MGH or Brigham & Women's Experience?

[minimus]

I had the iCPET and lived to tell the tale. Although the test is difficult and physically demanding, they did use IV saline to keep me hydrated after the test, which limited the post-test crash a bit. In my case, Systrom said right after the testing that my right atrial pressure was “the lowest of the low”, meaning veins do not return blood to the heart the way they do in healthy people/controls during exercise. The PA who spoke to me after said this is the result of dysautonomia. (All of my immune and antibody tests were normal, though they hadn’t yet gotten results of the skin biopsy to test for small fiber polyneuropathy.)

Systrom prescribed Mestinon on the spot, which I have started. He said he won’t know whether I also have mitochondrial problems - or how bad they are - until he evaluates the lab work on the blood samples drawn during my exercise test.

Does anyone know how long it typically takes for Mestinon to fully take effect? So far, I am not feeling any bad side effects (knock wood). I feel my exercise intolerance is maybe a bit better after three days on it, but I recognize this could be a placebo effect.

 

[Rebeccare]

I had the iCPET and lived to tell the tale.

Hooray!!! I'm so glad! And it seems like the test did give you important information about what's going on with your body. How are you feeling right now?

Does anyone know how long it typically takes for Mestinon to fully take effect?

I think (my memory on this isn't so good) that they start you off on a very low dose for a few weeks or a month to allow your body to get used to the medicine and minimize side effects. I think dose is below therapeutic levels. After a month they'll double the dose to a therapeutic level, and I think that it takes effect pretty quickly.

Good luck! Keep us updated!

 

[kikala]

So glad to hear, @minimus! Please keep us updated about the mestinon and whether you experience any positive effects. Fingers are crossed for you.

I had to decide last week whether to keep or cancel my appointment for the iCPET testing. After talking with 5 of Dr. Systrom's patients who underwent it and tried Mestinon, I decided to postpone the testing. Of the folks I spoke with, one felt that Mestonin had helped slightly, and the others either felt nothing or had bad side effects and stopped. Given my concerns about the kickback from the testing, I decided to hold off.

Because my blood work tested positive for rheumatoid factor, I am going to follow up with Paul Dellaripa, the rheumatologist Systrom works with. I'll let folks know if anything positive comes from it.

 

[minimus]

@kikala, thanks for the good wishes. I went ahead with the test because Susan Levine said a decent number of her patients have been helped by Systrom.

At the margins, I feel that some of my muscle stiffness and flu-like aches have lessened a bit since I started on Mestinon, though I have had an increase in muscle twitching and a few mild muscle cramps, which Systrom said is a side effect of the drug that he said tends to dissipate over time. I haven't experienced any notable GI side effects yet, fortunately.

That said, I definitely am not transformed by Mestinon, at least at the starting dose I am on. On Health Rising, Cort Johnson profiles someone with long-term moderate/severe ME/CFS who was practically cured by Mestinon. But she seems to be an extraordinary exception.

According to one of Systrom's physician's assistants, Jeff Voner, the standard protocol is to start patients on Mestinon if indicated by the iCPET, see if the medication helps them, and if not, switch them to another medication that improves autonomic function. Then, at a follow-up appointment approximately six months after the iCPET, all patients do a second CPET (non-invasive). If their exercise tolerance hasn't improved or if they show signs of autoimmunity, Systrom then considers a second line of treatments.

However, those treatments -- which seem to include self-administered IV saline infusions after the insertion of a central/PICC line, as well as IVIG, plasmapheresis, and possibly Rituximab -- are viewed as fairly aggressive, with more side effects and downsides. Also, I suspect it isn't that easy to get insurance to pay for a lot of these treatments.

In any case, I wish you good luck with the rheumatologist. I hope he can offer some treatments that will help. And maybe he can give you some guidance on whether it makes sense or not for you to do the iCPET at some point down the road.

 

[Mel9]

I started on Mestinon a week ago.

I am starting slow (30mg once a day)

I do feel something is working.

I have moments when I feel ‘normal’ for the first time in A long time.

Soon I will increase the dossge to 30 mg twice a day

I then plan to increase it to three times daily

 

[*GG*]

I started on Mestinon a week ago.

I am starting slow (30mg once a day)

I do feel something is working.

I have moments when I feel ‘normal’ for the first time in A long time.

Soon I will increase the dossge to 30 mg twice a day

I then plan to increase it to three times daily

I started at 120 mg 2 weeks ago, not feeling much, upped it to 180 today.

GG

 

[kikala]

Thanks so much, @minimus, @Mel9, @GG, @RebeccaRe -- I really appreciate you sharing your experiences with Mestinon and with Dr. Systrom. Please keep letting us know how you're doing!!

 

[*GG*]

I started at 120 mg 2 weeks ago, not feeling much, upped it to 180 today.

GG

Edit: Dr Systrom is not my Dr, did see him at the Hanover, NH event and learned of this drug there. why I'm on it now Would be great to get back more of my life!

