Please keep this on topic so that it might help MGers now or in the future. What I mean by that is start a new thread if you have your own personal concerns, so that they can be addressed separately and so they won't get lost in a long thread. Thanks.

NOTE: These are guidelines only. The best care you can get is contacting your doctors and getting direction from them!

I know that making the decision to go to the ER is a hard one to make, especially if you've been there before, don't have an MG diagnosis or have a "tentative" diagnosis and you've been treated poorly or had your symptoms "pooh-poohed." But your decision to go in (again) should be based on how badly you're doing and not on how badly the doctors are at doing their jobs.

If an ER doctor says that he can tell by LOOKING at you whether your MG is okay or not, that's not only WRONG but careless. Can they tell by looking if a cancer patient is okay? Or if someone with an as yet undiagnosed internal bleed is okay? NO!!! So if a doctor (like Seishin's) says this to you, it's bull. That's why there are objective tests for all conditions, like PFT's and ABG's for MG. Doctors get rated on how many tests they order and hospitals are notorious for ordering the least amount of them. Good health care should not be about money, especially when you have health insurance. It costs more to keep returning to the ER because they didn't help you the 1st or 2nd time!

There are very good ER doctors out there and sometimes it's a matter of giving them the right information or asking for the right tests so that you can both work together to get you the help you need. If you run into the same ER doctor again, who maybe didn't treat you well before, you can always say that you'd like it if "we" could get it right this time.

ER shifts have different doctors and you have to give them a chance to help you. An MG crisis or exacerbation is serious and you need help.

So knowing your "normal" really helps to make the decision about whether or not to go in. Is this a situation where you can rest for an hour and get better? Is it a situation where you can rest for a few hours and you still don't get any better? If you can't get better by resting, especially those of you not diagnosed or not on any drugs, that's when you need help. If you are diagnosed and are on drugs, you need to go in too. You need to either have drugs and/or ventilatory support like oxygen or BiPap or CPAP in order to get better.

The goal with MG is PREVENTATIVE MEDICINE and not crisis management. Why? Because the worse you get, the longer it can take to get better. And, more importantly, you can't know how badly MG will get or how quickly.

Whether you're having trouble breathing in or breathing out or both, that's an emergency for an MG patient. So is choking/not being able to swallow and having a sudden or even gradual increase in generalized weakness where you can't grip, walk, stand up and all of the other activities that our 640 skeletal muscles help us do! An MG crisis may not present with generalized weakness. It can be weakness of specific muscle groups, like the bulbar (throat/neck, jaw, etc.) muscles.

If you are not breathing well, can't swallow well or are generally weaker, go the heck in or, better yet, dial 911!

You don't want MG doing this to you!

There's a fine line between an MG exacerbation and an MG crisis. And due to the unpredictable nature of MG, both should prompt you to seek out care in an ER.

MG Exacerbation: Your MG is getting worse and may or may not be headed into a full blown crisis. You can't determine this by yourself and it's hard to tell if it's only an exacerbation (getting much worse) or an MG crisis. You might be able to speak to your neurologist about this to get more treatment but this should be done at the same time you seek out care in an ER.

MG Crisis: You need help IMMEDIATELY. This is when your MG is tanking fast and you cannot simply call someone or go on a forum for help! You need to dial 911.

An MG "myasthenic crisis" is when you are weaker due to not having enough medication and, therefore, not enough acetylcholine getting to your muscles.

An MG "cholinergic crisis" means that you are weaker due to having too much medication (Mestinon, Mytelase) and, therefore, too much acetylcholine getting to your muscles.

There are many "tools" ER doctors can use to assess how you are doing when your MG is getting worse. You, as an MG patient, can bring along "tools" as well.

ER Tools

1. Oximeter

This should not only be done while sitting on a gurney but while walking around - if you can walk. It's not only useful for showing your O2 but also showing what your pulse is doing.

An O2 is not a great measure of how badly you are doing and needs to be used in combination with the other "tools" like a clinical exam and ABG's. Although I have to say that if you have an oximeter at home and know what your daily normal is, you'll know if it is going too far below that normal. That, in addition to how you're feeling, should prompt a call to your neurologist, a trip to urgent care, the ER or dialing 911. It all depends on how badly you feel.

We all have a normal resting pulse or pulse "range." If yours is higher than normal while sitting during an MG exacerbation, it might show that your heart is working harder to get you oxygen. Or you could have "white coat" syndrome and be freaked out to be seeing an ER doctor again.

And what you'll often see when your MG is worse is that your pulse goes higher while you are walking and your O2 may be relatively okay but then when you stop walking, your pulse goes down and so does your O2. So keep looking at it! That's something many nurses and doctors don't do.

2. Clinical exam

A well-done clinical exam should look at basic muscle groups in MG to see if they are fatigable/weakening. Pressing ONCE against a doctor's hands is not necessarily going to show how bad your MG is because MG gets worse with repetitive or sustained activity. Of course, if you're as bad as you're going to get at the moment, it's hard to compare bad with bad. So if you have a photo of what your "normal" face looks like and one of what it looks like when you're worse, that is helpful to an ER doctor during a clinical exam. If you don't have photos, tell them that this is not your normal!

