The rate of progression and the severity of FSHD varies widely among those affected, but my experience may be typical of the “average” course of the disease. I was diagnosed in 1968, and the progression has been slow and relentless. The slow progression meant there was never a time where I felt that tomorrow would be any worse than today. In reality I was weaker, but the change over that short time period is imperceptible. Changes over the course of a year, however, can be dramatic. For example, I used to love basketball and had a decent jump shot at one time. I recall the occasion one year when I hadn’t play since the previous winter. I was standing just under the rim and was shocked to discover that my right triceps muscle was so weak that I could no longer even shoot the ball two feet into the air, much less into the basket. When I look at the cumulative effects of FSHD over my lifetime, the effects have been profound. For example, I was last able to “run” in 1990, was too weak to lift my 6-year-old daughter and cannot hold my two small grandchildren, and now can no longer walk or stand.
Still, day to day I learned to cope with FSHD and to develop ways to adjust to the disease. My attitude became “this is a drag, but it’s not so bad…it could be worse..and I can handle it.” I think this “I can handle it” mindset is common among many with FSHD, and it may be something that prevents many from becoming advocates for FSHD in the way that people become advocates for breast cancer or ALS, for example. Humans are motivated to act when there is a sense of urgency (just look at how difficult it is to get action on climate change), and with a slowly progressing disease we are not hit by that sense of urgency…at least I wasn’t.
But in recent years I have met a number of young boys and girls, men and women, with FSHD. Individuals who face the same lifetime of advancing weakness, and in many cases worse, unless there are significant advances in research. Unless those of us who have lived with FSHD come forward and speak out on behalf of awareness and research, the next generation of kids and adults to be diagnosed with FSHD will be no further along than I was in 1968. It’s my duty to become an advocate for FSHD.
Fortunately, there is growing advocacy among the FSHD community, and my story does not apply to everyone. I am particularly impressed by some of the young people I have met through the FSH Society, people who have put themselves out there and told their stories in print and with images. FSHD research is ahead of where it was in 1968, but it remains underfunded and FSHD in generally under the radar of the general public. I hope the Doug & Gracie fundraiser will do a little to shed more light on this disease.