What I want people to know about FSHD and its progression

The disease affects every aspect of a patient's life, this columnist says

Robin Stemple avatar

by Robin Stemple |

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As I was finishing up a recent interview with the host of “FSHD Radio: Straight Talk with Tim Hollenback,” a service of the FSHD Society, Tim’s final question was, “If there’s one thing you’d like people to know about facioscapulohumeral muscular dystrophy [FSHD], what would it be?”

It was a great question. I’m not sure I had a great answer.

This episode of “Straight Talk” will air sometime in August. I’ll have to give it a listen, as I really can’t recall how I answered the question. Hopefully, it wasn’t totally incoherent.

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There’s one thing I prefer about writing a column for Muscular Dystrophy News Today instead of doing a podcast or another type of audio or video presentation: As a blind person, I have the opportunity to listen back as Jaws, my preferred screen reading software, “reads” the column back to me. As I listen, I think about how the words will land with my readers. If a sentence doesn’t sound right, I can make changes before readers get a look at it.

That isn’t the case for interviews. Once you say something, it can’t be unsaid. Since recording the interview, I’ve thought about Tim’s question quite a bit. I can’t boil it down to just one thought, I can only get it down to two ideas that I’d really like people to know about FSHD.

FSHD is pervasive

First, I want people to be aware that FSHD varies greatly from person to person and changes over time. When I was diagnosed in 1972, I couldn’t raise my arms above my head. I couldn’t whistle. I also had trouble keeping my eyes closed. They would tend to drift open when I slept. Otherwise, I pretty much did everything normally.

The second thing I want people to know about FSHD is that, eventually, it can impact every part of a person’s life. My FSHD has caused devastating weakness from head to toe. FSHD affects me from the time I get out of bed in the morning until I lie down to sleep at night. Then it keeps going, even while I sleep.

Repositioning my body or rolling over in bed becomes more difficult every night. FSHD affects the way I get dressed and comb my hair. It makes it a challenge to get on and off a toilet. Brushing my teeth is a painful and messy process. Even holding my head up is hard, so I make sure I’m positioned over the sink so the slobber can be easily cleaned up with a rinse.

FSHD dictates where I sit. If a chair is too low, I will struggle to get up. If a chair isn’t padded, I’ll be in pain in a few minutes. If a chair doesn’t have a back, I’ll be slumped over, with my head down, very quickly.

FSHD also causes frustrating levels of fatigue. Many times there are things I want to do, but I simply don’t have the energy to do them. I’m not sleepy, my body just refuses to cooperate.

FSHD dictates that anything I need from a kitchen cabinet is stored on the lowest shelf, as I can no longer reach the upper shelves. FSHD has also stolen my balance. If I drop something, someone else will probably have to pick it up. If I bend down to pick something up, I not only lose my balance, but I also find it difficult to move back into a standing position.

I no longer get on my hands and knees to locate something I’ve dropped by doing a grid search, the technique I was trained to use as a blind person. If I do, it will require two people to get me back on my feet.

One word I recall coming up with during my interview with Tim was “pervasive.” There are very few activities I do or decisions I make that don’t include taking FSHD into account.

If you’ve read my columns for a while, you’ll know that I’ll keep doing as much as I can for as long as I can. I’ll keep doing the exercises my physical therapist showed me in hopes that I can hold off any further effects of FSHD a little bit longer. I’ll keep writing, playing music, and doing disability justice work as long as I’m able to. I’ll keep going until I just can’t.

I’m praying that I’ll know when it’s time to surrender and see how the next chapter of my life unfolds. I’m not sure what the next stage will look like, but I know it’ll be an adventure!

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 or its parent company, Bionews, and are intended to spark discussion about issues pertaining to muscular dystrophy.


Rich avatar


Everyone with FSHD will not have the life they should have and it will kill you quicker than if you didn't have it......as a community we need to stop pretending like we don't have FSHD at birth....all that have it , have it from birth and it ruins everyone's life....there is nothing you can do to get better ..only worse....hoping for treatments soon for a family of 3

Robin Stemple avatar

Robin Stemple

Good morning, Rich. I can't argue with anything you've said. The "roots" of our FSHD are part of the genetic makeup we're born with. We can't change that. Like you, I'm hoping for a treatment soon. I'm also hopeful that they can develop some sort of vaccine so future generations of our family can just get a one-time shot that will take care of Dux-4 before it has a chance to destroy our bodies! Hang in there, my friend. Our challenge is to make the best life we can with the cards we've been dealt. Not always an easy task! - Rob

Virginia Plihcik avatar

Virginia Plihcik

Thanks Robin! Great article as always!

Robin Stemple avatar

Robin Stemple

Thanks, Virginia. I hope you continue to enjoy my columns and that they resonate with you. I appreciate you taking the time to read them!

Kathy Senecal avatar

Kathy Senecal

Rob, you did a wonderful job describing the day to day life of planning, adapting, and navigating the day in a manner that shows the thought and "McGyver-ness" we do to manage, survive, and thrive.
Well done

Robin Stemple avatar

Robin Stemple

Hi, Kathy. I guess we all turn into MacGuyvers, don't we? (lol) Hang in there and thanks for reading my stuff!


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