Frankly, if you're undiagnosed, fatigable weakness is NEVER normal because normal muscles do not do that.

When you get weaker gradually, you may not notice it. That's when it can be more dangerous because, well, you can't tell that you're really bad! That's what happened to me right before my crisis. By the time I got to urgent care, I could not grip the doctors fingers AT ALL. And I "thought" I was squeezing them really hard.

3. Pulmonary Function Tests

They can do anywhere from a bedside spirometry, which is the very basic of "breathing in/breathing out" tests, to a peak flow meter (breathing out only) to full PFT's (pulmonary function tests) to arterial blood gases (ABG's). They could also check to see if you're turning blue.

ABG's show many things but it's often the carbon dioxide level that doctors focus on. Any subtle shifts in your body's metabolism are not a good thing. "Pulmonology and MG" is a complex issue and you can always ask to have a pulmonologist consult on your case. You have the right to ask for the appropriate care. Why? Because it's listed as a "right" in the Patient's Bill of Rights. And you can always ask your doctor directly to see a copy of the hospital's Patient's Bill of Rights. That'll usually get their attention but use it as a last resort, not a first plan of action.

ABG's can show that you are "hypoventilating" or "hyperventilating," both of which can occur in MG but that does NOT mean you are "anxious." You "have" hyperventilation, you aren't actively hyperventilating! This is a direct quote from a book on MG by MG experts. The book is "Neuromuscular Junction Disorders: Diagnosis and Treatment" by Dr. Matthew N. Meriggioli (University of IL Neuro Center), Dr. James F. Howard (UNC), Jr. and Dr. C. Michel Harper (Mayo Clinic).


That means that by the time your O2 tanks and then your CO2 increases, your MG is already tanking you!

MEP (Maximum Expiratory Pressure) and MIP (Maximum Inspiratory Pressure) are the MG specific tests that show how well you are doing at breathing in and out. MIP is sometimes called NIF (Negative Inspiratory Force), a.k.a. same diff.

This shows how NIF is used when deciding whether or not to take a patient off of a ventilator. He's not an MG patient but it's a useful video.

http://www.youtube.com/watch?v=qDNcgA9aZcU

An important note about MIP and MEP. You need to see ALL of the numbers they get. They often only pull the highest ones. With MG, that's not useful and it's downright stupid. Since we get worse with activity, then each recorded MIP or MEP shows a TREND in how your breathing is doing!!! So ask for all of the results.

4. "Other" useful tools

If you're in a crisis and don't have a diagnosis yet, doing an EMG to "prove you have MG" is not only stupid and dangerous but a little late in the process. They can always do an EMG later. They can do an "Ice Pack Test" or a "Tensilon Test" to see if you get better. The most important thing is to get you the treatment you need so that you don't stop breathing! Discharging a patient who can't breathe, swallow or move well is just bad medicine. They either haven't listened or looked at you well enough or they haven't done the appropriate tests to "see" what's going on. Machines aren't usually prone to hypochondria. And they aren't "anxious" or depressed.

If a doctor dismisses bad breathing tests or ABG's, ask them if the machines at the ER are anxious and if they need a paper bag to breathe in.

5. Ears, Eyes, Brains and Common Sense

The most important tools for a doctor to use are their senses. Compassion doesn't hurt either. If a doctor isn't using any of them, you can always ask them if they'd like you to stop breathing and then have to face your family and a lawyer next. Anytime you bring up even a hint of a lawyer, however, doctors can freeze up and turn away. And then turn to risk managers. Then they "red flag" your chart as someone who is "sue-happy." So it's not a great idea.

So if you are feeling powerless in an ER situation, simply ask to be fully assessed with the tools above (1 - 4). Then ask for the appropriate treatment, which is your right as a patient. Then calmly wait for help. Getting upset won't help your care or your MG. Being calm, rational and reasonable - and knowing what to ask for and look out for - will help.

If you are doing badly, either in an exacerbation or an MG crisis, they should be monitoring your EKG, O2 and BP. And they should be doing that in an ICU!!! They don't have the right equipment in the ER proper to do that. A lack of oxygen is very hard on the tissues, particularly the heart and brain.

If you aren't getting the care you need, pull out your medical alert card - if you have one - and ask them to call your emergency contact to come help you. Or point at it. Or use sign language. Or any other form of communication you are left with to let them know you're in trouble and need HELP.

Or ask for a neurologist to consult, which is your right. ER doctors have probably read that 2-page spread on MG in medical school and may have absolutely no experience with it. It might help to bring an article or two on MG and breathing (see below).

You might want to put instructions on your medical alert card/information to have the ER call BOTH a/your pulmonologist and neurologist. They work together for an MGer in an MG crisis.

6. For those of you who have bad bulbar symptoms and might not be able to speak during an MG emergency, consider having a tape recording of instructions to the 911 dispatcher. Or consider having an in-home service where you only have to press a button to initiate having help come to your home.

