year 5

I read an article recently highlighting the connection between hypoglycaemia and dysautonomia. Or more accurately how POTS can cause reactive hypoglycaemic episodes, particularly following high doses of carbs.

Reading the article, filled with research and insight from doctors, felt like a punch to the gut.

See, for so long I convinced myself I’d failed GSD. The majority of patients with glycogen storage disease do not need or receive liver transplants. When I was listed for transplant in 2018, the idea of a transplant for quality of life was newly on the table. To this day, I can count the amount of people who have been successfully transplanted for GSD on my fingers.

There was a large portion of time, and there is still but I am less aware of it now, where the discussion of transplantation as an acceptable form of treatment for GSD was highly debated. To the point I remember, when I first vocalized I was on the transplant waiting list, I was told by others on the other side of the debate that I was being selfish, that I should try harder to manage my disease through other avenues, that I’d failed my treatments and a number of other things I won’t repeat on this corner of the internet.

I internalized every word. To the point where even when I was in that OR on the table, ready to receive my new liver, with my brother already cut open in the OR beside mine, I wholeheartedly believed this was a choice I was making, the same way someone chooses what socks to wear in the morning. I believed, even with all of the pain and complications that my transplant surgery ended up involving, that I was being selfish. And when my brother had complications that threatened his life, I internalized the belief that this was my fault too.

So reading the article linking POTS and hypoglycaemic episodes felt like the ground gave out beneath my feet because it meant it wasn’t my fault.

I’ve been in therapy long enough to know that that isn’t what happens. No doctor decides to give someone an organ transplant for fun. And yet some part of my psyche didn’t believe it. I believed I deserved whatever pain or complications or misfortune I got because I chose this. I chose to be selfish and not try hard enough.

These were the same thoughts I had during pregnancy, when I believed that if I tried harder, if I’d been a better mother, I could have saved my son. Looking back at the medical reports, I know objectively this wasn’t true, but it was a belief I internalized and held for a long time.

It reminded me of a conversation I had once with a fellow TFMR mom who terminated for Down syndrome. In and of itself, Down syndrome isn’t a fatal condition. And, in listing all of the comorbidities of this diagnosis that her son presented with, but excluding the overarching umbrella of the Down syndrome diagnosis, it made absolute sense why she “chose” to terminate. We use choice in quotations because choosing implies there are decent options, and more often than not in the genetic lottery of life there are none.

I had no good options. My choice wasn’t a choice. And while GSD in and of itself may not always be life limiting, or life threatening, in my case it was. Most people, I learned, who are adamantly against transplant in the case of GSD, have far milder forms of the disease and less comorbidities. I wouldn’t have gotten better, and without transplant, even if I were still alive today I would have been a shell of myself.

The treatment for glycogen storage disease involved high dose carbohydrate dosages in the form of cornstarch. What we didn’t know at the time, and has only recently been documented in medical literature, was that this would have made my condition worse as my GSD was coupled with POTS. What they were trying in an effort to stabilize me was quite literally making me sicker. And it wouldn’t have gotten better. I couldn’t have tried harder. I didn’t fail.

I’m now 5 years post transplant, which feels like a wild thing to say. When I was transplanted initially, I wanted 1 good year. A singular year, and I wouldn’t be selfish and ask for more. 5 years out and I’m healthier than I’ve ever been, and there’s no reason to think I won’t live a good, long time. I get to live my dream every day working with individuals walking their own roads of chronic illness and organ transplantation. I get to tell them that you don’t have to pay a price for being alive. You don’t owe anyone anything. You do not have to walk on your knees for a hundred miles through the desert, repenting.

You get to let the wild animal of your body love what it loves.

You get to live life on your terms.

5 years later and life is beyond my wildest dreams. I am grateful.

I didn’t choose my past. but I do get to choose my future.