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MG crisis

alice
389 posts
Jun 30, 2009
7:12 AM 		a few months ago, when I was trying to make sense of this illness, by reading, learning from other patients and using my own clinical skills and common sense, I asked how crisis is defined, because I was very confused about it. (as I was at the time, about many other things regarding this illness).

recently, this topic was raised again.

and I think that now I can define it better and also in a way that might help others.

first, I am not sure that the exact definition of crisis is that improtant, as at least in the literature that I have seen there is no concensus about it. some would define it by parameters of respiratory muscle stregth, others would only define it in patients that require respiratory support (and with the introduction of non-invasive ventilation this has become more problematic, as obviously it is used more readily), and others require evidence of abnormal gas exchange.

the indications for initiation of respiratory support also vary, some would wait for clear evidence of respiratory failure (which again is not clearly defined) and others will intubate "prophylactically" once there is evidence that the patient has significant and progressive fatigue as would be evident by the MVV or other parameters. the indications for respiratory support have again become a bit more lax since the introduction (unfortunately not widely used yet) of non-invasive support, which as opposed to intubation is non invasive and carries very little risk other then some discomfort, and in a significant percent of patients that are compliant with it, and able to tolerate it, (at least in a few small series) has been able to replace invasive respiratory support all together.

in retrospect I realise that I personally, was extremly fortunate that during my first crisis (when I knew nearly nothing myself), I was taken care of by an excellent anasthesiologist who was both not a big "fan" of intubation and second-very old fashioned and used neostigmine, just like in the days of osserman, which is not used much any more. as well as strated plasmapharesis without delay, and "protected" me from all those that considered intubation, every time I did somewhat worse. I am also quite convinced that his very reassuring and confident approach, helped in my relatively rapid recovery, from that episode. ( I was back to work within a few weeks).


but back to the original topic. as I said, I do not think that the exact definition of crisis is that improtant, as only a thin line (again depending on the definition you use) differentiates between a severe exacerbation of MG, and a "true" crisis, and what differece does it really make? either way it requires emergent intervention to avoid further deterioration.

so what are signs that a patient should recongnize and know that he/she require emergent help, and can't just rest, take another mestinon and wait for things to improve on their own? or in other words, what are the signs that one's life may be in danger, as opposed to just feeling lousy, and not being able to do much?

well, the only thing that can lead to death is when you can't breath properly, and in order to be able to breath properly, you need two things- an open airway, and functioning respiratory muscles. if one or both fail, your breathing will be compromised to various extents.

your airway is kept open by a complex apparatus, but it requires that your laryngeal muscles will be strong enough.
if those become weak, then the airway will not be as open as it should, and compromise your ability to breath, many times leading to a chocking sensation, as if something is stuck in your throat. this does not mean that you can't breath at all, it is just harder for you to do so, and you need to use more force to overcome this resistance.

this will invariably be accompnied by swallowing difficulties, to the extent that you may find it hard to swallow your own saliva, as those muscles are invovled in the swallowing process as well.

if on top of that you also have significant weakness of your respiratory muscles as well, you will have a significant difficulty to get air in and out of your lungs, which you can experience in various ways. this of course will become more pronounced when you exert yourself and increase the amount of oxygen that your body needs, and the amount of carbon dioxide that it produces. as your muscles become weaker the amount of "excertion" that you will need to do in ordr to reach decompansation may be no more then moving your arm in bed, or trying to talk a few words.

if you are given non-ivasive respiratory support, and continue using anti-choline esterase medications (such as mestinon, or preferably neostigmine), there is no reason why you would not have transient improvement, when your respiratory muscles are given proper rest, and possibly worsening again, when the medications wear off, or you exert yourself a bit more then you should.

also, if you avoid any oral intake, when you are feeling that way, you will significantly decrease the risk of aspiration (also the result of the weakened muscles in your throat), and also give those muscles some rest. even drooling (which is not very nice, I agree), is better then trying to force yourself to swallow your saliva, putting an extra burden on your muscles and avoiding the opening of the airway.

any degree of weakness of the respiratory muscles can be overcome by non invasive respiratory support. only if the weakness of the lanryngeal muscles becomes severe enough to occlude the airway, or if there is no improvement at all in the respiratory muscle weakness despite all those measures, does intubation need to be done.

