Visit with the myasthenia specialist (my report)
 

First, I really got a kick out of seeing the plaque on his door. Right under his name it said "myasthenia gravis." Yay, a specialist!

Here's what he told me:

1) My SFEMG two years ago (by which I was diagnosed) was very strongly positive.

2) Hypokalemic Periodic Paralysis would not cause a positive SFEMG.

3) It's "common" for Mestinon not to help MG patients.

4) My spells of intense weakness are unusual for MG, but not weird enough for him to question my diagnosis because of them, especially since we've ruled out so many other things.

5) If Mestinon doesn't work, other acetylcholinesterase inhibitors won't, either, because they're very similar. (That said, I'm going to cautiously experiment with huperzine anyway--but remember, don't take huperzine if you're already taking Mestinon! They both inhibit the same enzyme!)

6) I should give the new higher dose of Imuran (I upped it to 250mg in October) another three months to kick in, and then consider either
Cyclosporin instead, or low-dose (10 or 20mg) Prednisone besides.

7) It's my hip muscles that are weakest. I could push back on his hand quite well in every test except the one where he had me sit on a chair and lift my knee up while he pushed down on it.

8) I don't present like someone who has a CMS, especially since I'm 46. Also, CMS's often give people a certain facial structure that I don't have.

9) He really thinks I have MG. It's true that a SFEMG isn't specific to MG, but the other diseases that yield positive results are nerve diseases that would have symptoms I don't have (I didn't ask what the diseases or symptoms are. I'm guessing it's stuff like numbness, pain, and tingling--just my guess).

That's all I can think of for now. My plan, subject to change, is to give the Imuran until June to kick in. The reason I'm waiting five months instead of three is that I got a huge boost in May last year. I want to see if it happens again, which might have something to do with getting more sun (yes, I'm taking vitamin D supplements). If not, I will consider the low-dose Prednisone.

Here's my thinking on the Prednisone (grateful acknowledgement to 4-Eyes for her part in this). There are two things about it that scare me: the immediate side effects (like weight gain, anxiety, and insomnia) and the long-term side effects (like diabetes, osteoporosis, and cataracts). The neuro suggested I try a low dose, and that might be enough, in conjunction with the Imuran. If I try it for a week and can't tolerate the immediate side-effects, I can quit. If I can tolerate them, I can hope to take it for a while (say, nine months) and then wean off it. It's something to consider.

Thanks to all who are following my continuing saga!

Abby

Very informative!!! I am on 20mg of Prednisone with no side effects but when I was on 80mg and 60mg it was horrible. I have borderline diabetes and take meds for it but not insulin, The 20mg has not changed that. My A1C test run 6.5 and varies very little.

I have gone downhill a little since middle of last week. My legs are always tired and I get short of breath pretty easy. Got my regular neuro visit in about an hour. Will see what he says.

Abby thanks for your great post!
Mike

glad it went well!!! and glad you saw someone who seems so knowledagble!!!

That sounds like great progress and info for your continued journey with slightly altered route......AWESOME :)

Anacrusis

Had my regular neuro visit today. Thank the good Lord for my wife 2 doctors missed my low level of Vit D. After she pointed that out he said to take 2000mg of D3 and reduced my Prednisone 2 1/2mg for 6 weeks. He said for me to call him if my legs or breathing got worse and we would IVIG again. I'm doing ok but could tell I had gone down hill a bit.
Mike

I'm glad you had a good appt. It sounds like it was very thorough. Except for one tiny little thing.

If looking at someone's facial structure was enough to diagnose them with a CMS, why spend all of that research money and time to develop all of those blood tests?! Come on!!! That's one of the biggest prejudices I've heard. That's like saying someone doesn't have MG if they don't have exaggerated ptosis. There is a CMS with facial malformations but that involves ONE mutation.

The doctor you saw was an MG expert, not a CMS expert. Yeah, there's a difference. I'm not saying you have CMS but there are so many variables within that group of genetic diseases that you can't say that age is the only factor. Or that facial structure is! :cool:

Have you tried physical therapy? Maybe they would be able to work with you to improve your hip area. It's possible. Or maybe your bed doesn't fit you well enough (seriously). I love how physical therapists can really help people with improving even the slightest problem.

