By the time I was diagnosed with a progressively disabling disease in March of 2006, I was walking with a slight limp and could no longer run or jog. Back then, my experience of disability was solely one of losing leg strength. This corroborated my naively simplistic, pre-disease definition of disability- not being able to get from point A to point B. Disability equaled wheelchair use. After my diagnosis, whenever I traveled down the worst case scenario path of GNE myopathy (aka HIBM), it always dead ended with me in a wheelchair.
I quickly learned that just because my ability to move around upright wasn’t impaired, it didn’t mean I wasn’t disabled from engaging in activities that had brought me so much joy and fulfillment throughout my life: hiking, biking, kick boxing, dancing, ice skating, skiing, walking on the beach, getting in the ocean….Though each of these abilities was painful to lose, eventual acceptance of their absences allowed me to access their alternatives. I eventually transitioned from mourning the losses to celebrating the gains that adaptive devices and motorized machines had made. In essence, I was able to appreciate that at least there were substitutes.
Then the upper body weakness set in. It had actually been there all along, but not in the significant, disabling way it was slowly morphing into. How foolish I had been for believing that not being able to walk was the most dreaded outcome of this disease. There is an easy fix for that disability. Such is not the case for hand, arm, upper back and shoulder weakness.
There are periods of time throughout the day when I don’t actively engage my legs-seated in a chair while working, eating a meal or watching TV or while sleeping in bed- but there are never moments when I don’t need to use my hands.
Early on, I mourned the losses of the fun, optional activities my upper body used to afford me: playing guitar, playing piano, throwing a ball overhead, walking while holding a beverage. But now, I’m gradually struggling to perform tasks integral to my daily life: opening jars and containers, taking things out of the refrigerator and oven, lifting something off a shelf, taking on and off clothing, shoes and jewelry, typing on a computer, writing with a pen, clipping my nails, cutting my food, firmly shaking hands, hugging. Yes, hugging.
Though there are few adequate adaptive devices to substitute for these upper body actions, there are at least other human beings who can and do assist me. For them, I am exceedingly grateful.
As exhausting as it is to navigate my weakened body through time and space, at least it is familiar. I can always rely on the fact that repeated exposure to a stressful stimulus results in a desensitization to it. I continue to struggle physically, but I am less reactive emotionally.
Such is not the case with the new loss I am currently experiencing. It feels very raw and depressing and scary. The irony is that it has nothing to do with moving.
I am ever so slowly losing my ability to stand upright, unassisted.
I never anticipated this particular manifestation of my disability. I always feared not being able to do or move. I never feared not being able to stand still. And yet, it makes perfect sense. So many healthy muscle cells are required to fire at once just to engage in the seemingly simple act of standing.
In the early days of physical therapy, I used to balance on one leg on a foam pad, not wearing my braces and without holding onto anything for stabilization, while lifting my other leg up and down 10 times. I would do three sets on each leg. Last week in physical therapy, I spent part of the session trying to maintain my balance while standing on both feet on the flat ground, wearing my leg braces and reaching out to the wall for support.
It is becoming increasingly difficult to convince myself that I’m not stuck on a slow, winding path towards quadriplegia when such traumatic losses like these lurk behind every corner.
When the music streaming service Spotify came out, I made a workout mix. The very first song I put on it was Elton John’s “I’m Still Standing.” Early on in my disease, I jokingly decided that this song would be my anthem. When we go on trips and I can still, however barely, get by on some treacherous terrain walking upright, I sing this song to my husband (and myself) as a way to break the nervous tension that threatens to contaminate my fun. It reassures me long enough to get me through the moment and move on.
But now. Now I can no longer blast that song. I don’t even know for how much longer it will be a relevant soundtrack for those particularly painful moments when I’m trying desperately to balance the dread and the joy. I am terrified. That’s my song. I don’t want to have to pick a new one.