My disease progression feels like barreling down a hill with no brakes
An onslaught of new setbacks leaves a columnist anxious about the future
When I was 13 and my brother, Tim, was 16, we got skis for Christmas. I’m not sure why Mom and Dad decided to get them for us. Jeannette High School, my alma mater, had a ski club, but Tim and I weren’t members. I don’t recall asking for skis. Perhaps Tim did.
However it came about, we suddenly had skis. Of course, we had to try them out, but how? Joining the ski club was free, but the cost of going skiing was more than we could afford. Tim had his driver’s license, but it was a long drive to any of the Western Pennsylvania ski resorts, another expense we couldn’t afford. We were advised that beginners should take lessons. We couldn’t afford those, either.
Tim and I decided to give it a try on a hill at the local country club where we liked to go tobogganing with friends. None of our friends had skis, so Tim and I were on our own. I’d also like to mention that while we had skis, we didn’t have ski poles. In retrospect, those might’ve been helpful.
I recall us walking up the rather steep, snow-covered hill, then strapping on the skis. We had no idea how to start, steer, or stop. We simply stood up, pointed our skis down the slope, and took off.
By now, you’re probably guessing that it didn’t end well. The hill was perhaps a quarter-mile long. It’s amazing how much speed you can pick up in such a short distance. It’s also amazing that neither of us sustained any permanent damage as we hurtled down the hill and crashed over and over again at the bottom.
Searching for that confounded ski lift
I’ve had facioscapulohumeral muscular dystrophy (FSHD) for over 50 years. I was actually diagnosed just a few months after that skiing misadventure. For many years, my disease progression was slow, with gradual loss of muscle function followed by plateaus when things remained relatively stable.
Now, my FSHD progression feels a lot like those teenage skiing excursions: I’m going downhill faster and faster, with no way of steering, slowing down, or stopping.
In the past, I felt like I had a little time to adapt to my body’s changes before the next ones surfaced. Currently, the changes are coming more rapidly than I can handle. As I barrel down this FSHD slope, I can feel the crash coming as I approach the base of the hill. All I can do is try to brace myself for the impact.
I’ve gone backward on my exercise program and can no longer perform the reps I was doing just a few weeks ago. As I move around the house, I feel totally unbalanced anytime I don’t have both hands on my walker. It seems harder every day to stand up from a seated position. I now have to brace my elbows on my knees and bend forward to blow my nose or anything else that requires two hands at head height. The elbow I hurt when I fell a couple months ago flares up every time I lean on it. And as I indicated in my previous column, I’ve had to stop using my accordion when I perform at nursing homes, because everything is too heavy.
All of these recent changes seem to have happened simultaneously, making me feel totally overwhelmed. It’s created a lot of anxiety, and I wonder how much time I have before I crash at the bottom of this FSHD hill.
What will my life look like after the crash? Will I be in a wheelchair full time? Will I be bedfast? Will my wife, Wendy, and my daughter, Jill, be able to provide the help I need? Can I get enough assistance to remain in my home, or will I end up in assisted living?
What, if anything, can I do to prepare myself for the impact? Should I stop doing things that are difficult and risky, but still possible for the moment? Should I lie down now and try to avoid a violent crash by gently sliding? Is there really such a thing as gently sliding with late-stage FSHD?
Right now, I have more questions than answers. I’ll be praying about all of this. I’ll share any answers I come up with in future columns. In the meantime, I’ll just try to stay upright on these skis and keep my balance.
If you’re careening down a steep slope of your own, try your best to keep your balance and ride it out. To quote a colleague of mine from years ago, “Are we having fun yet?”
Note: Muscular Dystrophy News Today 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 another 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 Muscular Dystrophy News Today or its parent company, Bionews, and are intended to spark discussion about issues pertaining to muscular dystrophy.
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Jennifer Egg
Rob, I so relate to this. It had been so slow for so long, I thought maybe I’d be just fine, it wouldn’t really happen… the past two years are different. And very anxiety provoking. Thank you for sharing this.
