Bloomin’ eck I’m grumpy today.
Facebook is such a pile of…
There’s a Causes page written by someone who has lost a relative due to the rare breathing problems that can occur with HMSNs, in this case CMT. It’s comes across as a bit dramatic, but I do not doubt that it has happened. Unfortunately there are those who really get irate with this, shouting and stomping stating that CMT is not life threatening, and really being, in my view very insensitive.
Anyway, I was prompted to reply, just to put across my distaste at their attitude and to try and strike a more balanced view of things… who the bloody hell do I think I am… balanced my arse!
So here is my response… (some names have been removed) … oh the other gripe, facebork buggered up all my formatting… pillocks!
Please stop shouting. Please have some sensitivity.
XXXXX, it may well be the case that if respiratory problems are recognised, that they can be managed successfully. However, many people come across the ignorance of some doctors who say it doesn’t happen, and therefore respiratory testing is delayed.
I have personal experience of this, so I do find the shouting hurtful, as I’m sure the others do who have also had these problems. I had to fight tooth and nail to get testing done, simply because the doctors didn’t believe that it could happen, or if they did, didn’t believe that I had a problem other than anxiety.
Eventually I was tested, and while it is true that other serious problems were found, asthma and copd, it was also found that I had notable respiratory muscle weakness, not just diaphragmatic weakness but other respiratory muscles too. The respiratory consultant thinks these are significant considering the other diseases, the neurologists couldn’t have cared less. Remember, with HNPP, palsy or paralysis can occur quickly, particularly if the muscles/nerves are physically stressed. Combine that with asthma and there is a dangerous and very unpleasant problem, one I’ve struggled with for 30 years or more.
Ascertainment bias is the real killer here.
Making absolute proclaimations, stating that it does or doesn’t, isn’t helpful. Careful investigative testing is required, not dogma.
I can ‘robustly’ say that some ‘expert’ neurologists need their backsides kicked!
I would like to see simple respiratory tests included for all patients with HMSN at diagnosis, it doesn’t take long, and it might reveal problems, including any other complicating diseases, before they become dangerous and potentially life-threatening.
I’m fairly sure I’ve read Mr. Y’s account of what happened to his daughter, and it was ignorance of CMT and the fact that in some cases it can cause breathing difficulties that was responsible. (Just to clarify, Ignorance by his Doctor not Mr.Y)
Well there it is… snippy git … int I…
What a crazy day… not really, think I meant lazy. Walked to shop again, or should I say staggered with an ataxic gait to the shop. Really breathless again, feeling very exhausted at this moment in time (which is 20.21). Hips not quite so bad today, but right thigh is very sensitive to any touch. So business as usual.