 

[Vineyard1]

Hello,

I am following this with great interest as I have an appointment with Dr. Systrom in June (long wait list). I have a diagnosis, in addition to ME/CFS of 18 years, of CVID and dysautonomia. I receive IVIG, every 21 days, for the CVID and take Mestinon 60mg three times per day for the POTS and NMH. In fact, I can't move about my house without air hunger and severe tachycardia without taking it.
I wanted to see Dr. Systrom to be evaluated for pre load failure and have a skin biopsy as my ME/CFS Doctor suspects I have developed small fiber polyneuropathy from a tetanus vaccine 11 months ago. If it is positive then my IVIG dose could be adjusted for SFN.
I am quite concerned about the iCPET as I am primarily homebound and needing to lie down a few hours per day. However, I am willing to see him in consultation and determine how to proceed.
Thank you to everyone who shares their stories and insights.

 

[*GG*]

Edit: Dr Systrom is not my Dr, did see him at the Hanover, NH event and learned of this drug there. why I'm on it now Would be great to get back more of my life!

Now I have come down with Bell's palsy, hope it's just a coincidence!

GG

 

[Mel9]

I am now taking 30mg mestinon twice daily. I have been on two half hour walks in two days and no PEM although I experienced a few hours of tiredness after each walk.

@GG: Bell’s palsy soundsnasty. I hope it is temporary?

 

[minimus]

@Vineyard1 Instead of waiting until June, it might make sense for you to call the office of Anne Oaklander at Mass General to see if they can give you the name of a local physician who does the skin biopsy for small fiber polyneuropathy. (Systrom sends the skin biopsy to Oaklander's lab and according to a recent talk available on Youtube by Oaklander, her lab accepts specimens from many doctors...you don't have to see her or Systrom to get tested.) That way, you can see whether it is advisable to alter your IVIG dose to try to reverse the polyneuropathy.

As far as the iCPET, I guess I would be somewhat concerned in your case about whether the benefits of the test are worth the risks. If the iCPET shows preload failure, Systrom prescribes mestinon at an ultimate dose of 180mg per day. Since you are already taking mestinon at that dose, there may not be anything to be gained in terms of treatment for preload failure.

As far as the downsides of the iCPET, the risk of a crash is meaningful. It is stressful to have the catheters inserted, though fairly painless, and the exercise test itself is exhausting. In my case, it involved about 7 minutes of pedaling against increasing resistance until my legs gave out. There are three or four people in the room who urge you to "keep pushing" during the test at 60RPM, because they want to make sure that you have met the parameters for fully exhausting your aerobic and anaerobic capacity.

If that seems like too big a risk and you cannot find another MD to do the skin biopsy, then maybe just go to the initial consult with Systrom, have them do the skin biopsy, but take a pass on the iCPET.

 

[Vineyard1]

@Vineyard1 Instead of waiting until June, it might make sense for you to call the office of Anne Oaklander at Mass General to see if they can give you the name of a local physician who does the skin biopsy for small fiber polyneuropathy. (Systrom sends the skin biopsy to Oaklander's lab and according to a recent talk available on Youtube by Oaklander, her lab accepts specimens from many doctors...you don't have to see her or Systrom to get tested.) That way, you can see whether it is advisable to alter your IVIG dose to try to reverse the polyneuropathy.

As far as the iCPET, I guess I would be somewhat concerned in your case about whether the benefits of the test are worth the risks. If the iCPET shows preload failure, Systrom prescribes mestinon at an ultimate dose of 180mg per day. Since you are already taking mestinon at that dose, there may not be anything to be gained in terms of treatment for preload failure.

As far as the downsides of the iCPET, the risk of a crash is meaningful. It is stressful to have the catheters inserted, though fairly painless, and the exercise test itself is exhausting. In my case, it involved about 7 minutes of pedaling against increasing resistance until my legs gave out. There are three or four people in the room who urge you to "keep pushing" during the test at 60RPM, because they want to make sure that you have met the parameters for fully exhausting your aerobic and anaerobic capacity.

If that seems like too big a risk and you cannot find another MD to do the skin biopsy, then maybe just go to the initial consult with Systrom, have them do the skin biopsy, but take a pass on the iCPET.

Thank you for your wise advice!

 

[minimus]

I had the iCPET on November 20th, then started Mestinon at Systrom's starting daily dose of 90mg for the next 3+ weeks. In the last four days, I have raised the dose to 120mg (as I try to get to Systrom's recommended therapeutic dose of 180mg).

My leg muscle fatigue/pain/leaden feeling has gotten worse on balance since the iCPET and the start of Mestinon, especially since raising the dose four days ago. I haven't been able to leave my apartment at all in the last 12 days and am having a tough time with minimal walking within the 3 rooms of my apartment without really bad leg symptoms and PEM in recent days.

Maybe my ME/CFS baseline, which worsened almost exponentially in September, is just continuing to deteriorate and Mestinon can't stop the decline. Or maybe I am having an unusual reaction to Mestinon. Am waiting to hear what Systrom's office has to say. In any case, Mestinon is not having a miraculous effect on me so far.