Please also consider the cancer risk with combining two immuno's. It's a very real risk and the kind of cancers you can get, not to mention the "cure" for them, are much worse than relatively ok MG.

I just want you to have all of the facts during your decision making process. I hope you come up with a plan that works for you.

Annie

My neuro did say he wanted me to walk 3 times a weel plus my table tennis 3 times a week. I think he is testing my legs. My wife says the low vit D could cause my tiredness.
We didn't discuss my facial feature or CMS. You may have confused me with someone else. He did his usual tests, checked arm strength, leg strength and eye movement. He wants to see how I do this next couple of weeks. He thinks I may need anouther round of IVIG. I haven't had IVIG since Sep.
Mike

Annie, just to clarify: he didn't say that a certain facial structure, or lack thereof, is diagnostic. He mentioned it as one factor to consider among many in the decision about whether CMS is a real enough possibility to be worth a muscle biopsy at Mayo.

He didn't suggest I take cyclosporin and Imuran together. He suggested that I stop taking Imuran and start taking cyclosporin--or that I take Imuran and Prednisone together. When you talk about the cancer risk of combining to immunosuppressant drugs, did you mean Imuran and Prednisone? Or just Imuran and cyclosporin?

Physical therapy...I dunno, that's a really hard sell. For me to go to the appointment (half-hour drive into the city) would utterly exhaust me. It's hard for me to believe any benefit I could get could outweigh that.

Thanks for your help.

Abby

I meant steroids with Imuran or another immuno like Cellcept. It can be hard for the immune system to bounce back after a dynamic duo like that. I've seen it one too many times with people I've known who have lupus.

The muscle biopsy they do for MG is different than the one they do for CMS. I've had the basic one at Mayo - ouch! And you should know ahead of time that the CMS expert there "claimed" that they don't often see the NMJ in adults but only in children. That's a load of BS, however, since there are loads of articles on seeing it in adults! ;) The biopsy for CMS is far more dangerous because they normally take it from the intercostal muscles. They can take it from the arm too but both are considered "surgery" (under general anesthesia) while the MG one is more of a "procedure" (under local anesthesia).

At Mayo, they don't do the CMS biopsy much anymore. Neither do the CMS experts in Europe. They can tell from genetics, clinical history and blood tests if someone has a CMS versus MG. So that information your doc had is outdated. Again, I'm not trying to show him up or anything! It's just that you deserve to have the most accurate info in order to weigh everything. I'm not a doctor but I do pay attention to details more than some of them. :cool:

I wish I could put the big PDF I have from Hantai, an expert in Europe. It's the most informative one, besides the WUSTL site. I can't find it on the web either - I've tried.

I totally understand the PT thing. But what if you had a consult and then one follow up? You don't always have to have a bunch of appts. Or if you qualify for home health care, that might be a way to get around having to drive so far.

You have a great couple of neurologists and they're taking good care of you!

:hug:
Annie

Sounds like everyone had very productive visits with their neuro this week. I am so glad.

But now you have me curious. What are the facial features associated with CMS?

kathie

I knew you would. My neuro really does get MG, but like a lot of doctors, he can't bring himself to say, "I just can't think of anything else to do for you," so he says, "Well, how about a little exercise?" I say, "My street is 500 feet long. If I walk to the end of it I collapse and can't walk back. He says, "Well, how about an exercise bike?" Yep, that solves the "can't walk back" part. The proper response to this suggestion is, "You're a stitch, Doc!"

I just remembered that I saw a PT when I was in the hospital for my first IVIg. He determined my hip muscles were weak, and gave me muscle-strengthening exercises to do. This just seems non-sensical to me. If I do the exercises, I'll just wear out the muscles and then they won't be there for, you know, walking into the next room. So someone would have to explain to me exactly what I could hope to gain from PT. The problem is not that the muscles themselves are weak; the problem is that the signal from the nerves is blocked, right? If I were a body-builder, and came down with MG overnight, I'd feel just as weak.

