So last night I started to get the same symptoms as I did last week that required me to stay in the hospital a few nights. The weakness, the swallowing difficulties, the SOB. So I went back to the ER. They gave me some solumedrol, and drew some blood. Long story shorter, they decided to ship me to the University where my neurologist is. I was willing, especially since I would finally be in the place where I could get proper treatment. So after a long ride, I was in the ER there.

My hopes was that they would start the plasma pheresis, what the doctor has been saying I need. So far he hasn't been able to get the insurance to approve it out patient, and had also considered just admitting me to get it started if i met criteria... which I think i definitely do by now....
Instead, they increased my mestinon to 90-120mg every 4-6 hours, and told me to come back on Thursday to see my neurologist and he would be doing an EMG...

I'm sooo confused. My last EMG came back normal, the neurologist was aware of it, and wanted to do the pheresis. Now he wants to do an EMG... for what, cruel and unusual punishment?

I don't get it.

Oh, my gosh, I can't believe they're putting you through this! When I read that you were finally at the right hospital I was so relieved...only to read that you're still not getting the treatment you need. You must be so utterly discouraged. Here's hoping things suddenly turn around for you. Thank you for keeping us updated.

Abby

I completely understand the frustration. I won't bore you with what I went through during my crisis but it has similar undertones. Since you didn't have positive antibodies, they want to make sure you have MG before they do plasmapheresis. I know, do they want you to die proving it? But that's their world of little algorithms. Mestinon and steroids are fine for mild or borderline MG cases. But no antibodies or positive EMG's and they go right for the risk managers (the ones who make sure they don't make any mistakes which might leave them open for lawsuits).

What about your TSH? Have they tested that again? You have antibodies, so any thyroid changes can make you worse.

The problem with doing an EMG now, after having steroids, is that the job of steroids is to make the EMG's and antibodies go negative. Some patients are so bad off that they will have a positive EMG but a regular EMG is not as sensitive as a SFEMG. Have you had a SFEMG? Just let them do it but make sure they do it in a very weak muscle. They won't do chest wall muscles because it's too dangerous.

Careful with those higher doses of Mestinon. To jump from 60 to 120, for example, can cause a cholinergic crisis! Sometimes it's not the dose amount but keeping a consistent frequent dose of it, like the every 4 hours. Talk to your neurologist about this before you do anything too drastic. And while I know some MG patients can handle doses above 100, there are a lot of studies that show anything over 100 can flood the neuromuscular junction too much, causing that cholinergic crisis (too much acetylcholine).

Try not to take what they're doing personally. I know, not easy. They'll "have to" do plasmapheresis and ICU if you get worse. You're on the edge of that right now. Just rest, take it very easy, don't stress out, keep cool and give your MG a chance to get better. I hope they will figure out something to help you get better. Hang in there!

Annie

How were you diagnosed?

I am asking this, because it is not yet common knowledge or fully accepted that patients with MG can have a normal SFEMG and no detectable antibodies. Some neurologists (in fact I believe it is still most of them) still think that a normal SFEMG in a weak muscle rules out MG as the cause of the weakness.

One of my neurologists was ready to accept that MG was the cause of my weakness as long as I had a relatively mild weakness when he did the EMG. But, paradoxically told me I was in remission (and not aware of it) when I had severe weakness with a normal SFEMG.

I felt as overwhelmed as you, when he said this to me. As, at that time I required respiratory support most of the time, required assistance in every daily activity. had severe swallowing difficulties etc.

This was the reason I gradually became an "MG expert" as I pretty much learned everything that was written about this illness, the way it is diagnosed and managed, in order to save my own life.

I do not know how much it can help you, but I can tell you after all the research I did that this "axiom" regarding the SFEMG is based on very poorly conducted studies. In fact, if you carefully read the literature you reach the conclusion that this test may be normal in a significant number of MG patients. In fact it is probably not much better than tossing a coin, in the seronegative group of patients. It definitely can not be used to "rule out" this diagnosis, regardless of the severity of the weakness of the muscle tested.

It took me an extremely long time (and only after I was no longer his patient) to convince one of the neurologists involved in my care, that it is possible to have MG with a normal SFEMG. So, I think that if your neurologists is one that believes in the "SFEMG" and your EMG will again be normal, you will not be able to do much about it, other than find another neurologist who is ready to accept this.

At the same time, I suggest that you try to get a referral to a pulmonologist who has a good understanding of neuromuscular disorders. In retrospect I can tell you that having pulmonologists involved in my care is what saved my life. I was very fortunate that I was initially diagnosed with "asthma", so had a very good pulmonologist taking care of me.

Like Annie said, you need a good respiratory evaluation and not just the bed-side tests they do on the ward, which can be very misleading in MG patients.

I hope some of this can help you.

Unfortunately, I know how overwhelming and frustrating this can be, but even though it is hard to see, there is a light at the end of this tunnel.

meanwhile try to give your muscles as much rest as possible, so that your condition doesn't deteriorate any further.

alice

Just FYI - Mestinon is one of those drugs where "more" is most definitely not always better. It's too bad that most doctors don't understand this.

