Although I write openly about my health, I don’t track what I’m losing or what FSHD is doing. Every time I see my specialist he asks what’s changed, but I don’t give him the whole picture. I forget things and kick myself after. This post is a way of tracking it better.
FSHD (Facioscapulohumeral muscular dystrophy) is broken down into different areas, beginning with the face (Facio). I’ve noticed some shift. My mouth drops when I sleep. Unlike people I know, I can close it when I’m awake, but when I relax it naturally opens and drops.
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| [Image 2. The image focuses on my the lower face and mouth, illustrating facial weakness associated with FSHD. The slightly open lips and visible facial hair highlight reduced control and movement, reflecting difficulties with whistling, using a straw, or puckering. This photo was taken in 2021. It's visibly the same despite being weaker] |
At night I drool and end up waking with pool of drool around my collarbones. I hate it.
My eyes have worsened too. I’ve used an eye mask for over a decade, but Hannah and the kids have noticed that if I fall asleep early or nap without it, my eyelids close halfway and my pupils roll back. My speech has also shifted. I mentioned this in March. It’s still the same letters, but I stop, change words, and say things wrong more often. Unless you know, you’d think it’s mispronunciation, but it’s not.
Shoulders (Scapulo) and upper back are next, causing my neck to worsen. Despite my neck twitching before 2014, it’s progressed. Hannah regularly applies deep heat to my neck and shoulders to help. The neck muscles weaken and pull forward, which forces the shoulders to compensate and makes me pull myself upright. We have a lovely family photo from Wrexham at Hannah’s brother’s wedding. I was in my manual chair, without the head support of my powerchair, my neck dropped forward, and it cuts my neck out of the photo. The photo highlighted how much it’s declined, especially compared to decades‑old photos.
Now.
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| [Image 3. A group photi of the six of us as we pose together outdoors in front of a decorative black metal gazebo. From left to right standing, James, Will, Hannah, Midge, and Arty. I sit in a wheelchair at the front. We are dressed in semi‑formal clothing. This highlights the neck change i describe above] |
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| [Image 4. A group photo of the six of us at Rochester pride 2022. From left to right standing, Hannah, James, Will, Midge, and Arty. I sit in my chair in front of James and Will. The kids are wearing sexuality flags and James and Will have their flags painted on their face.] |
FSHD then moves to the stabilising torso (humeral) region. This is the biggest and quickest degeneration. Despite writing about it in May, it’s weakened more. Travelling in my powerchair has become harder. I wrote in July that travelling isn’t easy. People make jokes, but the reality is different. I regularly navigate uneven curbs, cobbles, patchwork paths, bumpy pedestrian islands, and slopes that jolt my core and cause me to slip and slump in the chair, making me sore and physically tired.
It’s become so difficult that I now wear a Double Pull Lower Back Support Lumbar Brace. It wraps around the torso and uses adjustable tension straps to stabilise weakened muscles. It supports the lumbar spine, pulls the core into alignment, and reduces the wobble, slip, and fail. It helps, but it highlights how much strength has gone and why I tremble and almost fall as I try to hold myself upright during transfers.
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| [Image 5. A close‑up of the back and side of a person wearing a black and blue lumbar brace. The belt’s elastic bands and adjustable straps provide compression and support to the lower back, symbolising the physical adaptations used to manage FSHD‑related weakness.] |
My MD belly has also worsened. People think it’s fat or weight gain, despite losing weight. When I wear the brace, everything pushes in and reshapes. Without the brace, I fit a stretchy pair of thirty‑six‑inch waist jeans and need large shirts. With the brace I’m a thirty‑four‑inch regular fit waist and medium shirts. Instead, my back curves, changes shape, and arches, pushing my stomach out.
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| [Image 6. The image highlights the humeral region, showing visible muscle weakening and atrophy around the neck, shoulder blades, and upper arms. It visually represents the physical changes caused by FSHD, focusing on the biceps and triceps areas. At the abdominal area you can see how my MD belly pushes out and bulges, pushing down and forwards. Whereas, you can also see the curvature of my spine when its misaligned. These photos were taken in 2021. This is worse now.] |
FSHD then moves into the arms and hands. I feel this loss personally. My right hand has worsened. I struggled typing last year at college. I’m also struggling to play the piano, which breaks my heart. My fourth and little finger no longer straighten properly. Unfortunately, the shape I use to drive my chair matches my hand shape, making the repeated posture waste and weaken the muscle. The joystick posture shows again when I write, hold my phone, and use utensils. I tried correcting it. It mostly worked, but it’s off. My writing is messier than normal, which in turn makes me worry about drawing. Then there’s my left wrist, which is currently in a support strap. Without realising it, I’ve stopped using my right arm as much and compensated with my left.
FSHD eventually reaches the legs. Mine have thinned, weakened, and bowed more. When I stand during transfers my legs start shaking and pull me backwards. Hannah had to grab me this week before I toppled backwards. She thinks it’s core related, but I feel my legs pulling me back. My right ankle also gives way repeatedly. I end up standing with my left foot flat and the rest of my weight on the buckled ankle, which causes a sharp, deep, and unbearable painful throb.
This core and standing issue has also changed something else. We converted our bathroom into a full wet room in January and the Occupational Therapist pushed for a hoist. I hate them. They remove independence. I knew I’d need one eventually, but I was ok. I thought I had time. I don’t. I hate to say it, but it’s so bad we’ll need one soon.
I’ve written openly and honestly about my health for years, but never really tracked it. I need to do it to pinpoint those changes.
This is my FSHD now. It’s not dramatic. It’s simply the truth.
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