Neuromuscular Disease Causing Acute Respiratory Failure
http://www.rcjournal.com/contents/09.06/09.06.1016.pdf

IF you think your medical staff isn't adequately "informed" about potential respiratory problems in MG, you might want to point out this article.

When they WON'T "listen" to patients, they WILL often "listen" to other doctors.

Jana, That's nice but I think you have me confused with the firefighters, police, national guard and everyone else who's helping out after that B#$$@ Sandy destroyed everything. They're the real heroes.

I'm also very sorry you've known 3 MGers who have died. Could it have been avoided or could they have been helped?

wild_cat, I thought of something else. Do you know your normal pulse? When chest wall muscles get weaker due to MG, your oxygen saturation (how much oxygen is getting to your tissues) goes down. Then the heart pumps faster in an attempt to get you more oxygen, thereby raising your pulse. So if your pulse is going higher just sitting around, that's the "poor man's/woman's" way to see if you are needing oxygen more than usual.

There are other reasons why a pulse goes up but, in this context, that's what happens to someone with MG whose breathing is getting worse.

Are you doing okay?

Annie

Good PDF, Jana!

that's interesting Annie.. when my symptoms really started about 18 or so months ago, my heart rate went from my usual resting in the 60's to the 140's at rest, even when sleeping!!! It's almost always above 100 when I am feeling especially weak.. so happy now it's back to the 70's.. it's crazy- never heard it being related to MG, so that's good to hear!

Jana,

I went over it briefly. Overall it is a very good review, but it is outdated.
There has been significant progress in non-invasive ventiliation over the last 6 years. (fortunately for me, fully paralleling the deterioration in my respiratory function ).

So, there is probably no longer any need for early intubation, rather early institution of non-invasive respiratory support. In fact in makes very little sense to intubate a patient with an illness causing intermittent respiratory failure, when this is now avoided in many patients with ALS.

I personally find it ridiculous that patients with sleep apnea can easily receive proper non-invasive respiratory support, whereas patients with myasthenia have to jump through a zilion hoops for that. (unless they are fortunate enough to also have sleep apnea).

You are right that you can die from untreated respiratory complications of myasthenia.

When one of the technicians reviewed the data from my respirator card one time, he said-well, you only had 15 minutes over the last week in which you were unable to breath on your own.

I said to him- that is true, but what do you think would have happened if I didn't have my respirator with me during those 15 minutes? you don't need more than 4 minutes of not breathing on your own to develop hypoxic brain damage.

It was also true that on that same day, a few hours later, I was able to go to work. Had I not had my own respirator, I would have either died at home or been intubated in the ICU for who knows how long.

Hi all.

Thanks so much for your responses.

We rang the hospital last night to see if I could get readmitted to the same ward (acute medical assessment). Unfortunately they could only readmit me through the out of hours emergency Dr, and as my symptoms are worst when I'm trying to sleep we felt they probably wouldn't find a reason to send me. It was about trying to make a decision between a two hour car journey, which would potentially make me much worse, or see how it goes over night.

We opted for the latter. Fortunately although I had two episodes of waking up breathless it wasn't as bad as the previous nights. I think I may have been sitting too upright in bed before and the angle for me is very sensitive. However, last night and this morning the tense band around my ribs is spreading to the middle and the yogic breathing exercises I do every morning are markedly different. My cough is extremely weak and I cannot sniff properly. I certainly can't count to twenty out loud on one breath.

As my main breathing difficulties are occurring while I'm trying to sleep I wonder whether the hospital staff got an accurate picture. The did a pulse ox which was absolutely fine. However, this was after having been awake for quite some time. I am getting to the point now where I would sooner sit awake all night than risk going to sleep! But of course that won't help matters either. I'm still waiting to hear from my GP. We tried to ring him yesterday but he wasn't there.

Annie – thanks so much for your honest advice. I didn't have any of the tests you mentioned and the scan wasn't with iodine, fortunately. They gave me a sealed envelope report to give to my GP immediately and I just got a sheet of paper which listed my symptoms and said I'd been in hospital. My blood pressure certainly is elevated during breathlessness and I often get palpitations at rest but as it is suspected I also have carotid sinus hypersensitivity I had attributed these symptoms to that. As you can imagine, with a potential mixture of neuro (MG) and cardiovascular (CSH) causes it is difficult to separate out what is causing which symptoms.

Jana- thanks so much for the article and looking up the compatibility of CSH and ephedrine. Having no clear diagnosis makes my GP very reluctant to try any medication until we are certain what the root cause is. I've recently had bad exacerbations from taking some fairly innocent medications (cinnarizine, aspirin).

