Charcot Marie Tooth – Part of my Story

The most evil thing about Charcot Marie Tooth is that you can want to do everything in the world to get better and then you find that there is nothing you can do to get better. It seems like a worse diagnosis than if you were told you have cancer and there is only a 50 % chance of survival, at least in that case you can do everything right and you have a chance at regaining your health and strength. With CMT the reality that really does seem totally incomprehensible is that you cannot really get better, you cannot build up your muscles, the best you can hope for is not to lose more than you already have lost. No matter how willing you are to exercise for however many hours there are in a day, there is no way to build any more muscles, period. It really does seem like a death sentence of sorts because you can do almost nothing and be weak and you can exercise for hours a day and be equally weak or even worse. There is really no other disease that this scenario happens with, almost everything gets better if you work at it. That is the amazingly depressing scenario of Charcot Marie Tooth disease. I was three years old I have been told by my mother that it was when she noticed I wasn’t able to walk very well, even for a baby, the disease had onset and what it muscles were claimed first were those of my feet and ankles and I couldn’t walk the same way other children of my age were able to do. Then there were losses of different muscles, my hands and wrists became weak and I could not grasp things very well. Then when I was six, just after I had started grade school, the orthopedic surgeon my mother had taken me for what seemed like a million times suggested that they perform a surgery on each of my ankles to set the main bone in my ankle at an angle that would make it harder for them to twist them due to my weak muscles. I don’t really remember what it was like before those surgeries but I do remember the terror I experienced when my mother took me to the hospital and I woke up after the operation with a cast on my foot. She never told me what was going to be done to me and it freaked me out and created a lifelong fear of doctors and hospitals that possibly could have been avoided had she told me that I was going to have a surgery on my ankles so I could walk better, she never told me anything which created my being totally freaked out when I came out of the anesthesia and was in pain with a cast on my ankle.  I already felt like a freak because I couldn’t do the things other kids could do but this took feeling like a freak to a whole new level. Even at the age of 56 I still remember how scared I was when I woke up in that hospital bed, with three other kids in the same room with different kinds of problems. The only kid I remember was the one who had his tonsils removed because he got to eat ice cream all of the time, doesn’t that sound like a reason a kid would remember something?  I remember not wanting to go to school with my cast on as I have already stated that I had felt like a freak before the cast and certainly didn’t want to be any more different than I already was. Here is an excerpt from a paper my mother wrote for a class she took when I was going to college from a teacher I had taken psychology and philosophy classes from that I told her she would like. I only got to read this paper she wrote after she died of cancer four years ago when my stepfather gave me her class papers. It wouldn’t have changed my experience with the hospital I had as a child but might have helped me deal with how screwed she was later on in my life.  Here is part of what she wrote:

When Michele was six she had to have operations on her feet. I obviously did not prepare her for these operations as well as I should have. She is 27 now and the other day she said “when I overheard you talking to someone about that first operation, I became so frightened I nearly died!” I think that I should have taken her to visit the hospital before the operation, but even more importantly, I should have talked to her about her feelings, even though she didn’t ask any questions I could have said, “I know you are very frightened about this operation and I know it will be unpleasant and you will have some pain, but you will be able to walk better and I will be there with you to help you.”
When she returned to first grade on crutches, as we approached the classroom she didn’t want to go in. Her teacher suggested that I leave her and gently restrained her from going with me. I did leave her because I felt she could handle it, thought was difficult for me. 