Abby

I have to agree with you, Abby, that it's a NMJ thing, not a weak muscle thing---and I'm a therapist. When I had the reboot, and to a lesser degree when I had my two "mini-remissions" I would wake up and the muscles would just be "good to go." Sure I would say I was "out of shape" but not unlike anyone else who'd ignored their exercise program for a period of time. It isn't weakness like someone who has had a stroke and is working to rewire neurologic connections, or someone post-immobilization from a cast or other device. If you are able to exercise on a daily basis, more power to you, and you should be doing that within your ability.

Now what I could see as far as therapy usefulness goes, are instructions in proper body mechanics, functional transfers, gait, use of assistive devices, home modifications and energy conservation instructions. All of that could help you make the most of the energy and strength you do have. That would best be done in a home visit, so the therapist can actually see your environment and learn what you are and are not willing to do/use.

Hope this helps.

Abby, It's not accurate to equate PT to exercise. What a good physical therapist does is to evaluate any issues you may have physically, like any tender points that could lead to more weakness, give therapies that are useful like ultrasound or massage and, yes, give exercises if they are useful.

There have been many times when I get a particular muscle group that "knots up" and if I don't massage it out, it not only keeps getting worse but then strains the other muscles around it. It's a vicious cycle.

So think of PT as alleviating muscle issues due to MG and not as causing more. I'm lucky my neuro has PT in his office. They get NM diseases and know how to work with them. It makes a huge difference in the quality of my life.

Feel around on your hips and back for any tender areas. Find any? That alone would be reason enough for PT.

:hug:
Annie

Exercise, PT, muscle strengthening are critical for us. The reason is that we can strengthen our muscles so that they are stronger to begin with and that can help compensate for the MG weakness. We can do more with less if our muscles are stronger.

I've had PT come to my home many times. They have given me exercises that are simple but always muscle strengthening. They involve using stretchy bands, lifting soup cans in a variety of ways to strengthen my arms, etc. The weaker we are and the less we move about, the more muscle strength and tone we lose. We absolutely need to preserve and maximize our muscle strength through exercise.

I swam all summer long and it was great. My muscles were so much stronger. I haven't been doing much since summer. I really need to get a program. Plus exercise is critical to our health anyway.

I know I can't always exercise but even when I was just in the hospital in crisis I was shown exercises to do right in my bed to strengthen my muscles. They were very helpful.

There isn't much we have control over but this is one thing we do. We have to keep our bodies tuned and our muscles as strong as possible.

Wondering
 

Pingpongman, My forty year old son was diagnosed MG in October, 2012 via eye muscle tests, blood tests, and a CT. In December he had a thymectomy and had a thymoma removed. He does not need chemo or radiation, but needs to get a CT every six months. He is now on 60 mg of Mestinon three times daily, and 20 mg of Prednisone twice daily. The neurologist said he would probably have to adjust his dosage when he goes to back to see him the end of this week.

His ptosis and double vision have subsided. However, he now has problems chewing and weakness in his arms and legs. He also notices that he feels like he doesn't have enough air when he tries to sing and can't whistle. I know the neuro doc is still trying to find the proper doses of medicine, but I have read that IVIG helps many to get their MG under control and noticed you said that you would IVIG again. I am wondering if he should mention this to his neurologist.

I am better now. I was going through withdrawal of my prednisone. I have been on it for a long time and when you start reducing at the 20mg level it gets tough. Reducing 2 1/2mg doesn't sound like much but believe me it is. It really depends on how much and how long you are on it.

It took us 2 1/2 years to find the meds that work for me. It is all trial and error. As for IVIG I'm pretty sure it never did much for me. One time I had 24 treatments over an 8 week period and nothing happened. That said we are ALL different and react differently. Some neuros like to hold IVIG in reserve for crisis situations. My neuros didn't have that attitude. It is definitely worth a shot. I wish you the best in your journey.
Mike