@MikeO, @Pistol, thank you for the info.

I am contemplating asking my rheumatologist or autonomic specialist to contact Dr Novak in the US and ask him if he uses any immune treatments on patients with OCHOS, or what he does with patients like me who have trouble tolerating vasodilating meds.

I know he used IVIg on one patient with OCHOS, because he published a case study about it. So I suspect he might use “lower level” immune treatments as well.

Problem is my autonomic specialist is very unconfident and fears going outside his comfort zone. Rheumatologist is more likely to be open to the idea, but as I am a new patient I will hold off for a bit before asking for favours.

@mehaller, I do hope it helps. Fingers crossed for you!

@MTRJ75, that is very interesting about the carotid scan. I seem to recall that one research paper on cerebral hypoperfusion during TTT used four measurement points on each subject, including the carotid. I imagine it was because researchers wanted to see whether the points differed. If I remember correctly, all points showed similar reductions in blood flow velocity during the tilt test.

(Unfortunately, I can’t find the paper right now. I think it was one of the ones by Visser, van Campen, Rowe, et al.)

However, in my reading of papers a few months ago, most researchers seemed to use the middle cerebral artery. Not sure why. Might just be the most convenient. I did read that, in perhaps 10% of patients, the thickness of the skull and other normal variations in anatomy make transcranial Doppler imaging impossible. But maybe they can be scanned at the carotid instead of on the head?

I think you are right to assume that POTS could be the cause of the low blood flow finding on your scan. Do you remember how the test was done, lying or sitting? (Just an idle question on my part.)

I have been nagging my specialist to order me transcranial Doppler testing, medicated and unmedicated, to prove that I have cerebral hypoperfusion that resolves with vasodilators, which basically proves OCHOS. He has not been keen. He kept saying “Oh, but they might say no” and I kept saying “I realise that, but you won’t know until you ask”. He sometimes behaves like a an unconfident teenager who doesn’t want to make a phone call about a part-time job. I recognise myself aged 17 in him. I feel for him, as lacking confidence is horrible, and he is only recently qualified as a specialist and doesn’t have his own rooms yet, but at the same time he is 30 something years old and getting paid a lot of money.

So I am whiling away this hot day in Asutralia reading about cerebral hypoperfusion while I wait for my next appointment and hope he didn’t say anything stupid to the neurology department to put them off testing me …

Probably not. I think it was done more for research purposes, to see whether cerebral hypoperfusion could be contributing to brain fog and cognitive problems in Sjogren’s patients.

If you have Sjogren’s syndrome and can get diagnosed with autonomic OI problems via a tilt table test, then you don’t need transcranial Doppler testing as well, because the abnormalities in blood pressure and heart rate are sufficient. The cerebral hypoperfusion can be inferred as going along with POTS or OH.

If you have Sjogren’s syndrome with OI problems but nothing shows up on a standard TTT, then having a TTT with transcranial Doppler ultrasound added on could help by showing whether your cerebral blood flow is dropping independently of your blood pressure and heart rate. Then your doctors would know to pursue treatments that might help, such as volume expansion.

A different scenario might be adding proof of cerebral hypoperfusion to help with a claim for insurance or government support, if you weren’t getting anywhere. This might be easier to get checked via a SPECT scan, depending on the access in your area to either that or transcranial Doppler.

I know of somebody with POTS who had a SPECT scan that, even though taken supine, showed reduced perfusion to some parts of her brain. She had this in order to help her claim government disability support. I imagine it would be hard to argue with “reduced blood flow to the brain”, as opposed to trying to get bureaucrats to understand brain fog in Sjogren’s or POTS.

But this was just a single case, and I don’t know whether people with Sjögren’s, POTS or similar would necessarily show lack of perfusion to the brain on a SPECT scan. I can’t find any research on it. The person I encountered was being looked after by Australia’s top POTS specialist, who has access to resources that maybe your average specialist wouldn’t. He ordered the test in order to try to help with her support claim, knowing how unwell she was, rather than knowing that it would affect her treatment or even necessarily show anything abnormal. But obviously he must have had a hunch that it would, knowing her cognitive problems.

@CharlieJacks, welcome to the forum.

I would suggest that you start recording your blood pressure and heart rate, as well as your symptoms, episodes, activity, and any other information you think might be relevant. For example, feeling worse in hot or cold weather, or after meals.

For the blood pressure and heart rate, it is good to take “resting” readings at the same time each day as well as readings during an episode (if possible).

Resting readings need to be taken in a certain way. The dot points on this page give good instructions:

https://theskepticalcardiologist.com/hypertension-high-blood-pressure/

Also, you may find it useful to learn the meaning of the term “orthostatic intolerance” and do a little reading on it.

Good luck taking the first step with your GP and please do come back and ask more questions if needed. And folks here are always ready to offer support too.

@MaineDoug, I’m so sorry you are having to deal with other people’s poor communication and lack of co-ordination. In the olden days, I suppose a good GP/PCP would take care of all this, but they seem to have died out.

Your endocrinologist sounds awesome!

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.