David Systrom, researcher, Brigham and Women's Hospital, USA

I managed to see the doctor in question, after making a very big stink and filing a Human Rights Commission Complaint.

Initially I was refused an appointment and this was indicated in a form he had created that (iirc) specified 3 groups he would not see: those with a psychiatric diagnosis, those with chronic fatigue syndrome / fibromyalgia, and a third one that I cannot recall currently. He had checked the box next to 'chronic fatigue syndrome' thereby denying me an appointment.

During my appointment I asked him why he refuses to see ME patients and he denied he does that. I than asked how many ME patients he sees each year - he said one or two.

He was a stereotypical arrogant physician who only attended the last few minutes of my appointment. When he came into the room he rudely interrupted the nurse who was sharing info with me. She was not happy with his behaviour and I could've cut the tension between them with a knife.

 

Blog from Bateman Horne Center on this

http://batemanhornecenter.org/systrom_cpetresearch/

 

Yes, I am very intrigued with the preload failure as it fits with cardiac research in ME going back nearly two decades, or more. I think Cneney was finding such issues in the 80s, but only on standing patients, supine patients were almost normal. This also fits with OI and similar issues, including perhaps EDS.

 

I was prescribed Mestinon and had awful side effects as low as 7.5mg (sore muscles and feeling quite ill) I'm gong to try a much lower dose (like 1mg) and only increase if I feel no side effects. Hopefully I'll build up a tolerance like I successfully did with LDN (that took about 3 months)
Anyway I think thats what I'll try instead of giving up on a medication that may still work. Has anyone had a similar experience?

 

I was prescribed mestinon by my neurologist as an additional "test" for myasthenia gravis. I didn't respond to mestinon, and was at a fairly high dose.

I didn't have a bad reaction either. I just felt overall a little worse on mestinon.. a bit more "malaise".

 

David Systrom’s trial now on clinical trials.gov. Reiterating what he explained on the ME alert video. Interesting stuff.

https://www.clinicaltrials.gov/ct2/show/NCT03674541

 

Could it affect the eyes' light sensitivity? Or is that something different?

 

That's how I understand it, although I may be interpreting it. There's a threshold at which any sense will fire up saying "too much, signal pain", and it is probably lower with us, or some of us. Which is all pretty similar to how it is with the flu or any other acute illness. It's probably this idle "sick mode" our nervous system goes into to force us to rest.

I don't know how common it is. I have it for both light and sound. Up to about 3 years ago I was still able to listen to music and tolerate noise. I barely can anymore, which is particularly cruel for a musician. It just hurts, even at a barely above speaking voice volume. That's definitely not normal and clearly something is firing up the neural network about there being pain where it shouldn't. Sometimes I can, it still hurts, but it's tolerable and so damn worth it.

It would be so amazing if it could be managed. Listening to music doesn't use up much energy. Being unable to do that is a real kick in the gut for people who basically have as many fingers as things they can do for fun on a daily basis.

 

Might be. Vitamin B2 deficiency is mentioned as a possible cause of dry eyes and light sensitivity. As vit B deficiency was common among PWME in the metabolite test at Stanford, and also in peoples OAT tests, I think we should make sure to have enough of it.

 

Thanks, Jade.
Did I get it right that the dose used in the trial is 60 mg in a single dose daily? I´m taking Mestinon since some weeks and that would be a very high dose for me. My muscle weakness is gone for about 4 hours after taking 10-15 mg. If I take more than that the typical side effects occur; running nose and eyes and sweeting to mention some. The half life is short so after about 5 hours my muscle weakness is back and I need another dose ( I take it three times a day). I find a single dose of 60 mg high and odd in a trial. But maybe there is a reason for this or I got it all wrong.

I read on the other forum that some PWME need a daily dose as high as 180 mg divided into three, but that seemed to be exceptions.

 

When my neurologist prescribed a trial of Mestinon, I started at 30mg three times a day . I quickly ramped up to 60mg three times a day (180mg/day). Only when I didn't respond at 180mg/day did my neurologist have me taper off Mestinon.

 

No, EMG does not detect Small Fiber Neuropathy. A skin-punch biopsy is what is usually used to test for SFN. I had it done last year in my neurologist's office. The samples then have to be sent to a specialized lab to be processed & read.

 

So, now I remember it:
2 out of 3 criteria have to hold:
1) SFN symptoms
2) skin biopsy of the upper leg and lower leg/foot is positive
3) positive QST (quantitative sensory test).

