"Are you dying?"

Hereditary Inclusion Body Myopathy (HIBM) is a progressive and debilitating muscle wasting disorder caused by a gene defect, affecting all of the body’s skeletal muscles, those we use for movement.   Since the disorder is recessive (both copies of the gene are damaged), anyone may become affected, even with healthy parents and parental families. HIBM touches those between the ages of 20 to 40 and although progression is slow, it typically leads to total disability within 10 to 15 years. The weakness and severity can vary from person to person. In some, weakness in the legs is noticed first. In few others, the hands are weakened more rapidly than the legs. Weakness is progressive, which means the muscle become weaker over time. Patients with HIBM come from all different backgrounds; the disease has no ethnic or gender boundaries. Currently, there are no effective treatments for HIBM and there is no cure.

Whenever someone asks me questions about my situation, I appreciate their curiosity as much as the opportunity to raise awareness about HIBM. What is sometimes not expected is the way they interpret what is happening to me. The most profound inquisition was in the middle of a casual conversation and the person said, “…so when a muscle has totally wasted away, that part of your body is dead, I guess, because you cannot move it anymore. So does that mean your body is dying? Are you dying?” You can imagine my reaction, first shocked by the question, and then perplexed by the thought that my body might be dying. Never before had my condition be posed to me in such a way, nor, had I ever embarked on such a morbid way of looking at my weakening state. As I do in many confusing HIBM moments, I shot the thought out of my mind. I am not dying! The muscles are wearing away.  

I refuse to dive into such destructive states of mind. I refuse to let my head be clouded with ideas that can only leave me distraught. I refuse to be distracted from focusing the greater part of my energy on the wonderful life I lead… because YES OF COURSE there are instances of sadness, frustration, anger, fear or embarrassment; without them I would not be human. These intense feelings and emotions can easily take over and make you crazy. But thankfully I remember my father’s words, “Just relax and take it easy!”

So what I guess what I want to share with this entry is that I sincerely do not worry about what the future holds, nor do I harp on the scientifically proven fact that my body is becoming weaker every day. With all honesty this is the best time in my life and I have never been so happy.  I use the limited energy that I have to live it up and make noise about HIBM so we can fund the cure! Slowly but surely, (AJ baby), I can do anything!

To learn more about HIBM, please visit: www.hibm.org