what should my next treatment be?
 

I just saw my neuro. Right now I'm just on Imuran (200 mg.). I think I may be a bit better--it's hard to tell, because I'm so up and down. But I think the downs are a bit shorter and farther between than they used to be. I'm not really sure. I'm definitely not getting worse, at least.

Here are my options:
--Increase the Imuran (my neuro isn't sure if he's willing to go higher than 200)
--Start low-dose Prednisone (my symptoms aren't severe, so I've been avoiding it)
--Start cyclosporine (he says it's so toxic that a year or two is all most people can handle before their kidneys suffer)
--Start CellCept (he doesn't recommend it--he doesn't believe its effective)
--have a thymectomy (see my other post)
--do nothing and hope the Imuran starts working better (I've been on it a year, but only a few months at this dose)
--and? Anything else I should consider?

My neuro leaves an awful lot up to me. I am pretty well informed, so I guess I appreciate that. But I'm frustrated that this is going on and on and on--I feel we haven't exhausted all our options yet. But since my symptoms are relatively mild (eyes OK, no breathing trouble) we're not willing to do radical and dangerous things.

I would greatly appreciate some suggestions.

Abby

Any reason you're not taking Mestinon?

Brian

Brian,

If I take Mestinon regularly, it makes my eye hurt. I do take it occasionally, if I have to go somewhere and need some extra strength, but it only helps a little bit.

Abby

Odd...

I'd ask your neuro to look at a mestinon alternative:
Physostigmine
Neostigmine
Pyridostigmine - this is Mestinon
Ambenonium
Demarcarium
Rivastigmine

Mestinon does the job better than any other Cholinesterase Inhibitors but there are alternatives if you can't use it. All of the above are reversible so they would behave in a similar manner to Mestinon and have a relatively short half-life in your system. I would think that you would need *something* to manage the downs if you're experiencing a lot of ups & downs.

The imunosuppressant is great for long term reduction of the symptoms but on a daily basis I'd certainly want something to get over the hump on a bad one...

Wow, thanks. I didn't know there were so many. Right now my drug of choice for a mild lift is coffee. But I should try the others.

I have two distinct groups of symptoms. The first is mild to moderate weakness that's pretty steady. The Mestinon helps with that a bit, but not tremendously. The second group of symptoms is episodes of severe weakness. They last about two hours. My legs get so weak that I can't walk at all. These episodes interfere with daily living more than the general mild weakness, and the Mestinon doesn't do anything to prevent or treat them.

My neurologist is puzzled by the "episodes." He says that's not typical of MG.

Abby

My symptoms are episodic also. Usually if I do too much I will get more MG symptoms in my legs and throat. Other times I can do strenuous exercise with no MG and still other times I can do relatively nothing and get hammered with the MG. I take low doses of mestinon, 30mg every 4 - 5 hours, and take slightly more when I need it. Over the past two years this seems what works best for me. 60mg at any one time makes me sick unless the symptoms are severe. You need to find out what you body tolerates the best. If you can stay away from the stronger meds and live with the disease it would probably be the best as all the meds except Mestinon have side affects. At least that is my plan. I was on Prednisone for 4 months and just came off and am finally starting to get back to normal. As it did help with the disease the side affects for me were terrible.

Hope you can figure out what is best for you.
Al

Thanks, Al. I have been trying to avoid Prednisone because of what I've read here. I am fortunate not to have had (so far) any dangerous symptoms, or vision problems, so I have the luxury of being able to avoid it.

I was also taking 60 mg. at a time of Mestinon at first, and it was too much. I do better on 30. I thought all the muscle-twitching was a side-effect, but now I think it was a sign that I was taking too much.

My neuro says that even though I've been on Imuran for a year, I'm not at the point where we can say "Well, that's not going to work." He's seen it take more than a year to "kick in." So I am willing to keep trying, especially because I've only been on this bigger dose for a few months (I took only 100mg/day for the first six months or so), and because I think it may be helping--or at the very least it may be stopping me from getting worse.

Realistically, at this point, I do not ever expect to be fully rid of this disease, not even temporarily. But I also think it's realistic to hope that I can have some some real improvement.

Thanks for your help,

Abby

How's this for a long shot?
 

Abby,

You mentioned your episodes again. I know you have read about periodic paralysis, did you ever trial the medications for it? You're right - your case is not typical for it. But are you typical for MG? In addition to considering other MG therapies, would it hurt to ask your doctor if a Diamox plus potassium trial might be appropriate in your situation?

Diamox doesn't help all patients with ion channel disorders, but it might be worth at least a try. Ion channel disorders are known for episodic symptoms. You've talked about losing your balance, have you read about episodic ataxia?

