The Decision
I have been a patient at the National Institutes of Health, or NIH, (yes, I know Dr. Fauci) for 4 years. At our very first meeting, they told us that bone marrow transplants for patients with severe immune disorders have become an important tool in advancing the life expectancies of these patients. I had known this before but it was the first time a doctor had suggested it might be a viable option for me.
A year later, my brother had been tested and confirmed as a full, 10/10 match and transplant was scheduled for the end of April 2018. But in March, right before we began pre-transplant testing, I read a new study about bone marrow transplants in patients with my disease, STAT1 GOF. It was the only known study of Stat1 and bone marrow transplants at the time. Out of about 20 patients, only 40% survived, and life was hell for them, with patients requiring hospitalizations for months. If I were to transplant, my parents and I would move to DC for 6 months. I'd be inpatient for a lot of that time.
Reading that study put me off the idea, entirely. I asked to postpone the transplant. That was 2018.
And since that day, I have wrestled with this decision--to transplant or not. I met with the bone marrow team, and then the new bone marrow team, and we discussed it endlessly. My therapist has heard about it even more than my failed Tinder dates. I've even asked a magic 8 ball, but no one can truly tell me what I need to know, which is as follows:
How much time do I have left if I don’t do the transplant? Will this transplant save me or will all the chemo and inevitable infection and Graft Vs Host Disease kill me?
The fact is, no one knows. My disease is rare and awful, and the number of patients is scarce. There are only a few clinical studies; my case is written about in almost all of them. (Thanks, NIH!) No one truly knows how long you can live with this disease, but many people die in childhood.
And for me, everything has gotten worse with time, suggesting this disease is aggressive and progressive.
Since 2018, I have been on so many antifungal drugs that I lost count. When the drugs stop working, I get candida in my esophagus. It causes a fungal build up and I can’t swallow or when it's really bad, barely breathe. It’s always incredibly painful and requires hospitalization. Other things usually go wrong--my kidneys, my lungs, my heart fills with fluid. It’s awful, and shitty. The pain is so bad that I have become dependent on Dilaudid when I’m infected. It is an emotional and physical prison I don't wish on anyone.
There are only a few antifungals and oftentimes, the fungus growing inside me becomes resistant to certain drugs. My doctors biopsy the fungus while I’m sedated during an endoscopy. Then they test for resistance. I have built up resistance to almost every single drug.
Right now I’m on two particular antifungals. One requires daily IV infusions at the hospital delivered by people wearing Hazmat suits, which was comforting knowing that they were about to inject that poison into my veins. These drugs, plus a drug for RA, are working together but it is a temporary calm. We have, once again, put the transplant back on the books.
A part of me wonders if I’m courting death and suffering. I recently had gallbladder surgery where it felt like everything went wrong, and I knew I had nothing to do with it. But choosing to do this transplant, instead of waiting for the drugs to fail and for us to have to do it at gunpoint, makes me feel like this pain and suffering will be my choice.
It’ll be my fault that my dog has to live somewhere else. That my parents and I move to DC for 6 months. My fault my mom has to either quit her job or take unpaid leave. It’ll be my fault that the kids don’t see their grandparents, or me, for a very long time. My fault we waste a bunch of money on rent. It'll be all my fucking fault that my brother has to have 27 holes drilled into his hip to exctract the marrow, and my fault he'll spend the night in the hospital and will experience serious physical pain.
My fault if it doesn’t work and I die.
My fault.
Logically I know that this isn’t true; that I was born with this genetic mutation. And everything that happened since, the diseases stacked up, hasn’t been my fault either. I even got the type of diabetes that isn't at all caused by what you eat! But choosing to do this is choosing to suffer.
Or:
Is it choosing hope? Hope in science, and my doctors, and my family and friends and NIH?
Is it choosing to believe that I will survive this and maybe have half a shot at a decent life again as I did in my twenties?
I’m not sure. But I do know making this decision, this decision that is literally life and death, is the most exhausting mental challenge I have ever experienced.
I know only I can make it. And I will just hope and pray that the answer comes to me, that the correct way illuminates its own path, that I never lose another hour of sleep to this decision again.
(Or you can make it for me; just reply to this email.)
Thanks for reading,
kpb