Bny806 – yes, I have been getting horrible headaches, which seem to go hand in hand with the elevated blood pressure. It creates an enormous pressure behind my eyes which makes them feels as if they will burst out of my head. It's worse when I move around and it makes my nose bleed. I'd attributed that to the CSH.

I want to put my trust in the Drs I saw at the hospital and trust that my GP is arranging the referrals and will come back to me as soon as he can. However, I am in a difficult situation and I'm not sure how much longer I can continue this way. It doesn't seem very sensible. Part of my hesitation about pushing further is that it has previously been suggested my symptoms were psychological and I feel that pushing for a diagnosis or making suggestions of possible treatments only reinforces the idea that I'm suffering from some form of psychological disorder.

Thanks again for all your advice and support.

wild_cat

Support, yes! Advice - not sure!

Not pushing for a diagnosis or making suggestions of possible treatments in my case reinforced that I was not suffering from a neuromuscular disorder.

I regret not having been more assertive at a time when I was severely out of breath pulling a jumper over my head or unable to finish a sentence with 5 words in it.

I wonder exactly what kinds of muscle weakness psychosomatic disorders present with anyway and if they are progressive and fluctuating in nature?????

I've often wondered which psychosomatic disorders can be triggered by neck rotation and postural changes, but so far I've never been able to find anyone who can explain this to me...

Headaches upon awakening can be a sign of CO2 buildup (sleep apnea- not breathing well enough at night).. that's a sign you need some help- among many other signs sounds like.. Do you live in the U.S??

It just sounds ridiculous the way they are going about helping you, or not. I Hear ya, so many dr's have told me they think a lot of it is anxiety etc.. ha.. I had no anxiety when all of this started, but when you can't breathe, carry your kids, walk, then yea, your gonna be anxious.. and yes that can make the symptoms seem worse, however that does NOT mean that the anxiety is causing the initial symptoms.. I was also hesitant to push further at first, after being told it could be anxiety, you start thinking maybe it is.. however when they performed the pulmonary function tests and they were in fact low, and other tests started coming back abnormal, it wasn't all in my head afterall (as I told them - I wish it was, cause afterall, that would be the best case scenerio right!?) Waking up with a headache and all those other symptoms are not a psychiatric issue!
I really hope they listen to you and get you the help ASAP that you need.. Sounds like someone may need to scare them - and rip them a new one to get them to take you seriously.. so frustrating, I'm sorry!

I am so sad now. I have just been to an emergency appointment with a GP (not my regular one, he wasn't free) and they now have the report from the hospital, which basically says I should see a specialist in 'functional' symptoms. I cannot complete a sentence as I am so out of breath, and I told them if they didn't sort something out they could have a dead patient on their hands. I can't help being really upset by this and I have no idea what to do. I am waiting for them to ring me now.

One thing which confuses me is they say my neurological exam was normal, yet I cannot look to the right with my eyes or upwards. My eyes ache horribly and after a few seconds give out and close and my head drops, or else my right eye just refuses to look in that direction and rolls off upwards. I can't focus between one and object and another. I can't hold my arms out in front of me. I failed on nearly all of the strength resistance tests (can't hold fingers, wrists, ankles, arms or legs against any resistance). I became very breathless on standing and my voice very slurred and difficulty controlling the pitch. My left arm is so weak I failed to reach out and touch the neurologist's finger in the nose to finger test. Is this really a normal neurological exam? Are these really functional symptoms?

I had three neurological exams on Saturday and the last, and certainly my best, was with the neurologist. However, he is actually a brain surgeon and not actually a sepcialist in MG, and he says my symptoms don't fit an MG pattern. The GP now said there is somebody living with MG nearby and my symptoms aren't like hers. Are these Drs being really ignorant or am I truly the nutter they say I am?

so sorry to hear this.. ... seriously, has anyone even thought to do pulmonary function testing??? After all the neuro exams I have had performed on me, I can honestly say most of them are a joke as well.. I had one dr even say, since I wasnt' dragging a limb, he was sure i'd be ok ... ha.. If I can prevent dragging a limb, that would be nice!

Many of those neuro exams are so subjective too, it's frustrating.. whatever the cause is, if it sounds like MG or not to them, doing objective testing would be good - the PFT's would help show them, along with a sleep study sounds like.

I'm so sorry you have to go through this.. it's hard to not only feel bad physically, but not feel like your getting the help you need.