This post was written by Richard Wheeler. You can visit his blog at http://mma4cmt.blogspot.co.uk/
Arthur Keeney came to California from Oklahoma, with his wife Grace and the first few of thirteen children, back in the “dust bowl” days immortalized in John Steinbeck’s 1939 “The Grapes of Wrath”. He was in his late sixties in my early childhood and had the oddest hands and walking gait. The fingers of his hands were tightly gripped into his palms and his thumbs hung limp and useless. He could really work those knuckles though and would smack them down hard and loud on the kitchen table if any of us children became disrespectful. He wore the traditional hats, shirts, and trousers that many men of his generation did. However, he always wore the highest combat boots and walked with a “marching” gait as if he were always stepping over some obstacle.
While he was still with us, it had always been assumed that he had suffered “bent bones” from early childhood polio. None of his thirteen children showed any signs of his disability and no inherited disorder was ever suspected. I was the first great-grandchild and I did not show any early, obvious signs and there was no anticipation at that time. I was never first place in physical competition, but could usually place second or third. I used to have terrible “growing” pains and frequent neck pain that would be very painful and make it impossible to turn my head for several days.
I did not have a “dad” figure around until twelve and whenever I tried doing the “guy” things that required hand dexterity on my own I found it so frustrating that I suffered extreme angry melt-downs that I kept to my little self.
I remember trying to put together a model car and every time I tried to remove a piece I would break it, and got more glue on my hands, clothes, and desk than on the car. I smashed it into bits. I felt that I was defective due to lack of
father figure. I am not reporting this to gain sympathy nor cause my Dad who I now know, love, and appreciate any pain or guilt. Rather I intend to illustrate how important the medical knowledge alone could have been in helping me deal with the issues.
I am reminded of my Great-Grandpa Keeney, an extraordinary man who lived a long and hard working life. He did his best to raise a huge family and instill a proper Christian work ethic in them. He suffered pain and the inability to perform many daily tasks that most can take for granted without ever any knowledge of why or hope for any relief, let alone a cure. A cure for what? I don’t think he enjoyed the financial support that allows many people with disabilities today, in America, to live a relatively comfortable, secure life.
Some time after he passed away my cousin, the third or fourth great grandchild I think and my mom’s sister’s first son, received a diagnosis of Muscular Dystrophy at an early age and was placed in extremely rigid AFO’s immediately. Soon symptoms were beginning to be reported by some of Arthur’s daughter’s sons and more of the other great grandsons. At nineteen, I was diagnosed with Charcot Marie Tooth only experiencing a minor loss of foot strength and range of motion at that time. I was told by the diagnosing physician that there was no way to know the course of the disease for myself, no treatment or physical therapy to do, to stay active but not over exert myself, and oh yeah, stay off drugs and alcohol.
The doctors at that time were telling me I had CMT, a muscular dystrophy. The Muscular Dystrophy Association (MDA) paid for that first visit and later in life as my denial and fight against CMT began to take it’s physical and spiritual toll. The MDA has provided braces at times but I soon tired of EMG’s and NCV’s that were painful and provided nothing but confirmation of deterioration that was visually apparent. Many of those tests were required to prove I still had CMT for various services I needed. The clinics I attended seemed to use me as teaching tool and did not really interact with me as a person. I was even told at one point by a doctor that I did not have Muscular Dystrophy, CMT was a Neuropathy and that I should not count on MDA’s support much longer. Years later I learned that doctor was both right and wrong! CMT
is an inherited peripheral neuropathy but is still supported by the MDA.
Picture courtesy of Richard Wheeler
With bracing, further education, and stubborn drive I continued to work into my forties. I am now forty-five and have spent much of the last couple years on my couch and in a power chair. Due to one lousy toe bending increasingly under my foot, I could no longer wear AFO’s. I resorted to soft OTC braces and a cane which led to falls, a minor leg fracture, sore hands, elbows, shoulders. The information now available about surgical options were overwhelming and I have family that went through some pretty major procedures years ago that did not produce desired results.
I again went to MDA and they helped me coordinate a visit to Stanford’s neuro clinic in California. What an amazing experience, they showed me new AFO products and had a line of specialist waiting to answer any questions I had and offer resources. Jennifer Fisher from MDA also met me and spent time discussing my needs and concerns. I was really approaching a low in my life that was scary before that visit. With renewed spirit I went to the MDA website looking for support groups I had avoided in the past for various personal reasons.
There was nothing close enough to attend but I kept searching. I think I found the link for the Charcot Marie Tooth Association (CMTA) in MDA’s online Quest magazine. There I found online support and discussion groups that exponentially multiplied my access to others with CMT as well as medical professionals that will respond to almost any CMT related issue. I found many new friends that understand and offer insightful advice for CMT issues and even other more personal concerns.
One of these CMTA friends led me to CMTUS on Facebook and wow, it was like catching another gear in a muscle car. Now I have another group of people I consider family. We often have minute to minute discussions providing revelations in our knowledge and awareness of our disorder and treatment options. Many questions that so many doctors simply do not have the answers to, such as “should I lift weights?” are discussed in enough detail by a large sample of people with CMT that one can learn what to educate their doctors about to help. The experiences reported on CMTUS provided me with the confidence and knowledge to discuss my options with my podiatrist and finally get some surgery. Very simple procedure has given me new lease on life. I can wear my AFO’s again!
Part Two of this post will be added later this week.