You have also spoken recently about hormones and circadian cycles, both of which are triggers for some people with HKPP.

I know 4 people with HKPP who were originally diagnosed with MG, and even had positive responses to MG treatments, but not as much benefit as they get from HKPP meds.

A 5th person I know was originally diagnosed with MG but now is believed to have episodic ataxia (also a channelopathy.) She has weakness very similar to mine, and once she got on Diamox plus prescription K+ (same meds as treat HKPP) she "can't believe muscles can work so well." (The first time she tried Diamox, it made her weaker... she didn't get the prescription potassium to go with it. That's what happened to me when I tried it.)

You know I've been struggling with diagnosis, and have most of the same concerns as you do about not being a good "fit." However, just as with MG, not everyone with HKPP is a textbook case. HKPP can be sporadic. Initial onset has been documented in patients as old as 80. Potassium levels can remain within the normal range even during an attack. Many of the patients are not fine between episodes (I went to the 2009 conference, and a great many require the use of wheelchairs on a full-time basis.) I don't have a good answer for the "better in the morning, worse at night" dilemma that we share, unless it's an episodic ataxia or other channelopathy thing.

I just got a trial of Mestinon recently, but if that doesn't pan out soon, the next trial for me will be the Diamox. The good mornings/bad evenings and eyelid/facial involvement had me leaning toward MG, but recently I was put on Propranolol. I read it can be used to treat HKPP, but has the potential to make MG worse. So I took it cautiously to see what happens.... I seem to have fewer severe weakness episodes on it. So I'm thinking I need to check HKPP better for myself.

Sorry this is so long. Whatever route you end up going, I sincerely hope you find SOMETHING that works for you!

Tatia

Tatia, I really, really appreciate this. I asked my neuro today if he would be willing to keep treating me for MG while exploring other possible diagnoses. He said yes, if he could think of anything. I suggested retesting the basics (like CK levels) and he said that degenerative muscle diseases don't cause episodic symptoms. And my episodes are starting to really stand out. I think my general weakness is better, but my episodes are worse. Before, it all sort of blended together.

My symptoms aren't typical of MG. I have little or no eye involvement. I'm seronegative (AChR antibodies tested three times, MuSK and LEMS once each).

My balance is better than it was two years ago when I first had symptoms. I'm quite sure when I lose my balance it's because the muscles in my sides are weak. When I have an episode of weakness, I feel weak all over, but what stands out is that I can't walk because my legs don't hold me up. I don't fall, but I sink into a squat. When I'm weak like this, my husband stands behind me and puts his arms around me to hold me up and we walk like that to the couch or car or wherever I need to get to, but if the ground is slightly uneven or his foot gets in the way slightly, I can't recover, and he has to catch me (we used to both fall, but he's gotten better at it).

I have read about the different kinds of periodic paralysis, but there are things about it that don't sound like me so I had dismissed it. So it is startling to hear you say that there are atypical cases--because after all, I don't sound like a textbook case of MG either. So maybe I shouldn't rule it out.

I'm so glad to hear you're getting some trial drugs. When you posted recently that the doctor told you to give up the idea of a diagnosis and just treat symptoms, I didn't realize he was going to be willing to, well, actually treat symptoms with serious drugs. That's wonderful. If you got to the Diamox and it made a huge difference, wouldn't that suggest certain diagnoses?

I will talk to my neuro about ion channel disorders. Please do post here how you're doing. Thanks again for taking the time to make all those suggestions. I'm excited about looking into this.

Abby

Abby, Have you had your celiac antibodies done or a fasting glucose and A1C? They can also do a 24 hour potassium URINE test or other electrolytes. Has your endo checked other things like adrenal function (again, fasting)?

Maybe they could redo the LEMS test.

Imuran should've kicked in by now. At least that's what the experts say. It's supposed to kick in sooner than Cellcept. I know someone with MG who didn't know how well it was working until she went off of it. :cool: She went off anyway due to the side effects.

Don't forget about Hizentra.

While you're deciding what to do, think about your overall quality of life. I know MG sucks but you do have it. Can you really expect to feel like super woman unless you roll the dice and do reboot or stem cell therapy? Even that's not foolproof.

I don't have answers. I'm stuck with Mestinon only and a sort of half life.

Annie

I have never had the celiac antibodies done, but I eliminated gluten from my diet for a trial of about seven weeks. I didn't note any differences when I stopped, or when I started eating it again. I know that's not conclusive. I have no digestive problems, which I also know isn't conclusive. I suppose I should have it done. I get my potassium tested regularly because I'm on HCTZ (to prevent migraines that were consuming my life). It was borderline low at one point, but hasn't been since I started taking care to eat potassium-rich foods every day (at lunch--and my episodes of weakness hit around 5:00 p.m.--as far as I can tell, the episodes are totally independent of what I eat and whether I eat). My blood sugar has always been excellent. But I'll talk to my endo about these things.

