Sarah Tee

The joint pain in my hands is definitely reduced. This was not a severe problem, as it only showed up when doing weight-bearing exercise (e.g. push-ups) or when I did a lot of work with my hands (e.g. long day of gardening or cleaning). Not that I’ve been doing any of those activities lately, but before my OI got bad I noticed my hands would hurt when I attempted it. I don’t think I ever even mentioned the pain to a doctor until recently when I finally got referred to a rheumatologist and made sure to think of everything that could be rheumatological. I have had swollen PIP joints for many years, but again not painful. Just means I can’t wear rings from when I was younger. I also wonder about my hands overall, as, although my hands are average size, I can never get “one size” bracelets on. So I can now theoretically do push-ups or hand-stands. Ha! When I was younger and healthier and might have comtemplated such a thing, my hands stopped me. Now my hands are much better but I’m exhausted and out of shape from years of OI. The gods have an interesting sense of humour …

@MaineDoug, that is interesting to know. In another couple of months, I will ask my specialists how they feel about sending a general enquiry about medications and auto-immune treatments.

@Sea otter, that sounds similar to Australia. A lot of treatment is free, or subsidised, but there can be a long wait. I hope you can manage to get the additional tests you need.

I noticed that researchers at the Karolinska Institute in Sweden will be measuring blood volume (among many other variables) in a study on exercise intolerance in long COVID, and it looks like they will be using the Detalo Health device: https://clinicaltrials.gov/study/NCT05445830?term=blood volume rebreathing&page=5&rank=44 I hope this will improve the understanding of exercise intolerance for all of us who suffer from it.

Thanks, @MikeO! I think I am past the worst of the initial side effects. Now to just slog on for another 2.5 months and fingers crossed it does something!

@Sea otter, I struggle with my brain too. If a doctor (or any person e.g. bank clerk, government employee) talks to me in a helpful way, I am usually okay. But if they start saying odd or unfounded things, my brain freezes. It’s an awful feeling. I’m sorry you haven’t had any success with treatments so far. It’s really hard to get a diagnosis but then not be able to make progress with treatment. I live in Australia, and testing and treatment options here are limited. I know it’s not always easy for folks in the US to access the testing and treatments they need, but at least they know that they exist. I find myself daydreaming about winning the lottery and flying to the US for treatment!

@Pistol, I’m glad it helped with the joint pain and that it didn’t come back. Funnily enough, I think it may be helping me with some joint pain in my hands that dates back about 20 years, to the same time my OI symptoms started. The muscle aches that seemed to be a side effect have settled down again. I’ve made it to two weeks – yay!

@Caterpilly, hmm, I haven’t heard anything that suggests Dr Novak’s ideas on cerebral hypoperfusion are in any doubt. His research in this area dates back some years, and there have been other big names working before him on the same theme that are referenced in his articles. Funnily enough, a group of South Korean researchers published a study that identifies the same disorder as OCHOS, except calls it OINH. Their study and Dr Novak’s OCHOS one both came out in 2016. I don’t know whose came out first, or if they knew about each other. So in a way they replicated each other’s findings of the condition (although not the studies because the methods weren’t identical). Anyway, I dare say they’ll all “duke it out” in the journal pages if there is anything different to say 🙂

@Pistol, may I ask, did you take a loading dose when you started Plaquenil?

@MikeO, yes, plenty of fluids. I have had aching leg muscles as a side effect from several medications. I think I’m prone to it! On the plus side, no nausea or vomiting. Phew!

The muscle aches are bothering me a bit. If it wasn’t summer, and I didn’t have heat intolerance, I would have a warm bath. It’s not even a fortnight yet – I thought it was well over.

I’m finding it a bit hard to have four doses a day separated out enough and accompanied by a decent amount of food. The last one is the problem. I don’t want to be eating near bedtime. So I tried having 50mg with breakfast, 50mg with lunch, and 100mg with dinner. But this wasn’t successful. 100mg at once made me feel blah. Back to four doses a day. Will try to get up early to move everything forward in the day. I’ll try the 100mg at dinner again in a few weeks.