Well of course I didn’t ask any questions, I didn’t know what was going to happen so what questions could I have asked? When I was diagnosed with CMT 53 years ago they didn’t know very much about the disease and CMT was classified as a form of Muscular Dystrophy, the only form that didn’t continue progressing in its destruction of the muscles until you died from it at a very young age as is the case with MD. They now know that the atrophy of the muscles in CMT is not as direct as it is in MD proper, in CMT the nerves that control the muscles are destroyed which in turn cause the muscle loss. When I was directly asked what was wrong with me when I was young and very little was known about this disease, which didn’t happen very often, I could only answer that I had a rare form of MD that only affected my feet and hands and didn’t progress after its initial damage and this is what I believed about my disease until I was in my forties because even after the internet came out I refused to research my disease because after I had the second two surgeries on my ankles at 16 my orthopedic surgeon told me to go out and live a good life and I shouldn’t ever need to see him again. Well I always felt like this was a little too optimistic but I also feel in some cases ignorance is bliss, so to speak. I had noticed that no matter how hard I exercised I could never climb stairs any better than I could before I worked hard at building up those muscles, my thighs, stomach and upper arms would get more toned up but my grip never got any better and my ability to go up and down the stairs always required that I had to hold on to a rail or a friend’s hand to not fall no matter how many times I went up and down the stairs, in fact if I went up too many or too steep of stairs I would run out of strength in my legs and have to sit where I was as my legs would just give out. They would recover after giving them a rest of sometime ten minutes and sometimes it would take an hour but I have since found out that irreparable damage can be done by pushing my body like that and I am very thankful that I didn’t have that happen because I had no idea that could happen. Probably if I had looked CMT up on the internet I might have found this out but I was hopeful and optimistic about my CMT up until the first time it really got worse since I was a child at the age of 51. I feel that what my mother once said about me that I was “too optimistic”, this served me well in my not getting too depressed about not being able to improve my physical abilities and had I known that losing more of the use of my hands would most likely occur as well as my legs getting weaker I might not have traveled to Mexico or have gone to Spain, so since not knowing the truth didn’t hurt me I am glad that I didn’t spend a life in fear of losing the use of my legs or hands until it actually became a reality. I saw a video on You Tube recently where a young woman was determined to do a physical skiing activity so badly that she totally exhausted her muscles to the point that she never recovered, I have done things close to that but quit short of the point she did as I recognized I was getting weaker at what I was trying to do so I saw no point in continuing to do the activity that I was not able to do. If I had continued on I quite possibly could have done the same thing to my legs that she did to hers but since I wisely chose to give up when I found I wasn’t getting ahead I didn’t do any permanent damage but it could have turned out different and I do understand the young gal’s determination to try and do something that seemed like it should be close to attainable. The desire to improve one’s self is a very powerful and necessary element in a human being’s life and the removal of that ability is one of the most depressing aspects of the disease of Charcot Marie Tooth. There really isn’t anything we can do to get better and it is rather depressing, granted I am better off than a person in a wheelchair but the knowledge that the best you can do is maintain whatever strength you have and never ever get any stronger is a powerfully depressing notion as hope is one of the biggest motivators in any human being’s life and to take some of that away is to take away a little piece of your heart and soul, no matter how grateful you are for what you do have. It is a hard thing to come to terms with, you will find that most people with Charcot Marie Tooth are very quiet about their disease, they don’t complain and they frequently try as hard as they can to not have people even know that they have it and that starts for those of us like me that have the most common type, early onset 1a, because we have it when we are in grade school and junior high when kids care about not being different the most and when other kids are brutally cruel in pointing out how different you are. I feel this is something that parents need to pay more attention and teach their kids that it is not cool to be cruel and make fun of kids that are different. This applies to mental capabilities as well as physical ones. Ever since I can remember all I ever wanted was to be “normal”. As a child that was born with a physical disability my definition of normal was someone who could walk without twisting their ankles and falling down, someone who could hold their body weight with their wrists to use the monkey bars and someone who could run, all of the things that I couldn’t do. I went to a grade school which had a strange system for sorting students into classrooms. I guess the idea behind it makes a little bit of sense but they hadn’t really thought it through well enough to realize that while the idea wasn’t totally without logic but by unfortunately putting it into practice it brought out the “dark” side of children by using the method they did for determining which students was placed into each of the three classes needed to have each class be a manageable size for each grade. They gave all of the children a test when they first came to school in the first grade to determine a rough intelligence level for all of the students and split the 90 students into 3 classes of 30 children in each one for the approximate 90 first graders. I think this was the first year this school had done this, I don’t know this for a fact but they kept the same approximately 30 students together in each class as they advanced from first grade through third grade. I was put in the “smart” kids class and was with pretty much the same children for second and third grade as well.  The eventually found out that the “smart” kids were so much more capable of totally disrupting class so much so that they changed the whole system by the time I started 4th grade and separated the 3 biggest “trouble” making boys into 3 different classes and equally distributed the smart, medium smart, and the less smart kids into three classes not differentiated by intelligence. I am sure that their initial reasons for putting the children into classes based on their “raw” IQ was to give each group of children the proper education for their IQ. What they didn’t take into account was that children are pretty smart and they are “assholes” when they figure things like this out. So I had the benefit of being in the “smart” class even though I was physically handicapped so it sort of worked out good for me because the kids all knew that even though I couldn’t run like they could I was very smart so I didn’t get made fun of like possibly I would have been had it not been a known commodity that I was one of the kids from the “smart” class. But true to form as kids will be, as soon as we figured out the classes were arranged in terms of intelligence the kids took to referring to the different classes as the smart, stupid and in between. I am sure the intent of separating us kids by intelligence was to address the different needs of the kids as this was in the early 1960’s and addressing needs of kids based on their differing IQ’s and physical abilities was fairly new territory and I am pretty sure the school was trying to do the right thing but they didn’t know what that was yet, and maybe they still don’t but they at least have come a long ways from what they did when I was in grade school. There were 3 boys that were in my 1st, 2nd and 3rd grade classes that I believe were the reason for the teacher’s reevaluating the way the classes were divided. They were very smart and very busy boys, and disrupting class was their specialty and I think after 3 years of the teacher’s us “smart” kids had they were all driven crazy by those 3 boys antics in their classroom. While the motive on the part of the school administrators might have been a reasonable one but in practice it had a lot of problems, the least of which was “do you really want all the really smart kids in the same class?” When I started 4th grade each one of the “smart ass” boys was in a different class and all the rest of the kids were divided evenly and they started a system of the kids having three different changes of teacher’s in a day for Math, English and Social Studies to address the intelligence Factor. I don’t know what they do know because I have been out of school for a very long time but I suspect they don’t practice separation by intelligence like they did when I was in grade school. So in a way starting out my first three years in grade school by being in the smart class was probably somewhat helpful for my ego but it didn’t lessen my desire to be the “same” in my being able to do physical things. I have always wished I knew what it was like to be able to run, I was always the last kid around the track. When I went to my 20th year high school reunion I had one of the boys I went to first grade through high school with come up to me and tell me how glad he was to see that I seemed to be doing well because he remembered me as always being the slowest kid in class and said he had always thought that the teacher’s were cruel to even make me go out there and run when it was so obvious I had so much physical difficulties in doing so. It kind of made me feel good that he had that empathy for me as a child even if it probably wouldn’t have helped had I known it. The kids didn’t really make fun of me that much, partly I think because we did all go to school together for all those years and they knew me well which I do think makes it harder for kids to be mean if they aren’t anonymous. But I knew I was different and I also knew I was always the last kid to be chosen for anyone’s team and whenever I knew ahead of time that there was going to be one of the games like “dodge ball” that I hated and wasn’t any good at ahead of time I would try to get a note from my mother to say that I had to sit out of PE for the day and if all else failed I would tell my mom I was sick so I could stay home from school and not be humiliated. I turned into a real bookworm, for one thing I did love to read and enjoyed reading all sorts of stories about other people’s lives that always seemed much more exciting than mine and also it was something I could do all by myself, I cultivated a lot of hobbies that could be done alone so that way no one could hurt me. At least no one at school anyway, being molested by my stepfather would come along later on when I was in Junior High.  I became very good at what one CMT person calls “lying” but I call it self-preservation. One thing about this disease that confuses people, and allows those of us that have it to sort of avoid the truth, is that because it mainly affects the extremities our bodies are normal looking from the knees up and no one really notices that your hands are a bit skimpy on muscles unless you are trying to pull yourself up on the “monkey bars” so we can sort of pass for normal if we are really good actors and most of us are. For some reason people with CMT try very hard to hide their disability, well not really for some reason, it is because no one wants to be different or at not different in a bad way and to those of us with weak muscles being different means being physically weak. When I got older it wasn’t so important but as a child it was very important. Still I was a very busy and inquisitive child that didn’t let CMT stop me from doing a lot of things that I now consider myself brave for doing but I think I did them because I did have a curious mind that wasn’t able to totally accept that I was disabled. I took ballet lessons, I tried to learn to play the guitar but my hands were not strong enough to hold the strings down so I changed to piano and then in 6th grade I took up the flute and played it until I quit going to high school after having my second set of operations on my ankles and didn’t have my cast off before school started again for my senior year and I was then old enough to refuse to go with a cast on my leg like I wasn’t able to do when I was only 6. I did graduate by taking a high school equivalency test so I could go to college.