there are many reasons to try and avoid intubation, other then it is not very pleasant. first, the risk of infections is significantly increased in an intubated patient as opposed to one that is not. second- it is not rare that extuabation is relatively hard to achieve in patients with underlying neuromuscular disorders, and not all respond to immunosupressive treatment rapidly enough.

and last but not least, the management will vary tremendously between those that have a good understanding of the respiratory manifestations of neuromuscular disease and those that do not, and it is advisable/ and even impermative if at all possible to reach a hospital in which there is someone that has such understanding as lack of such understanding and experience can lead to both under ( failure to recongnize the severity of the condition) and over ( un-neccassary intubation) treatment.

as always, I say that although I am a physician, my understanding may not be totally acurate, and may lack something, as I am not a pulmonolist nor an expert in neuromuscluar disease, and had to gradually learn this on my own, with the help of some consultants, with very minimal background in either of those fields, and this is a very complex issue that even many pulmonologists don't quite understand.

yet, as at least for me gaining a better understanding of this illness,and especially the more frightening aspects of it, has been of help, I thought it might be of help to others as well. and as I never recieved any clear answers regarding those questions, and felt that it would have been very helpful if I did, I thought that others may feel the same.

and if any of you think that some of what I say is wrong, from your own experience or from what you have been told, I will be glad if you correct me, as I may possibly have some missconceptions, and will be glad to know more, which of course will be of help to me in dealing with this at times quite frustrating situation.

alice
Debbie210
3 posts
Jun 30, 2009
7:56 AM 		Dear Alice

You post couldn't be more specific to my current situation, thank you for taking the time writing it.
I suffer with Asthma as well as MG. I have recently been taking Mestinon which is a god send, but sometimes I forget to take my 2nd dose and I can start to feel my breathing taking a downturn. I have also noticed a numbness between my eyes, not sure if this is related? Anyway, I just wanted to know why a Crisis occurs even when taking medication, given that anyone who suffers from MG can be affected by a Crisis, which is a worrying thought. Is there anything you can recommend (having had experience of a crisis) as a preventative, other than taking the meds, for example do you know of hayfever causing MG issues?

Thanks
Debbie
alice
391 posts
Jun 30, 2009
11:41 AM 		Dear Debbie,

I truly wish that I knew.

if there is one thing that I was totally unable to find any answer to it is this question. at least in my case, I could not figure out any thing that would predictably make my condition worse or better. and believe me I have been trying to do that for the last four years!!! this was one of the most frustrating things for me, and it took me a long time to come to terms with the fact that there is jsut no way I can know.

obviously exertion always does, but some days, going from one room to the other will make me crash, another day not even that, and another I could actually walk 100 meters without becoming severely short of breath.

even heat, which in general makes my condition worse, will sometimes hardly have any significant effect, whereas on another day will totally wipe me out.

emotional stress which is supposed to make MG worse, will actually make mine better sometimes, have no effect or worse, with no predictable pattern, at least not a very clear one that I could use. ( eg having an arguement with my husband every morning, just joking).

although during a severe exacerbation/crisis, stress should be best avoided as it will increase your heart rate and oxygen demand and lead to a viscious cycle of increase shortness of breath/ increased stress/ incereased shortness of breath.

as I could not find any thing to prevent worsening of my illness, I chose to do my best to be well prepared for those events, so that they will be least harmful and with a good chance of fast and relatively non-eventful recovery. so far, I have mostly suceeded in doing that, although I have to admit that I have had times when I was on the verge of not. but, I guess it is better to be on the verge from this side, then from the other side.

and the point I was trying to make is that recongnized in a timely manner and properly managed, a crisis need not be so traumatic of worrisome, as people think, at least in many cases.

as to your question regarding hay fever, I would think that it could exacrbate any autoimmune disease, so MG included, I would also think that having a combination of asthma and MG, could be quite tricky. where you told that mestinon could lead to bronchspasm? on the bright side steroids (if you respond to them) could take care of both.

I would think that you need a good pulmonologist, who is familiar not only with asthma, but also with MG to be involved in your care, and a good communication between him and your neuro would be benficial both for your management and to give you confidence.

hope this is of any help.

alice.