Magazine article about CMT

Well i suffer from CMT and been asked to write an article about it for a disability magazine...
so this is what I have so far!

CMT – The biggest disease no one has heard of

My name is John Hosking and this article is to promote awareness of CMT and show how I have coped with it through my life.

What is CMT?

CMT is the abbreviation of Charcoot-Marie-Tooth. CMT affects 1 in 250,000 people which make it as common as MS, yet few people have heard of CMT.
CMT is a hereditary condition which can affect males and females.
CMT is a degenerative condition that attacks the motor and sensory nerves in the body. It effects how the signals travel to and from the brain. The signals in a CMT sufferer are not as strong as a healthy motor or sensory nerve; this causes the muscles to get a smaller and smaller signal as the condition progresses. This signal loss in motor nerves leads to muscle wasting and many CMT sufferers become very thin due to this, much like me. As this progress’s it makes even everyday tasks more difficult, as I shall describe in the article. In the sensory nerves the signal is slow reaching the brain or may not reach the brain at all, this makes handling hot or sharp items dangerous, as the hands may not send the signal to the brain that the item is hot or sharp.

My life with CMT

Firstly I’m a fighter and tend to struggle on not asking for help and cover up my problems, which is probably something I should not be doing when I’m trying to cope with CMT.
The strain of CMT to which I have seems to affect the motor nerves but not the sensory nerves at the time of writing this article, although this may change in the future.
I was born with CMT in the early 80s when very little was known of CMT. My granddad had a dormant CMT gene, but he suffered polio and this triggered the gene. My mum carries the CMT gene but shows only very small signs of it herself. I was labelled as a quiet or lazy baby as I was not very active and didn’t move around much. I was late learning to crawl and my speech has always been a little slurred.
When I started primary school I had already developed the high foot arch and was lifting my feet very high to walk due to the foot drop. I was at a small school in a village and most of the other children knew me so this was not a problem. In my last year of primary school I had a nerve test on the recommendation of my GP; this showed that I had CMT. Very shortly after this test I was fitted with AFOs, these are leg braces that prevent movement in the ankle. These braces have helped me a lot but they do prevent me doing some things too. I am not able to run or jump in them, which at the moment is not a problem but back in school it did present a few problems. When I started secondary school things changed. Physical Education was the lesson I really disliked and was when I was taunted most about my AFOs. I did take part in P.E. for roughly 6-9 months but I was not able to continue and got a letter from my GP stating I was not fit to take part. I did get picked on quite a bit at secondary school due to my CMT and my physical appearance. I did make some good friends despite this and some still last to this day. I learnt to adapt over the years as it slowing got worse to the stage of the last year in secondary school I was given an electronic word processor to help me as I found holding pens for any length of time difficult and my writing became very messy. Towards the end of secondary school I was given a prediction of how my CMT may progress, this prediction said that I would be in a wheelchair at the age of 20. I’m a fairly positive person and through all the taunting and bulling at school it never really upset me, and I just ignored it. This prediction was not something I wanted to hear at the age of 15. I decided that I was not going to let it happen and really started to use my bicycle a lot more. I would ride it everywhere I went, including to college after I finished at secondary school. The college to which I enrolled was some 14 miles away and I would ride that most mornings unless the weather was bad then I’d use the bus, and the same distance back in the evenings. Riding my bike did slow down the progress in my legs although as of yet there is no known cure for CMT.
During later years at secondary school and my time at college I began to notice that my hands where getting weaker and that I had developed very strange ways to open and close my grasp on objects. By the time I had started college I had lost all motor control of my thumbs and as I type this article I am also without control of my little fingers on both hands. During the time I have been wearing my AFOs I have been unaware of the degeneration of my feet. The AFOs prevent movement of my feet, they are held at a 90 degree angle while the supports are in place. This has caused severe muscle wasting as they are not used at all. I have no control over my feet at all other than very small movement in a few toes with the AFOs removed. Without these AFOs I am pretty much house bound, which makes them vital for me to stay active. The nerve damage in my lower leg and feet are now bad enough that even with my supports on if I was to stand unaided for any period of time I would fall over. The reason behind this is that the nerves are not sending the signal to the brain that my feet are level, so the brain has to guess where my feet are, and it gets it wrong sometimes and I have to catch myself from falling over.
I am also experiencing lower back pains which at times can be bad enough to cause me to pass out; thankfully this is rare at the moment. During the day I have noticed that my lower back is quite tense but the pain is normally when I relax at night in bed. During the day I sometimes get a painful shock in a joint if I move and catch it wrong but I can deal with these incidents. I rely on my bike to get to and from work at the moment as I do not have my car working at this time. I do not qualify for transport help as I have adapted too much on my own to get help and as such I persevere with the bike. My legs are now deteriorating faster than the exercise of my legs can keep up with and this is going to mean I will not be able to ride my bike eventually. Without the bike and without the car means I will find things difficult. Although I am able to walk I do suffer tight chest or pains in chest after a fairly short length. My CMT seems to come in waves rather than a constant deterioration. I may have a good period of 6 to 18 months of little noticeable difference from day to day, and then all of a sudden I may have a 6 to 9 month period when things seem to go down hill quickly. Oddly at the end of every bad patch there seems to be a small improvement just before the calm 6 to 9 months of little change. During the bad stages things can be difficult; I am usually tired a lot of the time during the day but not so tired in the evenings. The back pain I talked about before also does not help me get a good night sleep either. CMT can affect other things in the body as well and this varies depending on the individual. With me, I find it hard to regulate the temperature of my body; not helped by poor blood circulation. Even in the height of the warmest summer my hands have been know to feel like blocks of ice!
The severity of CMT in my hands is very noticeable, if I am asked to pick objects up.
In order to open my grasp I tilt my hand down and to close this grasp I am forced to lift my hand up again. My grip is quite poor and if objects are of a certain size I am unable to release my grasp enough to fully let go, in some situations I have to use my other hand to remove the object from my first hand. This will only get worse with time; it is already causing problems on the bicycle as my braking force is not as strong as it used to be. This muscle wasting in the hand can lead to a clawed hand or curled fingers. This is when the muscles have eroded away completely and only the tendons remain, as these tendons are no longer in use due to the muscle erosion they dry out and shrink. This causes the fingers to slowly curl over until they are fully curled over and no longer able to be moved. This then causes severe problems with simple tasks, like getting dressed. I mentioned before that my thumbs had lost all motor control as well as its muscles but as of yet they have not begun to curl over. My little finger on my right hand has not yet lost all its motor control but it has begun to curl over, it can be straightened with my other hand but always returns to a slightly curved position afterwards.
My mum has recently noticed in my hands which I had not that my little fingers and the finger next to them have developed a small shake to them sometimes.
Due to the severity of my CMT I have problems making my own hot meals; I rely on my parents for these. A simple meal like bowl of soup is difficult as I am not able to use a tin opener to open the tin. My hand grip is poor and I find lifting heavy object like pans or kettles very difficult. As my hands worsen I am only going to find things more and more difficult.
I am experiencing an increase in deterioration in my legs, riding my bicycle is slowly getting harder as I loose the muscle mass in my legs. I am beginning to experience craps or pains in my upper legs while riding my bike home from work which is all up hill.
Also after riding home I have sometimes experienced a tight chest or slight pains in the chest.

John,

Thank you so much for posting - I certainly haven't heard of CMT. What a great testimony to your strength - you are indeed a fighter.

S xx

thank you!

indeed i am a fighter!

yes i have low moments but i have great friends and family