Does it plateau?

[sheerkhan]

Hi,

I have CMT, no idea what type, but I've had it since I was 2, I'm now 37. It's a lot more severe than the CMT I'm used to reading about online, and so I'm concerned where it's going to end. I haven't been able to walk since age 21, and I rely on my arms to do everything (transfers, dressing, washing, etc).

I wouldn't mind if my CMT paused, it feels like it's not gotten worse in the past decade, but I'm worried it might. Is it known to completely waste arm strength away? By that I mean biceps/triceps/shoulders.. or in other words, does it stop a person's ability to lift things/their own weight?

OK, not sure my title even makes sense but I'm so worried about how it's going to affect my future. I've booked a neurologist's appointment but that's going to be in a few months time. Does anyone have a complete list of possible symptoms, even rare ones?

Thanks

[Amanita]

Hi, Sheerkhan,

I am so sorry you have such a severe version of CMT.

Your title makes perfect sense. For many of us CMT can appear not to change for a period -sometimes several years - before taking a downward step. I think this is because although it is actually very gradually worsening all the time we notice when something changes - from just about managing a particular activity - to not managing.

I think it would be useful for you to get your neurologist - when you see him/her - to try find out what type of CMT you have (done by means of taking a blood sample and sending it to a specialist lab). This is expensive, and not guaranteed to produce a result unless it is caused by a fault in one of the genes already known to be implicated. Unfortunately the results can take several months to come through, but could be useful if a type can be pinpointed. To discover whether it is the axons ("electric wires") part of the nerves or their myelin sheaths ("insulation" ) that are primarily at fault there are nerve conductivity studies.

Did you inherit it from a parent or does it seem to be a spontaneous mutation?

One of the problems with CMT is that even two siblings with the same type can experience different symptoms and different degrees of severity.

Are you sure it would be really helpful to know in advance all of the the possible symptoms of CMT ? I can understand that you are very concerned and fearful for your future, but would you not be increasing your worry - while there is the possibility/hope that you will not have to endure all of them ? Having said that there are some posts here from people with severe CMT, and there is a certain amount of information - not just about mild to average (if there is such a thing) versions - on the internet.

I hope you will get some useful answers from your neurologist, and that you have supportive people around you.

[gunner]

Hi marie was reading that you say you have had cmt since you were 2 but cmt is hereditary caused by faulty genes so I'm letting you know that you've had it since u were born u don't catch it u are born with it. And yes depending on what type u have it will usually cause muscle waste and poss nerve damage. U may also get a body tremor and in time maybe diabetes and depression. But u need to do as much exercise as u can and keep Urr weight and diet under control this may help but as we cmtrs know their is no current cure. It also usually gets worse when u hit Urr thirties. Remember it's a debilitating degenerative disease that will only get worse with time. So sorry to hit u with this but that's what cmt is and does but everyone is affected differently.

[sheerkhan]

Hi, thanks for replying. I guess it's no good worrying about what might happen. My first diagnosis was MD and docs said I'd be dead in my 20's, but that didn't happen.

Like you say, CMT affects people differently and progresses in an unpredictable manner. I vaguely recall from.. oh it must be 27 years back.. my neurologist at the time was showing my parents a drawing saying that neither of them had CMT but both carried the faulty dna and unfortunately I got the same faulty bit from both of them. I guess this means I have the recessive type of CMT. Also my doc said something about Dejerine-sottas and hypomyelenating something or the other.. polyneuropathy. He wasn't 100% though. If that's what I have, is it likely to make me non-functional any time soon? By that I mean, does that condition usually kill off the arms? Also... is it common for it to cause other functions to fail.. digestive, reproductive, etc? I can't find anything definitive.

[CMTSECRETS in reply to sheerkhan]

Sheerkah,

For you to question yourself those you are listening to your body and it’s giving you the answer.

Cmt is a neurological disorder disease starts manifesting visually in feet and legs the nerves are damaged and progressively die out in the peripheral neuropathy system the muscles will be affected right away with loss of muscles and strength it gets worse it doesn’t stop its progresses worsening abilities to do everyday simple tasks. Don’t overdo yourself don’t overwork . Although exercise and losing weight is the advice from neurologists it will reduce your strength capabilities noticeably, the muscles could getting more damaged and wasted because the nerves aren’t helping the muscles because they are damaged themselves.

[MommaWheels]

Sheerkan,

Thank you for writing. My situation is similar to yours and it has affected me at very early age and I am now 41 and use a wheelchair for my mobility. Like you, I use my arms and hands for everything. Because I use them for everything I have found that there has been some increased muscle weakness. It is difficult however to tell if that is because of the CMT or because I use them so much. Some of the trouble I have with my arms and shoulder are typical of people who use wheelchairs for a long period of time.

There are few of us that have it as severe and there is much less information and research about people in our situation. I have been genetically tested and my results came back inconclusive but they believe that I may have type 4 Because of the severity.

I have seen my CMT progress over The years but it’s definitely true what they say about everyone’s progression goes at a different pace. I highly recommend you see a neurologist and ask for referral to a physical therapist who may be able to give you exercises and strategies for keeping your arm strength up for as long as you can. It’s important to listen to your body and to try to find the balance between activity and rest. I work full-time and have an active 12-year-old boy so i’m not so good about getting myself the rest I need but sometimes you just have to give your arms break. Good luck with everything and post again with any more questions.

[MommaWheels in reply to MommaWheels]

One more thing. With a severe type of CMT you can have issues with respiratory but I’ve read of no issues with digestive or reproductive systems. If you’re experiencing digestive issues they may be due to the fact that you were unable to walk. For those of us that use wheelchairs we can have issues with our digestive system because things are not getting a chance to stretch out or move around the same way as somebody who is walking. I found that my body often has similarities to someone with a spinal cord injury although I clearly have CMT. I know it can be frustrating to find answers but I urge you to keep up your search and to remain. You are still young and you’ve got a lot of life ahead of you!

[CMTSECRETS in reply to MommaWheels]

I have digestive respiratory mobility swallowing speech heart problems and I’m in my thirties. CMT neurologists apparently say it only affects the peripheral nerves not the central nervous system or the cranial nerves, not in my case..

[rgeyesl]

Hi. I have CMT 1a.. I had a sister with CMT too who was 11years old than I she is dead now! She had a problem with dairy product I am gluten free! Physio said my nerves are thinker at both of spine so this will affect digestion. So I would try changing diet. I am still walking and excise daily. But I have paralised diaphragm ventilation (all Night on machine. ) be positive and fight anyway you can!! CMT will be cured soon..

Rose