@Pistol, in my case, the cerebral arteries are constricting and stopping the blood getting to my head due to something in the immune system interfering with the normal vasoconstriction/vasodilation process. At least, that’s what Dr Novak has hypothesised. And that’s why vasodilators make me feel better. It’s also why I feel better on steroids and have a partial remission afterwards – the steroids damp down my immune sysytem so it is no longer interfering with the normal blood flow to the brain. Funnily enough, lying upside down improves the blood flow temporarily, maybe just by forcing more blood through. So maybe giving me a lot of IV fluids would end up forcing blood through just because I was hypervolemic (but of course no-one would do that because it is dangerous). It would be interesting for me to have a tilt table test and get tilted the other way, head down, for a minute or two, while having the Doppler ultrasound. I understand that other people have problems with cerebral blood flow for all sorts of reasons. Bodies are complicated. I apologise for giving the impression I was describing what happens in everyone with low cerebral blood flow. My explanation is only applicable to hypertensive-type OCHOS, as described by Dr Novak and borne out by my personal experience with medications, infusions, etc. The fact that IV saline didn’t help me was just one clue gathered over two years leading up to me being diagnosed with OCHOS, and not responding to IV saline doesn’t mean you have OCHOS, because some people with OCHOS have low blood pressure and signs of hypovolemia, so they could well respond to IV saline. Other folks will of course have to go on their own odyssey to figure out what is happening (or have already been on it and can offer suggestions from their experience). Sorry for the confusion.

@Caterpilly, I’m so sorry to hear that Dr Blitshteyn wasn’t any help. What a disappointment. I apologise for bringing back an unpleasant memory for you. I wish I could wave a magic wand and introduce quality control over doctors. And also clone a few of the good ones. *** I didn’t get any benefit from IV fluids, which was a big puzzle at the time, but makes sense now that I know that my problem is my body restricting blood flow to my head rather than low blood volume. Some people with refractory POTS have had success with IV albumin. It ”sticks” better than saline and has a mild immunomodulatory effect. I have a long thread about it on here, with links. Just search “IV albumin”. It has to be ordered by a specialist, and has risks beyond those of saline because it is a blood product. It is reasonabley well established as a treatment in Canada, but not really anywhere else. I think it happened simply because one specialist there decided to try it and a few of his colleagues followed suit after seeing it was effective. I’d guess there are about 15 to 20 patients receiving it, many of them teenagers or in their early 20s.

Better day today. No muscle aches, almost no headache, no generally horrid feeling in afternoon. Feeling slightly overstuffed with cushioning foods (ha ha). Reading a bit about Plaquenil. Scientists don’t quite know how it works. Feel vaguely hopeful in that some of the things we know that it calms down seem to be related to abnormal vasoconstriction (and maybe vasodilation too). I cannot seem to get into reading this stuff and may have it completely wrong. A little learning … etc. etc. Has anyone written a book for the layperson on the immune system that makes it literature, rather than a jumble of letters and numbers? My dad reminded me that my Uncle Tom caught malaria while serving in the Far East after the war. My dad, the youngest brother, remembers him talking about it and sometimes being unwell after he returned to Scotland. I imagine he had to take quinine. I hope he didn’t suffer too badly.

@Caterpilly, my symptoms are not exactly linked to being upright. Mine run according to the time of day, and once they start, lying down doesn’t make a big difference. I do feel worse if I stand still while symptomatic, of course. (No headaches.) There is research into autoimmune diseases showing a daily pattern, so I just figure that’s what is happening with me. There has been some research on patients with chronic fatigue syndrome who have low cerebral blood flow showing that it did not resolve immediately after they were lowered down to flat again from tilt table testing. So although being upright is important, it obviously isn’t the only factor. I think many folks here feel unwell on waking or wake up during the night feeling unwell. I go through phases of feeling shocking when I wake up, or feeling okay until I eat. *** Change of topic: Have you ever managed to get IV saline, and did it help?

@MaineDoug, I hope you can get back to Humira soon.

@Caterpilly, maybe you could go on a waiting list to see Dr Novak. Even when you are ill and travel seems impossible, you never know how things will be down the line. He’s written a couple of articles on testing, which you can find on PubMed, and the department has something called “The Brigham Protocol”, which sounds like a 1960s spy novel. It’s discussed towards the end of this article: https://www.brighamhealthonamission.org/2019/06/03/expanded-autonomic-testing-helps-to-pinpoint-causes-of-orthostatic-intolerance It’s basically all your usual autonomic testing as done at a specialist lab with cerebral blood flow velocity (measured with Doppler ultrasound) and end-tidal CO2 added on to the tilt table testing. I don’t know that he is particularly into the immune side of things, but he would have colleagues to help, I am sure. Dr Tae Chung at Johns Hopkins and Dr Blair Grubb at the University of Toledo seem to be involved a lot in immune aspects. Dr Svetlana Blitshteyn also has an interest in that area, although she doesn’t take insurance and writes up a treatment plan that you need a local specialist to follow. (She does do telehealth.) I’m sure other folks can chime in on who or where might be good.

