I have CMT 1a andhave always been told it has zero effect on longevity, however recent articles in the newspaper suggest it can effect the phrenic nerve that controls the diaphram.
If that is the case surely it can lead to breathing difficulties and ultimately death?
Hi there
We always say that CMT is non-life threatening. We have members who are well into their 80s and 90s - my own auntie died at 99 and a half!
I'm interested that there have been articles in the newspapers recently - can you let me know which ones? Just as a matter of interest.
You're right in that, in very rare cases where people are severely affected, the phrenic nerve can be affected, and yes, this does control the diaphragm. The problems this causes is usually carbon dioxide retention at night - during the day, gravity helps the diaphragm work, but when lying flatter at night, this doesn't happen. Carbon dioxide retention leads to severe headaches on waking in the morning.
But the good news, and the main reason why this still is not life-limiting, is this can be managed. There are certain drugs that can help, and when it becomes more advanced, a Bi-pap ventilator at night assists the breathing to keep any issues at bay. One of our Trustees has used a Bi-pap for about five years, at night only, and has no issues with this at all.
I know there is a small CMT organisation in the States whose mission is to correct the "wrong" view of the rest of us who state that CMT is not life limiting. The daughter of the founder died - as a result of CMT breathing difficulties, he says. BUT, there were other factors - yes, she had phrenic nerve involvement, but she visited Denver Colorado (high altitude) and then got pneumonia. So what killed her, the CMT, the altitude or the pneumonia? Perhaps it was a combination of all three, but to blame CMT solely for this person's death is misleading, I believe.
So, to summarise - phrenic nerve involvement is rare. Most people with CMT go through their lives without any breathing difficulties and therefore, I am still confident in saying "CMT is NOT life limiting"
Karen
PS - and you may also see on various CMT Facebook pages and on the internet, that CMT causes heart problems - their theory being that the heart is a muscle, surely it's affected like all others. That is utter rubbish - I have it on authority from Professor Mary Reilly! The heart is a highly specialised form of muscle, that self-activates without any input from the nervous system and therefore is NEVER affected by CMT. Other internal organs, whilst they are still attached to the peripheral nervous system, are protected from CMT by a genetic modifer for those nerves that stops the CMT from having any effect.
Nice one ! Karen ! as is usual !
Karen has explanations for everything.
CMT brainwasher.
I’m sorry I cannot help myself, so much bullsh*t gets to me.
Ps: I respect you Karen just not what you been rehearsed to pass on.
Karen, Thanks for the detailed response which is much appreciated, I will try and find the article I read and will let you know.
Proff Reilly seems to be the only person in the NHS who really understands CMT, our local NHS Trust is totally useless and has virtually zero support services.
Although I have three sons only one has been tested for CMT which I think is a disgrace and only highlights the limited worth of Primary Care Providers i.e G.P's .
Mary Reilly is fantastic, but there are others around the country who are equally good. Unfortunately they are few and far between and finding them can be a bit of a mission,.
As far as testing your sons is concerned, there are ethical issues around testing children (are they?) if they do not have any symptoms... So it may not be a lack of care, but that, maybe? Just a thought!
Karen
All the very best, Cmt symptoms will manifest themselves within its progression, sooner or later it will be diagnosed, Let’s HOPE NOT that would be better news. I recommend to study research and write down everything that’s happening with Cmt , being informed & educated about Cmt-Neurology is best .
Unfortunately there are many practitioners out there still have no idea what CMT is i went to podiatry the other week and ended up having a row because she said my hammer toes were nothing to do with my CMT even though I have it on authority that it is as is the high instep
I wonder how easy it will be to get access to the Pleodrugs when they become available when most G.P's don't know what CMT is?
If and when they go through human trials successfully, I would hope that access will be easy - she says, crossing her fingers.
However, we are a very long way from that yet, so lets cross that bridge when we come to it!
Karen
I disagree. I say plan ahead. Don’t wait
I have my phrenic nerves affected by CMT, and it does affect your life, and even though I am on a Bipap machine I still stop breathing throughout the night, if I die from respiratory arrest or a heart attack because believe it or not your heart can be affected by the breathing issues , then of course it was caused by CMT, what you said Karen is so patronising to those of us who have this problem, of course I can die from this, but do i worry about it, no, but please stop pretending it doesn't kill, because it can, even if only a few of us..
I'm sorry that you felt my comments were patronising - all I can do is pass on the knowledge I have. Of course, there are always going to be exceptions to every rule. However, I would say if you're not breathing adequately at night, perhaps your BiPap needs adjusting? Do you have regularly checkups with your respiratory team to make sure that all is working properly?
Karen you wondering if her bipap machine needs adjusting... telling her to see her respiratory team .. you know CMT IS LIFE THREATENING SO PLEASE DONT PATRONISE US!
I refused bipap I went to A&E .. funny how they know it’s life threatening and CMT has “a” life expectancy.. at least mine has.. my own neurologists are panicking that I’m refusing help, I’m going to prove then wrong for hiding that we CAN DIE FROM CMT! I need oxygen and cpap machine my respiratory system is struggling and it has been for a while that now I have Heart failure! Strange part they couldn’t answer why I had to be intubed for a weak because my respiratory system was down other than CMT !
I regularly see my respiratory specialist, you treat people as if they are idiots by saying such things, that is what is patronising, they upped the air pressure and i stopped breathing more (yes that can happen) because of the weakness of my diaphragm the air went into my stomach and caused problems, did you also know that CMT can cause arrhythmias with some people who have CMT, especially those with the Bi-lateral phrenic nerve affected. Yes there are lots of things they don't know yet and are just discovering... and if you have an attitude that it can't be happening or isn't CMT, then you limit research that could actually help people who have it severely.
Dear CMTSECRETS,
I totally agree with what YOUR saying about CMT life threatening my mother passed about at 62 breathing problems and my dad died at 47 massive heart attack
ABSOLUTELY AGREE WITH YOU ! Karen makes Cmt sound like a typical physical disease that can be controlled and managed ! I respect Karen but not what she been told to say !
Grannymaz im in the same situation or similar as yourself! I’m young and Slim and by god what I found from the A&E Hospital doctors it’s Tests and exams is an eye opener.
GrannyMz you’ll be told that’s nothing to do with cmt or that you’re very old everyone to distract from relating to cmt !
Even when you’re diagnosed with phrenic nerve palsy after cmt heart failure after swallowing after hoarse voice vocal cord etc.. this brainwashing should stop although cmt affects diferent ways but when affects respiratory system through phrenic palsy heart failure due to its nerves muscles weakened and dying IT IS LIFE THREATENING IT IS THE CMT IT IS A NEUROLOGICAL DISEASE ITS DOESNT ONLY AFFECTS FEET HANDS !
Thanks CMTSECRETS, yes I am having heart stuff done now because though i have had palpitations for a long time suddenly I have SVTs, and yes it affects my whole body now (I have had tests done) it affects my voice yes and my hearing, it's almost like some people want to "protect CMT" as if it's a person from the truth, and that just stops any research or Drs from listening to us, makes me mad <3
For those suffering more than just the hands and feet problems with CMT . -
I have CMT and am at an advanced stage of the disease. I am having breathing problems because of the CMT and the weakness of the diaphragm. My neurologist told me that, YES, indeed, although very few, but people do die of CMT.
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