Sarah Tee

It’s my impression that doctors have a sort of brotherhood (for want of a better word) that means they can contact each other to ask for advice about difficult or unusual cases, even across jurisdictions and borders. Am I right about this?

@MikeO, I agree, we have to work on this individually, and also get help from other patients. I know that’s true to some extent for all medical problems, but I do find it hard having no medical support to speak of. I now have three medical conditions that are rare and are only able to be treated properly by a handful of specialists in the US or Europe. I can read their research, and watch their lectures, but the specialists here in Australia appear to be less educated than I am. Sadly I can’t do my own bowel surgery, write my own prescriptions, or order my own neurological testing. Too bad because I know exactly what the research recommends! (Okay, the bowel sugery would take practice. But there is a robot-assisted version in the Netherlands!)

@MaineDoug, I’m not sure whether I had a CRP test. The rheumatologist did a basic panel of about ten things and all came back normal. I will ask for a copy of my results at the next appointment. Nobody know which antibodies to test for in OCHOS yet. Dr Novak might look at it one day, but there aren’t many of us so I’m not sure if it will happen in my lifetime. I suppose he is reluctant to prescribe anything that has risks of permanent damage or infection due to a lowered immune system because “I’m not sick enough”. I also have no aches, pains, fevers or inflammation in my joints. I am a bit of an oddball in terms of being treated by a rheumatologist. I do have an immunologist in mind as well, who was recommended to me in some patient groups as very intelligent, but that means more long exhausting car trips to the nearest city. Not silly questions at all. I am at the point where I want to pin doctors to the wall and tell them at length how this has affected my life for 18 years, how I can’t work, can’t think clearly, have emotional dysregulation, fatigue, etc. etc. It’s affected so many things. Relationships with family and friends, my career (or lack of one), stress levels, personal development, menatl health. So don’t tell me I am not sick enough!!! At the same time, the rheumatologist spoke very sensibly about the risks of infection that come with the big gun medications, not only “catching things” from other people, but also the risk from the normal pathogens on your body and in the environment that are usually no problem but could lead to hospitalisation when you are immune compromised.

After reading all the research on hypoperfusion in autonomic conditions and CFS that’s been coming out lately, I wondered if anyone had checked for it in Sjögren’s syndrome. It seems likely it would be found given the close connection between SS and autonomic problems. And sure enough, I found two studies. One goes into it in some detail, but is behind a paywall. The other is free but only touches on it as it is a review article. “Intracranial hemodynamic changes in primary Sjögren syndrome: a transcranial Doppler case-control study” (behind paywall) https://link.springer.com/article/10.1007/s10072-015-2204-3 “Cognitive Function in Primary Sjögren’s Syndrome: A Systematic Review” (available) https://www.mdpi.com/2076-3425/9/4/85 I also saw in Dr Peter Novak’s lecture at the Dysautonomia Intnl conference last year that he and other dostors from Brigham and Women’s are about to release research showing cerebral hypoprefusion in people with Ehlers-Danlos syndrome, and not just in those with POTS. (I am keeping an eye out for the paper and hope it will be out soon.)

IV albumin is another treatment that appears to have an immune-modulating effect, alongside its volume-expanding properties. (I wrote a long thread about this with lots of links that you can find by searching this forum.) It has been used with some success over the last decade or so for refractory POTS patients in Canada in combination with IV saline. Seems to be mostly given to paediatric (teenage) patients. Has to be done fairly frequently and comes with the problems of IV access.

Also, here is a an article on LDN in post COVID: https://pubmed.ncbi.nlm.nih.gov/37804660/ Presumably some of the patients had orthostatic intolerance and other autonomic symptoms. (I haven’t read the paper.) I note that Mitchel Miglis is a co-author. He has given several lectures over the years for Dysautonomia International. I believe he worked with Dr David Kem on some very interesting research in the auto-immune area. Dr Kem sadly passed away in 2020, but his ideas and research have inspired many: https://dysautonomiainternational.org/blog/wordpress/in-memoriam-dr-david-kem/

@mehaller, sorry to hear it didn’t happen, and with such a long wait. Have you felt better generally from steroids in the past? I felt better from my orthostatic intolerance and went into partial remission for three months after a short course of oral steroids. That’s what made me (and some of my doctors) realise my condition was auto-immune. Took me a year and a half to get to a rheumatologist though!

