The Feisty Fontan

This folder is a scary folder. I know that it looks pretty plain and inconspicuous. It didn’t try to bite me or cut my hand off or anything. But I’m going to need you to trust for me a minute when I tell you that getting this folder was panic inducing.

I had my first appointment with the Cardiac Transplant team this week. When I booked the appointment, and the corresponding day full of tests, I was told that I would meet with the transplant doctor and, if warranted, a transplant coordinator nurse would come in to talk about the next steps in the process. The “if warranted” part meaning that I was ready to start the transplant evaluation process.

When my regular Adult Congenital Cardiologist and I talked about referring her me to them, we talked about how it may be a little early but that research shows that Fontans that start the process early tend to have better outcomes. Regardless, it was time to bring them into my care team and get their opinion on long term treatment.

I went into my appointment thinking it was going to be nice “get to know each other” conversation and we would talk about when in the future we would start the evaluation process. I was sure I would be told I was headed down the transplant road…. maybe in the near future, but still IN THE FUTURE.

Now, why did I think this? I thought this because I am a big dumb dumb head. The same big foolish nitwit who came close to dying from Protein Losing Enteropathy because I didn’t want to collect my poop. The same big doo-doo brain who stands in front the of the washer and dryer trying to catch my breath because moving wet clothes into the dryer is now a strenuous physical activity. The same big nincompoop who is huffing and puffing from doing laundry because she’s walking around the house with an oxygen saturation of 81, yet not wearing her oxygen.

If you’ve read my blog before, you may have figured out that my stubbornness and my denial tendencies are two of my most endearing qualities.

My appointment did not go the way I had imagined it. The doctor came in, she had already talked to my other doctor, read thru all my medical records, looked at the images from my cardiac cath and she was in full agreement that it was the appropriate time to start the heart transplant evaluation process. Oh, and probably looking at getting me a shiny new liver while they are at it.

Out the door she went and in came the nurse…. carrying THE FOLDER. Now, the folder itself is pretty benign, but the contents of the folder are what make it scary. Inside this folder is a hefty stack of paperwork….lots of consent forms, information about high risk donors,  documents about having an advanced directive. Getting this folder is the firing of the starting pistol that begins my race for new organs. Reading thru this folder is when it hit me upside my dense and stubborn head that this is really happening.

There are still some serious hurdles to figure out. Just because my level of heart failure makes me a candidate, doesn’t mean that something else could eliminate me as a candidate. I have isolated dextrocardia, meaning my heart is the only organ in my body that is backwards. I also have a modified version of the Fontan surgery, meaning that the way everything is hooked up and routed to my heart is unique to me. In layman’s terms, the arteries and veins around my heart are about as organized as a pile of broken spaghetti noodles.  While I was getting the scary folder from the nurse, the doctor was working on getting me scheduled for a same day CT scan. The first step before any other tests is having a cardio-thoracic surgeon look at my pile of spaghetti noodles and see if a normal heart can be made to fit inside the crazy puzzle that is my chest cavity.

Despite my strong denial abilities, I’ve known for a long time that the big scary heart stuff was coming. Heck, I’ve been telling my now husband this for while and encouraging him to run before it started. All I can do is read thru my scary folder until it stops being scary and move on to the next scary part of my health journey. For the last few years I’ve jumped from one scary health thing to another and despite my abysmally low oxygen saturation, I still have a lot of jumping left in me.