In 2006 my Mom was in hospital for a minor operation. She had been DX with MG just a week before but in my opinion she had it sometime before. She had neck and swallowing issues at least a year before. We took her to several neuro's and the most we got was she was de-conditioned. Anyway when they put her under for surgery when she came out she couldn't ever breathe on her own and we lost her. So I would say she died from MG but her DC says "old age"
Mike

Before there was the ability to provide respiratory support the death rate from MG was about 30%.
In patients who had significant respiratory symptoms it was nearly 100%.
I believe this is also the source of the scary name of myasthenic crisis.
At that time it made no difference if you recognized early signs of it, because there was nothing you could do anyhow. Physicians could just hope that their specific patients belonged to the 70% who would do reasonably well.
The word " crisis" was (I believe) coined by Hippocrates to describe pneumonia before the era of antibiotics. All a physician could do was sit next to the patient, hold their hand and hope that they will recover from it.

This changed dramatically to less than 5%, once there was proper respiratory support using mechanical ventilation. (it also led to many needless intubations because of the fear of rapid respiratory deterioration).

Assuming that MG remained the same disease, it is reasonable to assume that patients with significant respiratory symptoms, who are left untreated would die just like they did before.

This also means that if a patient with MG dies because of respiratory failure, it is quite likely that he/she did not receive proper treatment which could have saved their lives.

This doesn't mean that if a patient with significant respiratory symptoms doesn't have severe and rapid deterioration, it is not serious and can't be due to his illness. The respiratory symptoms of MG are fluctuative (just like all other MG symptoms) and patients can have continuous worsening and improvement. They can be on the verge of requiring respiratory support and then recovering with rest and medications. There is no need to wait for that one time in which they will have more persistent respiratory failure.

If the cause of respiratory problems such as shortness of breath on exertion is MG, what can a pulmonologist do other than the same treatments that are already being done for the MG? Does it make any difference?

Thatīs a good question. Donīt know but Iīm hoping that an astute pulmonologist would be able to assist in the diagnostic process and help propel the other doctors into an appropriate course of action for treatment and also provide base readings for measuring fluctuating respiratory function. The breath count only does so much. I know of a few seronegatives who are hopeful for an MG diagnosis via a pulmonologist.

If you have a good neurologist and you only have mild respiratory symptoms, and are receiving adequate treatment, you probably do not need a pulmonologist. (although it is probably good to have your baseline levels of respiratory tests).

If any of the above is not true, than a good pulmonologist can probably save your life.

IMHO, anyone who has BULBAR symptoms needs to have a pulmonologist "on their team". The potential for a respiratory crisis is ALWAYS there. Waiting UNTIL the crisis means that your fate is in the hands of whomever is on call at the hospital ER.........a chance I'd rather not take.

Of course I realize THIS is how it WORKS in the States (ER docs, etc.). Alice may experience something totally different where she lives.

Jana

Do you know what tests a pulmonologist would do with bulbar symptoms related to MG rather than those for pathological swallowing problems?
I know having a grain of rice lodged for 45 minutes is not normal and nor is choking on saliva/a surprise gush of juice from chewing a piece of fruit. But I couldnīt guarantee presenting with those symptoms at the exact time of an appointment for inspection since they come and they go. That could put me off going. (Here Iīm talking about initial appointment and not ongoing care)

Anacrusis

PS....

I havent' been to a pulmonologist yet, as the Neuromuscular clinic does PFTs (pulmonary function tests) at the clinic.. They do a FVC (forced vital capacity, i believe is what it stands for).. I like that they do that each appointment, so that I have concrete numbers that I can track... When I feel like I can't breathe, usually they are low.. however my pulse ox remains good..
Im sure an actual pulmonologist would do more tests even - PFT's, and something called a snif test or something - I believe it tests your diaphram strength?
Of course they have swallowing studies - barium swallowing I believe to test that part of it..

Its so anxiety provoking not to be able to breathe, so getting those numbers is really useful I think.. mentally too!

Ok, I hope I get this RIGHT.........if I don't, there are several people here who can add to/fix the info I'm gonna give!!

There are several tests to measure the muscle strength of an MGer:
VC -- Vital Capacity
NIF -- Negative Inspiratory Force
MIP -- Maximal Inspiratory Pressure

You need to get at least one of these tests done as a "baseline" on a "good" day. That way, IF/WHEN you have respiratory problems, you (and your medical team) will have something to compare it to.

WHEN you are having trouble breathing, O2 saturation is NOT a good measurement for MGers. You know that finger "thingy" they put on you? An MGer can appear to be FINE as far as their oxygen levels go -- and ACTUALLY be about to go into crisis. ABG (Arterial Blood Gas) is FAR more accurate for us -- it shows CO2 (Carbon Di-Oxide) levels which build up because our muscles are too weak to EXPEL air properly. For example, I can inhale "ok" -- exhaling is HARD.....as in blowing up a balloon!

I have all of this info in my purse so that I can REMEMBER to ask for a VC or NIF and an ABG if needed. I also have info about trying a CPAP or Bi-PAP first -- before intubation. Even if I can't talk, I'm gonna be pro-active!


A problem I have (and more often, recently).....I am sniffing at the same time as I swallow food (allergy season, here). YIKES!! Food is trying MIGHTILY to go into my lungs. I can FEEL it. SCARY!! Also, everytime I throw up (and I've thrown up SEVERAL times this year), food comes out through my NOSE! Painful AND disgusting!! And, when I take my pills at night with a little water, once I FINALLY am able to swallow the pills, water comes out my nose. YUK!!!!

My MG doc appointments are always in the afternoon. I suspect he wants to see the "real" me -- the weaker me. Perhaps you could make a pulmo appointment later in the day?

If you take tests with a pulmonologist before Mestinon and then again after and the second set of tests show there is a significant improvement....would that actually be accepted as the most objective measuring system for Mestinonīs efficiency?