Rob Stemple
Hi, Jennifer. Yes, I don't usually let things bother me too much. I believe gOD has a plan and, somehow, FSHD is part of that plan. Still, I struggle with how HE can make good come out of something like what's happening to both of us. It's a puzzle, but I still believe that, when I pass on to the next life, I'll get a higher view and it'll all make some sense Hang in there!
Robin Carlino
Oh Rob, my heart goes out to you. You so candidly expressed so many feelings that I have myself. I am still mobile but thinking of asking again about getting a mobile scooter. It's kind of like waiting for the other shoe to drop. Besides FSHD, I have 2 artificial heart valves and all that entails. Last year I had a brain bleed and several TIAs. I no longer drive. This puts quite a heavy burden on my husband, who has his own health problems. Only God has the answers, so we continue to pray. What else is there?
Thank you for expressing so poignantly what we feel.
Blessings,
Robin
xo+
Rob Stemple
Hi, Robin. You're in my daily prayers, as you've shared your health situation with me before. It doesn't seem that having FSHD immunizes us against other health struggles. For you, it's your heart problems, the brain bleed and those TIA's. For me, it the blindness and the damaged joints from my car crash. Still, I know you're a talented artist, as per the last FSHD Wellness group presentations and I have my music! We'll get through this struggle and, hopefully, our art will help others get through, as well! GOD bless!
Debra Sue Love
I can totally relate with you Rob. I had an appointment via telehealth with my specialist this morning in St Louis. Unfortunately I have slid downhill a lot in the last 4 months since I have not had transportation to aqua therapy. I was making improvements doing the aqua therapy and hopefully will get back soon as I can get some dependable transportation. My heart hurts for you too. I am thankful that you have your family to help you through this time and I would like to encourage you to keep on doing all you possibly can. Your music 🎶 was such a blessing to everyone at the meeting this week. We were all singing along with you and we were all tapping our toes to the beat. My doctor said there's a trial with a new drug that they are trying to get approved. Praying for you Rob 🙏
Rob Stemple
Hello, Debby. Glad you enjoyed the tune at the Wellness group meeting. I enjoyed hearing about the art some many of us FSH'ers are creating, including your paintings of barns and other images. We'll make this world a better place than when we got here, GOD willing, right? Thanks for those prayers!
Sarah Loyd
Hi Rob, if you do need a wheelchair, that really isn’t a disaster though it might feel like one until you adapt. I have had to use a powered wheelchair for about four years now and whilst it initially felt awful and had lots of issues to solve, life goes on.
Rob Stemple
Hello, Sarah. I use a wheelchair whenever I'm out of the house. That's been the case for quite a while. This was a transition I struggled with a few years ago. As a blind person, a powered chair isn't really an option, but I have a wheelchair that's equipped with a Smart drive. This helps take the load off my pushers, so all they need to do is walk behind and steer. Inside the house, I get around pretty well with a walker, but I'd have a lot of trouble navigating and actually pushing myself in a wheelchair. We've got some tight corners. I've also tried pushing with my feet when I'm using my transport chair and that doesn't go well. My arms don't do much better with my regular wheelchair. It's a bit of a knundrum. I'm hoping the walker is going to continue to be my friend for a while. Thanks, so much, for reading my column and taking the time to respond. I'm glad that your power chair gives you the freedom to move around independently! Keep on rolling! GOD bless!
Kathleen ruiz
Very insightful sharing of your adventure with FSH. I plan on reading it regularly. I have a friend that has FSH and this helps me understand the situation better.
Thank you!!!!!!!!
Robin Stemple
Good morning, Kathleen. Thank you for being a friend who cares enough to do some research on your friend's disease. FSHD is very unpredictable. Some folks folks have a faster progression than others. I'm praying that your friend's progression is so slow that it's barely noticeable for years to come. Thanks, again, for being a good friend!