 

@Helen - it looks like they’re doing a single dose of 60mg in the trial vs placebo and testing it’s response after the cpet. Maybe during recovery cycling, it’s a bit unclear.

I wonder whether they are using a single dose big enough to show an effect in their trial design. I assume it doesn’t mean this is the dose they may routinely recommend. I think one of their earlier papers showed patients took doses ranging between 30mg-180mg/day. Most likely divided or as a single prolonged release version.

https://www.atsjournals.org/doi/abs/10.1164/ajrccm-conference.2016.193.1_MeetingAbstracts.A5664

It’s great to hear your muscle strength has improved. Any improvement is welcome in this disease. Does anyone else take pyridostigmine for muscle weakness or PoTS and respond well to it?

 

@jaded I see, although I find the test a bit odd as it is mentioned that a too high dose might cause a muscle weakening in some people. It seems to me as if the dose is highly individual and should be optimized. But Dr. S surely knows what he is doing. Thanks for the article. I´ll read it later.

Interestingly the experienced Dr. Peterson mentions Mestinon for POTs at 47:00 - 52:00.

https://www.youtube.com/watch?v=Eq7UwBS-dFU

Maybe we are going to much off topic @rvallee ?

 

You can add Microneurography to that list. I had it done yesterday and it found evidence of autonomic SFN.

Really fascinating technique, I wondered why it isn't used more given some of the limitations of punch biopsies but now I understand, it is a very skilled procedure and very time consuming - seems more appropriate for research setting than clinical. It involved 2.5hrs of sitting very still, getting little shocks from electrodes inserted into the nerves of my ankle and foot

It was very smart though, the software was able to pick up several nerve responses simultaneously, then by looking at the response characteristic the different functional types of C-fibres could be isolated and examined. At one point they found the nerves that respond to hot/cold, you could see the change in electrical response as the doctor warmed/cooled the skin

In my case only the autonomic fibres were affected, they couldn't find any evidence for my leg pain being SFN related. Early this year when the appointment got booked I was experiencing classic SFN-type burning pain & numbness, but that has since resolved in the months after starting methylated B vitamins, so perhaps that was a separate issue altogether. Good to have some diagnostic proof of autonomic fibres being damaged though...will bring it out the next time a doctor tells me I am just deconditioned

 

@Ryan31337 Hey Ryan, I think I may have asked you this before but, who are you seeing and is it on the NHS? I remember you saying you received good service in London and I thought that I may have been referred to the same clinic as you but it turned out to be a different, and so far pretty useless one.

 

What's that?

I was just citing an ANS doctor, I cannot say if it is accurate, but this seems to be consensus. Maybe microneurography is too "modern"/new? I understand it's used in research.
(But honestly, I wouldn't want to do it. It's needles and electric currents.)
(Sitting 2.5h still? Would be too much...)

Interesting indeed. So microneurography combines skin biopsy and QST in a way, correct?

 

Hi @sb4,

Yes, its an Electrophysiologist at Kings NHS trust. He seems to get the whole POTS/EDS/MCAS/SFN constellation and refers on as appropriate, whilst keeping you on as a patient to oversee and manage. In my experience he hasn't paid any attention to my prior CFS diagnosis nor has he pushed exercise therapy. I understand he is a victim of his own success though and can now only accept referrals from your local cardiologist, who he asks to arrange testing with in advance - PM if you want his name.

You're absolutely right @Inara, what you list is consensus for diagnosis. Microneurography is just an alternative that is only very recently being used in this context, and isn't that practical for clinicians so probably won't see wide adoption.

The guy that performed my investigation was originally looking at Fibromyalgia patients a few years ago and discovering that many of them actually had SFN (which seems to mirror the findings of Systrom). Now he's been working with my cardiac doc looking at the POTS patients. Here's a couple of articles if you're interested:

https://onlinelibrary.wiley.com/doi/abs/10.1002/ana.24065
http://www.bscn.org.uk/abstract-details.aspx?id=85

Although long, the test wasn't that bad, I actually nodded off at one point and was shouted at because the muscle twitching you experience when you fall asleep was messing up the results

Inserting the bigger needle wasn't any worse than a blood draw, and the shocks from the electrodes were just like poking your skin with a pin over and over, nothing major. Kind of similar to the Single Fibre EMG i had previously. Was a bit odd having someone stroking and blowing on your toes quite a bit though

Ryan