I’ll wait until after the holiday period to email Dysautonomia International, but I will definitely report back. In the meantime, I will post a few topics that people might find useful. Low-dose naltrexone (LDN) is a possibility for POTS patients, according to this article from Cleveland Clinic doctors containing six case studies. Three of the patients had a positive response: https://pubmed.ncbi.nlm.nih.gov/37706146/ There is one other case study, but the patient recieved IVIg and LDN: https://pubmed.ncbi.nlm.nih.gov/29326369/ (@MikeO, you might be intersted in this one because the patient was also treated for SIBO.) LDN appears to have an anti-inflammatory effect, plus other beneficial effects, none of which is well understood yet. It is being tried for chronic pain and auto-immune conditions. I couldn’t find any other case studies of LDN for dysautonomia, so this might be a hard sell unless your specialist is at an academic institution or established autonomic centre, or already prescribes LDN for other conditions. This rather ambitious study in Canada might change all that: https://clinicaltrials.gov/study/NCT05363514?cond=Postural Orthostatic Tachycardia Syndrome&term=ldn&rank=1 It is being led by Dr Satish Raj and will have 80 participants with POTS. That is huge for a POTS study! It is supposed to be starting January 2024 and finishing in 2026. The study will look at fatigue and cytokine levels, among other things. I would be interested in trying LDN if my trial of HCQ/Plaquenil doesn’t work out. I don’t know whether my rheumatologist has experience with it or not.

@little_blue_jay, I’m sorry you don’t have a specialist at the moment. I know there is a good POTS support group for Canada on Facebook. I joined once briefly when I was researching something. Maybe they could help. I did a quick search and there are “medi-spas” in Canada in the major cities. You could ask on the Facebook group and see whether people are happy with one near you. Some people feel comfortable using them, others don’t. (Because it’s not a hospital environment.) I don’t have POTS but I do know that many (but not all) POTS patients can benefit from IV saline in a flare. I’m from Australia and I think our health systems are fairly similar. There is good and bad in each model, I think. There are long waiting times for specialists in the public system here. I was able to go private – still a fairly long wait – because I moved back in with family to reduce costs and can use my govt pension to pay for appointments. Up to a point, anyway.

Just adding a link to the study about methylphenidate, as it is no longer available from previous link: https://pubmed.ncbi.nlm.nih.gov/20460983/ Just in case someone else is searching this too 🙂

@XxiloveaotxX, glad I could help a little. There has been a little bit of research into cognitive problems in POTS, especially to try to pin down “brain fog”, but nothing definitive yet. I think most researchers agree that low blood flow to the brain plays a part, but that there may be other factors too. Other folks here with POTS may be able to comment on whether they have had memory problems like this too.

Well, it remains a slight mystery to me why there are clinical trials, small studies and case studies of people with POTS receiving IVIg, but almost nothing on anyone trying DMARDs or biologics. Aren’t these often cheaper and easier to get than IVIg? Just wondering why specialists seem to go straight to IVIg in the immune area. I might email Dysautonomia International and ask. Maybe DMARDs and biologics just don’t target the right bit of the immune system?

@little_blue_jay, I’ve watched/read maybe hundreds of lectures and articles about POTS, and heart attack is never mentioned. One article that might help you is this one, in which researchers used various measures to look at the decrease in quality of life in 94 patients with POTS. They found that the impairment experienced by POTS patients in their lives were at a similar level to people with COPD and other diseases/conditions that are considered very serious in medical circles. https://www.mayoclinicproceedings.org/article/S0025-6196(11)61995-2/fulltext The unusual thing about POTS and other types of OI is that people can feel so awful, and be so disabled, and yet by most measures appear to “have nothing wrong” in terms of their heart, for instance, or a standard blood panel. Can your specialist adjust your medication, or make any suggestions? If you feel better after IV saline, any chance of your doctor ordering this, or getting it at a “medi-spa”? I hope you feel better soon. It’s worrying when things get worse and you wonder whether you will return to baseline.

@MikeO, yes, I have seen a case study in which Dr Novak, who discovered/named my condition (OCHOS), used IVIg on a patient. However, that person had a lot going on and I imagine was much sicker than me (probably hospitalised). I have had basic auto-immune/rheumatological testing, but nothing unusual came up. I’m glad your endo helped somewhat. Hope your insulin and monitoring continue to go well. I will keep on plugging!

@Pistol, I’m sorry you had to pay for it yourself and that it didn’t end up helping. I am lucky in that mine will be govt subsidised as I am having it prescribed for “unknown rheumatological reasons” (having had two definite auto-immune diseases and my improvement in the OI symptoms with steroids). I really hope it works for me. I am coming to the end of my options. A few more calcium channel blockers, one of which I would have to pay for myself (nimodipine), and that’s about it, unless a knight in shining armour appears on the scene. I hope Dr Grubb can get his study going again soon.