What makes you think of LEMS?

I asked my neuro about Hizentra, and he said exactly what I predicted he'd say: "I don't have any experience with that." This is frustrating. He's the myasthenia expert in the area. MG is a neurological disease, but many of the treatments are more in a rheumatologist's area of expertise.

I'm holding out hope for the Imuran because I was on a dose that was way too low for most of the year. When I get my bloodwork for toxicity, the lab measures the size of the red blood cells. Evidently if the Imuran is having an effect, they're supposed to be bigger. Mine weren't as of February--that's when I got my last dose increase. Will see if they are now. And you know what, I am better--when I look at the whole picture--better than I was a year ago, and what could account for that but the Imuran? I bet I would find out I was much worse off of it. I'm fortunate that it doesn't give me any side effects that I know of.

Thanks for taking the time to respond. We appreciate what you do here!

Abby

Tatia, I've been reading about the different kinds of periodic paralysis. I never wake up weak, or very rarely. Here's something, though: I often find out I'm weak when I stand up after sitting for a while, at a movie, at a restaurant, or a church. Many's the Sunday I breezed into church on my own two feet but had to be dragged out. All the little old ladies are very concerned about me.

I know you said that it's been documented as starting in very old people, but that seems to be extremely rare. from what I read ("almost without exception, the first attacks begin before age 25"). I'm trying to think back to anything I could have had when I was younger that was an episode. I used to have sleep paralysis: I would be awake and conscious (able to hear things going on around me) but completely unable to move. But I think that was textbook sleep paralysis. If someone touched me, I'd immediately snap out of it. That's sleep paralysis: my mind was awake but my body was still asleep.

Anyway, I got my first noticeable symptoms of what I think is myasthenia at age 42.

I'm going to do some obsessive journaling.

I haven't had an attack for the last few days, but I can feel the normal weakness in my arms and neck. Today (a good day) I had to rest halfway through slicing a loaf of bread, and as I type this I'm slumped back so that I can rest my head on the back of the chair, because my neck's tired. That's normal myasthenia stuff--worse in the evenings. The "episodes" are in addition to that.

Abby

I am unlike others here in that Prednisone makes me feel so much better! I only take low doses and only for short periods, but for me it is like energy in a bottle.

Abby,

Your quote comes from Deb Cavel-Greant's website. Incidentally, she is one of the folks with HKPP who was originally diagnosed with MG. She even authored "You, Me, and Myasthenia Gravis." Her site also says about Hyperkalemic PP: "Symptoms usually develop in the first years of life, but invariably begin by age 20" The gentleman I sat next to at the conference did not get symptoms until he turned 42, and he has been genetically confirmed with HyKPP.

Please don't misunderstand; I'm not trying to push this on you. I even used the words "long shot...." It's just that you asked "What else should I be considering..." Since you've been looking at things like surgery and worms, I just thought I'd mention it in case you wanted to try the meds for a month or two just to see if you might be one of the irregular cases. Take it or leave it as you will, but please don't let your age be a determining factor for you.

It's probably a long shot for me as well, but I'm at the point where I may never get definitive answers and I'm just going to try treatments for similar conditions until I find something that helps or die trying.

Tatia,

You've been very helpful to me. Because of our discussion, I gathered the courage to ask my neurologist to consider ion channelopathies (he's a good doctor, who has never treated me disrespectfully, but I am not over the fear of being pegged a hypochondriac, or having my illness labeled psychosomatic--I'm sure you know what I'm talking about!). He's going to look into it, and talk with his partner.

I also read somewhere--maybe on the same site--that many people with HKPP only have attacks in their sleep and don't even know it, for many years. So, that makes me think it's possible that a patient does start under the age of 24, but doesn't know she has it until she's 43.

I have not had an episode in several days. This is most likely because I'm in the "good" part of my monthly cycle. In fact, I've been feeling quite good--the best I've felt in a couple of years, with the exception of six weeks after a successful course of IVIg. I went (gasp) to the DMV this morning, and took the kids to a (very) nearby lake in the afternoon. Sounds like I"m "normal," right? But when I pumped gas, I could only manage ten gallons before my hands completely gave out. And I hadn't even noticed they were weak. So, there's that: I'm not normal between episodes. I read that neither are people with HKPP--it's just that they're used to the weakness, and think of it as normal, especially against the background of periodic extreme attacks.

I really just meant to say: I greatly appreciate your suggestions, and I'm following through. I have the general impression that there are many diseases that the textbooks say show up in childhood that actually sometimes show up much, much later. CMS is one of them.

Abby