 

[wigglethemouse]

@minimus
From Mestinon Wikipedia : https://en.wikipedia.org/wiki/Pyridostigmine

Pyridostigmine is used to treat muscle weakness in people with myasthenia gravis or forms of congenital myasthenic syndrome

Just be aware that for some forms of congenital myasthenic syndrome Pyridostigmine will worsen muscle weakness, and for those different medications are recommended. Not saying you necessarily have CMS but there is evidence that Pyridostigmine can either help or hinder health. So unless you have a firm MG diagnosis proceed with caution.

I like to remember these two sayings for supplements and medications
* Start low and increase slowly.
* They can help, harm, or do nothing. Listen to your body.

 

[minimus]

Thanks @wigglethemouse. Useful advice that I usually internalize. ME/CFS is brutal enough as it is. I once was prescribed IVIG and stopped after the third monthly infusion because I couldn’t handle the side effects. In retrospect, I feel like I pulled the plug on IVIG too early. When I saw Systrom, he seemed to think mestinon or another medicine would help, so it is pretty depressing to be heading downhill, either because of mestinon or in spite of it.

I described my worsening muscle weakness in a message to Systrom’s physician assistant. He responded today and recommended dropping the daily dose back to 90mg for a week to see if the leg muscle pain/fatigue resolves - which seems unlikely because (a) I had leg muscle pain/fatigue before starting mestinon and (b) I will still be on mestinon, just at 90mg rather than 120mg daily.

If my leg weakness/pain resolves, he says they may either consider a different drug or have me stick with mestinon because muscle weakness may be only a temporary side effect of the drug. On the other hand, if the weakness doesn’t improve after going back to the 90mg dose, then it’s a sign my ME/CFS has worsened further, so in response I should increase the mestinon dose to 180mg.

This seems a bit frightening to me, as most roads lead to more mestinon in the proposed decision tree.

 

[wigglethemouse]

This seems a bit frightening to me, as most roads lead to more mestinon in the proposed decision tree.

I hear you. I learned the hard way with a PA telling me to increase meds when I got worse, and I ended up even worse. You could reduce the dose even further if you really want to try it, or stop and try again in a month or two. You need to be the one in control.

I've heard of some docs starting Mestinon at 15mg per day, and increasing slowly at a weekly intervals depending on how you tolerate.

Just to back up my previous statement here is a link describing that Mestinon can be harmful in certain cases. There may be others also, that are yet to be determined.
https://www.mda.org/disease/congenital-myasthenic-syndromes/medical-management

The cholinesterase inhibitors used to treat myasthenia gravis (MG) are helpful in some types of congenital myasthenic syndromes (CMS) but may be harmful in others.

 

[Rebeccare]

When I saw Systrom, he seemed to think mestinon or another medicine would help, so it is pretty depressing to be heading downhill, either because of mestinon or in spite of it.

I'm so sorry it hasn't helped. From my own experience and the experiences of others on this board, I know Mestinon isn't a miracle cure for everyone (it made me feel terribly out of breath and exhausted). I know it's still discouraging when a treatment doesn't work, but at least you're in good company. The good news is that Mestinon is not the only weapon in Dr. Systrom's arsenal, so hopefully if it doesn't start to help you in a few months (they do encourage you to stick with it for that long just in case it takes your body a while to adjust to the medication) there are other options you can try.

Muscle pain is definitely a known side effect of the drug, so hopefully lowering the dosage will help your legs feel a bit better. It usually took me 5-7 days before the muscle pain/cramping wore off after increasing the dose. I'm sending your legs good wishes and hope that you'll be able to move around your apartment with greater ease soon.

 

[Jersey08]

So glad to hear, @minimus! Please keep us updated about the mestinon and whether you experience any positive effects. Fingers are crossed for you.

I had to decide last week whether to keep or cancel my appointment for the iCPET testing. After talking with 5 of Dr. Systrom's patients who underwent it and tried Mestinon, I decided to postpone the testing. Of the folks I spoke with, one felt that Mestonin had helped slightly, and the others either felt nothing or had bad side effects and stopped. Given my concerns about the kickback from the testing, I decided to hold off.

Because my blood work tested positive for rheumatoid factor, I am going to follow up with Paul Dellaripa, the rheumatologist Systrom works with. I'll let folks know if anything positive comes from it.

How did you manage to talk to 5 of his patients who took Mestinon? I am debating whether to pay the big out of pocket expense to get the iCPET (I have an appt) -- not sure it's worth if it Mestinon is not very likely to help? Wish there were clinical trial results.

 

[Learner1]

I don't see Systrom, living across the country. But, I did see a dysautonomia specialist who ran the full suite of dysautonomia tests, a CPET, and I was positive for 2 of the CellTrend antibodies.

My ME/CFS specialist and the dysautonomia specialist put me on 90mg pyridostigmine and I'm currently on a beta blocker as well, and I wemt from veing unable to stand in a grocery line or stand and talk to people and unable to e excuse more than 2-3 minutes without colkapsing to being able to walk and stand without collapsing snd to do some aerobic activity. I want to know if things will work on me and not people with different genetic and environmental factors in some RCT.

I also support exercise with antioxidants, like glutathione and amino acids, like BCAAs, and NAD+, hydrocortisone, and T3/T4.