Just answering my own question: Dr Grubb is hoping to do an immunotherapy trial for POTS soon using Kevzara (sarilumab), a biologic. He talks about it in this lecture, from 2021: https://www.youtube.com/watch?v=P5xSgiwueHw (It’s mentioned right near the end.)

Oh, and I forgot there are a couple of folks here taking it too, and a couple who have tried it but discontinued.

Ha, well, I just found a comment on another forum about someone with POTS being prescribed HCQ by Dr Grubb. They say they had tested positive for some of the antibodies he has been studying. Of course it’s just a forum comment but it makes me feel a little better. Edited to add: 21 people on this health tracking website report taking HCQ for POTS. If you scroll down, there are positive comments in its effectiveness and reports of side effects. https://www.stuffthatworks.health/pots/treatments/hydroxychloroquine-sulfate I also found that HCQ is sometimes used to treat anti-phospholipid syndrome, also known as Hughes syndrome, which seems to be connected to POTS: https://www.standinguptopots.org/resources/antiphospholipid-syndrome-and-pots

As I’m going to be trying it soon, I’ve been trying to find research on hydroxychloroquine (HCQ) being used in POTS* or any dysautonomia, but so far haven’t found anything much at all. Is there a reason that HCQ wouldn’t be considered? I see studies of people being treated with IVIg, which, while often beneficial, has its risks, and wonder why they haven’t been tried on a DMARD first. Is it because DMARDs are useful for treating traditional muscle aches and pains, and POTS doesn’t really come with those? I also don’t see much on biologics being tried in POTS or other dysautonomia. (Please excuse me if this is an ignorant question. I am new to this auto-immune stuff.) * I don’t have POTS but there’s not much research on OCHOS so that’s why I’m looking there.

“Overview of Austonomic Disorders” https://vimeo.com/185276627 In this lecture, Dr Grubb gives a lot of interesting background on the history of our knowledge of POTS. Note that he does have a slightly quirky sense of humour. Stick with the lecture. By then end you will probably want to give him a hug/medal/Nobel Prize. I was especially touched by the way he must have really listened to his patients, and picked up on the unusual things that were happening, rather than dismissing it all. A true sense of scientific curiosity. Another very interesting section covered potential wrong diagnosis of epilepsy.

@MaineDoug, I’m so glad CHOP exercises are helping. It must have been so hard at first, but great to hear you are seeing results. Also – nice reference to Flat Land!

@little_blue_jay, this blog, written by a cardiologist, has several articles about smart watches that measure blood pressure. https://theskepticalcardiologist.com/category/medicalfitness-devices/ When I read about this topic some years ago, I believe it was the case that they were not as reliable as your basic home blood pressure cuff. This may have changed. Can you switch off the blood pressure function, or check it against a cuff monitor?

Well, it’s (mostly) good news. I will start HCQ in the new year. Woo hoo! He only wants to try me on it for three months, but I will tell you folks here a secret: Even if I feel nothing at three months I am going to fib because almost everything I read says six months. This is the first treatment for my condition that will be getting at the cause and I am not going to stop it until I can be sure. The not-so-good news is that he said he will not be trying me on anything else if this doesn’t work. Argh. He said the risk was too great as I am not sick enough. It’s very hard getting across how non-functional I am, especially the cognitive side. I only have one support person, an older family member. Not to be too blunt, but when he is gone, I will be alone and I cannot manage alone in this state. Accessing support is enormously hard because of all the paperwork, lack of support from my specialist, lack of a decent GP, and the endless wait times for tests. Will I ever get a transcranial Doppler ultrasound? Or a cognitive assessment? But then maybe I can persuade the rheumatologist to correspond with Dr Novak (discoverer of OCHOS). I know he has put one patient on IVIg. Maybe he has put others on immune treatments? But for now I have to not think about all that. I managed to make some pasta sauce to keep myself fed for the next few days on something vaguely healthy. There have been many days when I couldn’t do this, so I am grateful.

Thanks, @Jyoti!

Today I am supposed to be having a phone consultation with the rheumatologist I saw a month ago. I hope my blood tests have gone through – his office is not open so he must be working from home for a couple of hours. We can’t find the info about the appointment though, so not sure what time it is. At the in-person appt he said he would put me on hydroxychloroquine (HCQ), brand name Plaquenil, pending the blood tests. I really really really hope he goes through with it! The other thing is that HCQ can help with another condition I have that’s been deteriorating. It’s an autoimmune skin disease. I would probably never be prescribed HCQ for it here (Australia) but if I get it for OCHOS then that’s a double win.