How I Make Pompe Disease Treatment Days Fun
Columnist Dwayne Wilson brings humor to the long days of receiving infusions
After receiving my diagnosis of late-onset Pompe disease, it felt like a weight had been lifted off my shoulders. Finding out that I had a rare disease with a treatment was amazing. At the time, I don’t think I comprehended the fact that those treatments would last forever.
There is no cure for Pompe disease. My body has two gene mutations that cause a reduction in the production of the GAA enzyme, acid alpha-glucosidase. Its role is to break down and process glycogen stored in the muscle cells. When glycogen builds to toxic levels, it starts to break down the lysosomes and burst the muscle cells, resulting in muscle weakness and atrophy.
The disease affects patients differently with a wide range of symptoms. Enzyme replacement therapy (ERT) only slows down the progression of muscle weakness. ERT is delivered through IV infusions every other week for the rest of a patient’s life. Eventually, I began to realize that my life is now on a different path.
A sloth called ‘Hope’
My journey “On the Road to Pompe” started when I received my diagnosis on Nov. 19, 2018. I then needed the fuel to propel me down the road. It was time for me to get my “muscle juice” during my first infusion on Dec. 19 of that same year. I was fortunate to only have to wait a month before filling up my tank.
On that first infusion day, I decided that I wanted it to be as fun and enjoyable as possible. Physically, I was able to continue working a full-time job, and I could do the work on my laptop while in the infusion chair. But I felt I needed something more to do. I discovered a Facebook group called “Hope Travels – Pompe Awareness,” and I became an ambassador for the group with my sloth, which I call “Hope, the SoCal Beach Sloth.”
A sloth called Hope is the unofficial mascot for Pompe disease. Some may laugh or tease me about my many stuffed animals. But the joy and encouragement that the Hope Travels group brings to others — I wanted to be a part of it. Maybe it would help me on my journey, too.
I thought it would be fun to take photos of my sloths and me with the IV pole, while I did my “pole dance” of funny poses. All of these things helped to make the infusion go by more quickly while having some fun in the process.
Every other week, I drive to the infusion center to get treatment. The number of infusions keeps getting bigger and bigger. Last week, I celebrated being on treatment for four years — with 105 infusions. That seems like a lot to me, but there are Pompe warriors who have had more than 300 infusions. A few have even had 400! I can’t fathom that yet.
Some people ask me when my treatment will be finished. My only response is: never. I will receive infusions until the day I die — or until a cure is discovered.
But I hope that better treatments and outcomes will soon come for Pompe patients. And I have hope for a cure in my lifetime. Until then, I will have fun and take photos at my infusions to halt the negative thinking that can happen when I’m just sitting around for hours with an IV in my arm.
Another thing I can do is spread awareness about Pompe disease. I am living my best Pompe life, smiling big each day while bringing a smile or laughter to others as well. I’ve got four years “On the Road to Pompe,” and I’m gearing up for many more. Let the muscle juice flow!
Note: Pompe Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pompe Disease News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to Pompe disease.