In many cases, when you find a dosage that works, if you need "more" you decrease the TIME INTERVAL between doses.

If your body gets too much Acetylcholine, you'll go into a cholinergic crisis. Unfortunately, the symptoms are almost identical to those of a person who isn't getting enough ACh.....

And let's face it - we are all dealing with a disease that is only diagnosed about 600 times a year in this country, so even neurologists don't always know what is going on.

If you don't think you are well enough to leave the hospital, then INSIST on being allowed to stay. If they balk at that, tell them that you will be contacting an attorney to take your family's malpractice case if things go South.....

Forgive me for saying this, but I do not think this is good advice.

First, patients who insist on being in the hospital when their physicians clearly think they shouldn't, receive the worst possible care. Such patients are in general seen as attention seekers and suffering from emotional problems. This is what I call the "follie a deux" of medical teams. I have seen that happen in the best hospitals in the world. An entire team can ignore ominous signs once this label is put on a patient. It is an extremely dangerous situation in which the worst mistakes can happen. It is worthwhile doing this only if you have a consultant who you know is fully aware of the severity of your condition, and has enough influence to change the situation and ensure that you receive proper care.

Second, Threatening with a law-suit will automatically (and understandably) disrupt trust and make the patient a potential enemy (in court) and not a true partner. As it is physicians are practicing defensive medicine way too much. Except for rare situations this does not lead to better care, but less good and much more expensive care.

A true physician-patient relationship can only be based on mutual trust and respect. This is even more so for a life-long illness which is not by the book, in which you need a physician who really cares and is ready to bend the rules, take reasonable risks, think out of the box and fight for you.

Alice, I'm not so sure Teresa wasn't being "tongue in cheek" when she said that (about the lawyers). I don't think there is anything wrong with asking why a doctor thinks you are well enough to be discharged. That's our right to know and understand.

Back in 1999, I had a sleep study. Afterwards, the organization was not forthcoming with all of my medical records. I asked in writing three times to have them, once in person at the medical records department. When they still wouldn't give them to me, I sent a letter to the CEO saying that I would get a lawyer to get them for me if I didn't receive them in a timely manner. Even the mere mention of a lawyer makes medical organizations go bonkers. Hiring a lawyer, or even thinking about it, in order to get medical records is not suing, it's PURsuing. And pursuant to law, it's our legal right.

I think the knee jerk legal reaction to a patient who is desperate for help - and so obviously needs it - is so sad. I know it's a reality but it's a stupid reality. I don't know of any patients who want to sue a doctor. But when you're in a hospital bed and no one is doing enough to help you, it's simply the most powerless feeling ever. The people who can help you aren't. So about the only power we patients have are patient representatives of the organization or the dreaded mention of "legal assistance."

And, frankly, the protocols in hospitals are not set up to the advantage of patients. So what do we do when "talking" gets us nowhere? Go somewhere else?

I get both sides of this argument but the ONLY discussion should be about how to make a patient better. And if doctors won't fully assess a situation, then they can completely miss the point that a patient indeed needs better care.

I know that you know all of this, Alice, it's simply a topic that gets my ire up. We patients are not the enemy. But we sure as heck deserve more care than what some of us are getting.

That's yet another reason why a health directive/will is so important. In case there is a time when we can't defend ourselves while on a vent!

Annie

Annie,

This is a very complex issue and I am not sure there are easy answers.

In general I do not think you can force someone to think and care, you can only force them to do something. You definitely can't force them to think out of the box, or find innovative ways to treat you properly, with all the constraints of managed health care.

Obviously, if they are doing something which is clearly illegal (such as withholding your medical records), you can and should use the legal approach.

I am also not saying that there aren't physicians who are truly negligent, but I think it is only a minority.

Much worse than that. I think it is one of the most frightening and humiliating experiences a person can go through. It can leave scars that will take many years to heal.

But, I think that medico-legal practice of medicine only encourages this. It significantly encourages less thinking and a more "by the book"- no risk approach.

Sadly, Protocols in hospitals are not set to the advantage of patients nor the physicians taking care of them. An average ward physician spends less then 10 minutes during the day, next to his patient's bed. (and many times even less ).

Yes, I personally think that as hard as it is, this is what we have to do, if despite our efforts we are unable to have a fruitful dialogue with those that are supposed to take care of us. Or if their misconceptions (we can't change) lead to management errors.

As you well know, I have personally done that more than once.

Unfortunately this can be a two-sided sword. I have seen quite a few physician who translate DNR to "do not treat".

I think what is important to do as a patient is make every effort to find a physician or group of physicians you can trust. Those who are ready to "waste" the time required to get to know you as a person, not a chart or a result of a test. Those who are ready to put the effort and combine their knowledge and experience with yours in order to make your life better and hopefully longer.

I also think, like you, that it is important to learn enough about your illness, so you can become an active partner in its management.