Hello, friends: I Have Scleroderma
Over the coming months, you’ll see photos and social media posts from me in hospitals and clinics, and wonder what’s going on and why I’m interrupting your aunt’s very important stream of political conspiracy theories. Well, I have a story to tell that should clear some of that up so you can get back to learning about the lizard people who ACTUALLY control the world’s governments. Or something.
Tl;dr: I have scleroderma, which has been bad to my body, and I’m in Chicago undergoing a stem cell transplant to try to reboot my immune system.
Back in August 2015, I was diagnosed with scleroderma, an autoimmune condition in which the body attacks its own tissues. In the more common variants of the disease, the body’s immune system attacks the skin and connective tissue, hence the name of the disease, which is Greek for “hard skin”. (You can learn lots more about the disease at the Scleroderma Foundation website.)
In my fun, sexy flavor of the disease, known as diffuse systemic sclerosis, my body has been attacking my lungs, heart, and digestive tract in addition to my skin. It’s caused inflammation in my lungs (interstitial lung disease), leading to fibrosis that has knocked out half of my lung function. It’s darkened and stiffened my skin slightly. It’s weakened my esophagus, so I can’t do that neat trick where you can eat something while hanging upside down. And now it seems to be stiffening the chambers of my heart, which is…not great. If it had been left untreated, I would have had maybe 3-5 years left, given how rapidly my symptoms had progressed.
Immediately after I was diagnosed, I was referred to the rheumatology team at the Scleroderma Center at Johns Hopkins University Medical Center. I was put on a heavy course of the immune suppressant Cellcept to weaken my immune system to slow or halt the progression of the disease. And it worked for a while.
This past April, I saw a talk by Dr. Richard Burt, an immunologist at Northwestern University, who has been researching a pioneering treatment for autoimmune diseases called the Hematopoietic Stem Cell Transplantation (HSCT) for a variety of autoimmune diseases. The field has been making steady progress for 30 years, especially giving relief to multiple sclerosis patients. And for the past 15 years, he’s been treating Scleroderma patients as part of a long term trial, which has shown some notable improvement in patients’ skin and organ involvement. It’s not a miracle cure, but it’s given a number of people a much better quality of life.
I went to Northwestern in September to see if I might qualify for the trial. The procedure is intense (more on that later), so they don’t take patients who are in the late stages of the illness—it’s definitely not a last resort. During the screening tests (3 days of stabbing, x-ray shooting, and imaging), I learned that my heart tissue was also now showing signs of stiffening. I didn’t have a lot of time to lose if I wanted to go through with the treatment.
The HSCT process
So that brings me to Northwestern University Medical Center in Chicago today. Over the next six weeks, I’ll be going through the HSCT procedure, which will basically wipe out and reinstall my malfunctioning immune system, like you might do with a jacked-up computer. It’s also largely the same procedure as modern bone marrow transplants. Here’s how my journey looks over the next few months:
For a couple weeks after that, I’ll be kept in the hospital for observation until my immune system has effectively re-grown.
From there, I’ll spend a few weeks recuperating with family, and hopefully be back in New York by March. I’ll be working remotely for part of this time, but I promise I’ll totally binge watch that show you told me I HAVE to watch. Definitely top of my list.
Anyway, that’s my story. Your thoughts and/or prayers are very much appreciated. Some of you may be wondering what you can do to help. I’ve actually been fortunate along this journey, in that I’ve had access to great teams of doctors, mountains of information, and a network of friends and family who’ve looked out for me along the way. And also a wife who keeps nagging me to “wash my hands” and “remember my pills” and “not eat that thing off the floor because the five-second rule isn’t a verifiable scientific theory.”
A lot of people don’t have these things, and a cure is a long ways off besides. The Scleroderma Foundation is working to support existing patients and to find a cure in the future. For those who want to help, consider contributing to their efforts here.