This only describes my physical own disability and not how I feel about myself or disabilities overall, but this was not always the case. I was born with my disability so I have never known anything else, I didn’t become paralyzed in a crash like one of my best friend’s I had for the last 31 years, I didn’t develop MS at the age of 30 like another friend, I didn’t have a stroke at 50 like another friend, I have always been disabled and therefore I don’t know what it is like to have ever of been able, so to speak. All of my life I have always watched people who were able to run with envy, I have never been able to run, I can’t even jog, so watching people who can run causes me to wish I could experience that, but the reality is I never will. But I can walk, and that is a reality that a lot of disabled people have never been able to do and I do appreciate that. I also do believe there is a difference of the perception of the different kinds of disabled people.  I have had many friends, and have many friends, that are disabled for various reasons.  I don’t feel more sorry for myself because I have never been physically able to do the things that people who have become disabled after having lived for ten, fifteen or thirty years of their life, I just haven’t experienced the luxury of having a normal body for twenty, thirty or forty years and I never will. I can’t ever walk up stairs without pulling myself up, I can’t pull my body weight up with my hands and I have never been able to run. I will never experience these things, it doesn’t make my disability any worse than anyone else’s it just means that all disabilities are different. None are better or worse, just that they are all disabling in one way or the other and that includes mental disabilities as well.

I wasn’t miserable when I was in grade school but I was a very shy and timid child but I was bright and did try to experience as many things as I could. I was a good swimmer, I didn’t have to have as strong legs as you do to walk, and I did pretty well in swimming competitions and diving, I didn’t usually win but at least I wasn’t always last.

1969 - Camping In Canada

1969 – Camping In Canada


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