The people who respond first in a 911 emergency are your local police. So, let them/the dispatch department know this information as well. You don't have to give your entire personal medical history, simply that you have myasthenia gravis and may not be able to move, speak or breathe well in an emergency. They are invaluable and are the best first responders!

7. Give the hospital you live closest to any medical instructions regarding your care ahead of time, just in case something happens. Ask them to make sure it's in their computer system, along with your medical alert card information.

ER Treatments: What you are given for your MG exacerbation or crisis is between you and your doctors. It can be an increase in Mestinon or a decrease if they think you're having a cholinergic crisis (too much acetylcholine from too much Mestinon/Mytelase), IV Mestinon, oxygen, IV Solu-Medrol (steroids), steroid tablets, IVIG, plasmapheresis, BiPAP or CPAP (there and/or at home), intubation or a combination of the above. And don't forget about the "sleep" prescription, which is really necessary when you get worse. It's hard to sleep in a hospital but you really need to. And if the nurses or doctors don't wash their hands before they get near you, ask them to (if you can speak). You don't need to have an infection on top of your already poor condition.



If your MG is getting worse and you continue with a "normal" schedule, MG will get even more worse. So you have to adjust (lower) both your activities and your expectations of what your body can or should do. That's probably the most vexing part of having MG. You want to do what you want to do but it's not usually "your" decision.

It's scary thinking about having an MG exacerbation or crisis but it can be managed and managed well. So prepare yourself and those around you - including your family and friends - for those possibilities. We all need to be set up for success so that we can have the best possible outcome.

If I missed anything or you have something to add, please do! We all need help on how to survive an MG exacerbation or MG crisis, unless you're lucky and your MG never gets worse. I hope this has been helpful.


Annie

Seishin, you asked for it, you got it. Now I can't use my arms.

Attached Files

	MGCrisisExtubation.pdf (73.1 KB, 478 views)
	NMDiseaseCausingAcuteRespFailure.pdf (120.9 KB, 371 views)
	NonInvasiveVentilation.pdf (76.9 KB, 414 views)
	MIPMEP.pdf (192.8 KB, 517 views)

Wow, this is AMAZING! Thank you! I just got out of the hospital two weeks ago after a 9 day stint, and one thing I did was make a list of all the warning signs I became aware of. I will share them. I am actually in bed feeling really horrible today so I have to dig up my notebook, but I know I MUST make up a little packet and have it ER ready...this was my second hospitalization and I was VERY nearly intubated this time. There were most definitely times I could not speak; one once I got admitted when my breathing muscles kept paralyzing for a few seconds at a time, followed by spastic breathing, which was really rather alarming. I'd never had that before this crisis and I did not like it. No one would listen to me gasping for help and my husband was NO HELP AT ALL.

Thanks again!!!

Do you know how to pull up this article? Wondering if it is any good. I used to work with this doctor.


Display Settings:
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Neurol Clin. 1998 May;16(2):391-417.

Neuromuscular disorders and acute respiratory failure.

Bella I, Chad DA.


Source

Department of Neurology, University of Massachusetts Medical Center, Worcester, Massachusetts, USA.


Abstract

This article discusses the assessment and management of rapidly progressive weakness due to neuromuscular disorders. The authors review elements helpful in determining the causes of weakness including pertinent history and laboratory studies. Disorders are classified according to the level of the motor unit involved and triage/management decisions are described. In addition, respiratory function assessment is reviewed. The latter part of this article is devoted to evaluation and management of two of the most common disorders, Guillain-Barré syndrome and myasthenia gravis.


PMID: 9537968 [PubMed - indexed for MEDLINE]

Perhaps Alice could access it but the problem is that none of us can without paying for it, even if the link to it is put up. Why don't we stay to what we can post on the topic?

No one should have to be treated as poorly as you were. One thing I've asked family members to help them understand MG is whether they can remember what they felt like when they were coming out of general anesthesia. You can't move or breathe well then either. If that happened to them from a disease, how would it make them feel? Would they think that was normal? Time is of the essence in those situations for MGers because our MG doesn't "wear off" like anesthesia does.

Annie

Here is a similar PDF.

Attached Files

MGRespiratoryComplications.pdf (95.6 KB, 367 views)

When you are officially diagnosed with MG, ask your neurologist to write a "diagnostic letter" that outlines that you are diagnosed with MG, by what means you were and what course of treatment you are on (subject to change, obviously). Maybe ask them to put in the letter instructions about calling them directly and calling in a pulmonology consult. Or get a similar letter from a pulmonologist.

Make copies of that letter and have one in your wallet/purse for when you need to go to the ER.

Since doctors are quite likely to oppose the requests of a patient and not at all likely to oppose the "orders" of another doctor, this might help during an MG exacerbation or crisis.

Trying to get proper medical care should not be this difficult!

Are you out there fellow boffins? Jana? We haven't heard from you in awhile - hope you're okay.