The other one sucked too. I totally believe these two blood tests were the game changer. These results meant that Vanderbilt “needed to have a greater sense of urgency in getting me prepped for listing,” as my transplant coordinator informed me. All the traditional biomarkers for regular heart failure on me were normal; another challenge I had with getting listed at Ochsner. The heart failure committee was using regular heart failure metrics, metrics I would never meet, to decide if I needed a transplant. An anatomically defective heart fails differently than an anatomically correct heart does.

So, eight days after my first Vanderbilt appointment I was approved for listing. I was told I needed to relocate to Nashville ASAP for close monitoring and to work with their nutritionist to correct some of the lingering deficiencies that my PLE has left me with. We agreed I will be there March 1^st.

I want to make it clear that I am in no way disappointed in Ochsner. I’ve had amazing care there. They are the #1 liver transplant center in the country. Their heart failure team just doesn’t have the congenital and Fontan related heart failure knowledge. It’s not like 100 of patients like me walk into their office every year.  Vanderbilt is the #1 heart transplant center in the world, and they do get 100 patients like me thru their doors in a year. As one of my favorite doctors at Ochsner said to me, “Your needs have outgrown our level of expertise and that is OK.”

While the change in pace threw me off guard and I was hoping to be able to delay transplant a little longer, I am glad to finally be making progress on getting my life and my health back. It’s a risky surgery, but so was my Fontan. I have exceeded the odds my whole life and plan to keep doing so. I have a husband to grow old with, step-kids to watch grow up, a niece who thinks God saved the other half of my heart to make hers, and two young nephews that need their fun Aunt Boo Boo Heart to chase them around. And I have a lot more of my story I want to tell. Heck, with all the time I have on my hands sitting in Nashville waiting on the most important call of my life, you might be reading a little bit more of my story on a more consistent basis.