Please give me some advice

Hi, my son is 10 years old, he has high arches, scrunched up toes, walks on his heels rather than heel-toe action. His fingers are clawing too. This was picked up by his swimming coach 2 years ago. Due to my gp's negligence it's taken us this long to see specialists. At first it was thought to be cmt then after the nerve tests it was said it wasn't! He's had mri scan that came back normal. X-Rays show deformities to his feet. Now they are saying that it's cmt again so they are testing his blood. His genetics Dr is saying that he could have a strain of cmt that isn't connected to his nerves! I'm so confused as I can't find the strain she is saying he might have! He is having both feet operated on, on the 21st August this year on both feet. Can someone give me some advice on what to do for his future please? And or what to expect? Thank you xx

Dear lunakitten,

I can understand your concern and confusion. My heart goes out to you. Though I am a volunteer moderator, I do not have CMT, so I will hope that this wonderful group will be able to offer you information and support on this. I'll have a look on the internet and see what I can find.

Wishing you well,

SK

Here is some info on CMT from the National Institute of Health, it explains the types, but I'm not sure if it answers your question. I still have much to learn about this disease.

http://www.ninds.nih.gov/disorders/charcot_marie_tooth/detail_charcot_marie_tooth.htm

My son was diagnosed with CMT1A at the age of 13 after 4 years of misdiagnosis by his gp as well. I completely understand your frustration and we now 1 year into this. From my experience, they try to confirm the most common forms of CMT first since a full blood panel runs in the range of $16,000 and where as the test for just PMP22 duplication (CMT1A) is around $800. So depending on what exactly the test is for, it may come back negative and they may need to run additional blood tests. If the genetics counselor has stated something different, find out exactly what what that is. I was under the impression that CMT was a peripheral nerve disease so I am also interested to hear which CMT does not involve the nerves.

I was not happy with the delay in treatment and decided to take my son to one of the CMT Centers located in Johns Hopkins. http://www.cmtausa.org/index.php?option=com_content&view=articl.... I have found that hospitals focused more on CMT have a different approach than those in our area (proactive versus reactive) so I used our local hospital for day to day stuff as it comes up and Hopkins for the big decisions. The most important thing is to find a doctor and facility that you are confident will give your son the best care possible once you have a diagnosis (or you may want to involve them prior to diagnosis).

No matter what kind of CMT this is, stretching can play an important part in helping your son. It may help to see if they will hook you up with a physical therapist who can design a stretching regimen around your son's needs. In my son's case, this is 20 minutes in the morning and 20 minutes in the evenings and when ever he thinks about it throughout the day. This will not slow down the disease but helps with ambulatory issues (stairs being a huge one) and quality of life for us.

The frustrating thing to accept is that this will do what this will do and no one can tell us exactly how it will progress. We are focusing on what we need to do to help him learn to adapt and live the life he wants to live. Yes there has been some changes (sports really is not an option any longer) but I keep at him to pursue his other interests. As a parent, the one thing I can think to advise is to start thinking about how to handle the weakness in his hands when it comes to school. I have worked with the school since holding a pen/pencil for long amounts of time difficult and he has transitioned to relying heavily on an iPad. While iPads are in use at school, he is allowed to use it for notes instead of being required to write notes. We have some other accommodations in place if he needs them. The important thing was to communicate with the school. Not everyone will be receptive or event competent but by keeping the eye on the ball, you can get through this.

It is important to remember that each case it its own experience and what has happened for my son may or may not happen with yours. My previous paragraph may sound a little dire but if you met my son with a group of other kids, you probably would not know he has this. The pt has helped with is gait and he has adapted in all other facets so he appears to be a regular nearly 15 year old, attitude and all!

ItGal, at what age did your son present with symptoms? Mine started at 2 years old. He is now 3 and just been diagnosed. No specific gene yet. Cmt1a ruled out as from nerve conduction tests it is axonal rather than myelin. Recessive too as nothing in the family (apparently)! I am in Australia and we don’t have any cMT centres. We have amazing doctors, but with much less sufferers, there are much less specialists in the field !

Also, I am traveling to the cmt conference in sept 27, California. Is any one going, or has any one been in previous years?

Wow, thank you all so much. I shall read the information you have given me, and thank you all for your support. I shall keep you all informed as the operation takes place and shall pop on and off in the mean time xx

The first I noticed was around age 9 when I felt his gait was off. His handwriting started declining around that time. But looking back, I feel like he was showing symptoms as early as age 5 or 6. He played soccer and just could not keep up with all the running. We also do not have it in our family as well and the genetic counselor feels like his is a genetic mutation - I went as far as getting tested (I do not have it) and from what I can tell his father did not have it (his father passed away 3 years ago) plus there is no one in his father's family who shows symptoms of neuropathy. I have not gone to the California conference but hope to get there or to the one in Florida.

I would highly encourage you to see if they will recommend PT. In our area, they do not prescribe it but at Hopkins they do. Once we finished with PT (I had to push for it), I found a young man who was getting his Ph.D. in physiology who was a physical trainer and he has created a regimen for my son that is a high intensity/low impact workout focusing on strengthening his core muscles and the muscles we have not lost. This has helped my son with fatigue and walking down stairs. I had to push for the initial PT because I was told that there was nothing that really could be done other than stretching (that was before Hopkins). Each patient will have a different response so I went through several groups before I found someone that "got" it and my son connected with.

I understand your frustration. My son was diagnosed at age 6 through blood tests that he has CMT-1A. Armed with this information, we have been pro-active in trying to address his issues. The most helpful thing, from my perspective, was to keep him mobile and stretched. We visited with a PT who adapted a stretching and strengthening program for him which we still use to this day. My son is now 23 and doing well. Unfortunately, there is nothing you can do to stop the disease but you can do things to help delay the symptoms of the disease. Knowledge is powerful. Once you get a proper diagnosis, arm yourself with information on what muscles/nerves are affected so you can help him as best you can with exercising, stretching, everyday use and activities. Always remember, you are your son's best advocate. Good luck to you and your son.

How are you doing luna? I've been sending some good wishes and prayers your way, I've been doing that for all these wonderful folks with ill children! Kids should never have to suffer more than an occasional skinned knee!