@MaineDoug, sounds like that saying “If you want something done, ask a busy person”. I am glad you have such a conscientious person on your team.

I am now up to the full dose prescribed by the rheumatologist, which is 200mg. Managed it quicker than I thought I would. Starting to have vivid dreams, but they aren’t unpleasant. Last night I was working as a desktop publisher (an unfulfilled career goal of mine) and Grant from Mythbusters was at the next desk. We started chatting animatedly about our favourite books and how much we had in common and got told off by the boss for “Doing nothing but talking for half an hour”. (I had been watching Mythbusters before going to bed.)

@Pistol, @MaineDoug, thank you for the encouragement.

I have survived three doses of 50mg: breakfast, lunch and (late) dinner. I get a “peak of unpleasantness” in the afternoon after the second dose. Bit of a headache, muscle ache all over, feeling seedy. Lasts about two hours. A shower would probably be soothing, but I’m too tired to manage one during the day, so I just take OTC painkilkers (which may or may not do anything) and try to ride it out. I always feel my worst in the afternoon, so this may be why I feel the effects more then. I can see that taking the whole dose and falling asleep might work, but I hate the idea of waking up feeling sick in the middle of the night, so I won’t be trying that. There’s nothing to do at 3am and no-one to make me a cup of tea! GI disturbances are continuous but bearable if you don’t have to leave the house.

@Caterpilly, yes, my diagnosis is OCHOS, which is low cerebral blood flow with no other problems (no tachycardia and no blood pressure changes on standing). I do hope the Stat gadget will be helpful for you. I would love one, but I live in Australia, so I’ll have to wait and see whether they release it here. Dr Peter Novak is the researcher. I don’t think he has found any antibodies for OCHOS – I think he is getting clues from people’s history that point towards immune involvement. He did try IVIg successfully on one patient who had OCHOS plus a lot of other issues, but I think the person was so unwell that they just decided to try IVIg knowing it was likely to help in general. (No mention of antibodies in the case study article, but I think it was written in a bit of a rush.) https://www.sciencedirect.com/science/article/pii/S2405650220300551?via%3Dihub In my case, I worked out that my symptoms were autoimmune based on partial remission after steroids, feeling better after IV albumin (it has transient immune modulating properties), and having another autoimmune disease start at the same time as my OI symptoms (ulcerative colitis). I do respond to vasodilators, such as calcium channel blockers, but am yet to find one I can tolerate at a sufficient dose. Right now I take a low dose that gets me out of bed at least. Hoping the two-pronged approach will work: a) Plaquenil reducing the antibodies or whatever is abnormally constricting my cerebral arteries, and b) vasodilators to reduce the constriction. (Some people with OCHOS have abnormal cerebral vasoconstriction, which actively stops the blood getting to the head. Others have low blood volume and blood pooling in the lower body, so enough blood just doesn’t make it to the head. I’m the first camp.) Sorry to hear mestinon didn’t help. It can be a long slog trying to find something that works.

Found a trial of a biologic called efgartigimod in post-COVID POTS patients: https://clinicaltrials.gov/study/NCT05633407 Efgartigimod, also known as Vyvgart, is an antibody fragment that binds to the neonatal Fc receptor (FcRn), preventing FcRn from recycling immunoglobulin G (IgG) back into the blood. The medication causes a reduction in overall levels of IgG, including the abnormal AChR antibodies that are present in myasthenia gravis. (Sorry, I can only copy and paste!) It was developed to treat myasthenia gravis. The study, which is being conducted by the manufacturer of the drug, states: “Efgartigimod may be a viable treatment option for patients diagnosed with post-COVID-19 POTS because it has been shown to reduce IgG levels, including IgG autoantibodies, which may underlie some of the autonomic disease manifestations in these patients.”

@Caterpilly, my prednisone was 25mg per day, which was given to settle an itching/hayfever attack that I coukdn’t get under control by other means. I hope the prednisone doesn’t make you feel too awful. If it does, maybe you can try hydrocortisone instead. (I should mention that I don’t have POTS, but something in the “POTS family” that has been described as probably autoimmune by the researcher who discovered it. He’s not my doctor, I just read his research.) I think it’s a good idea to start medications separately when possible. I’m writing a separate thread called ”The Plaquenil Diaries” to document my progress in case it is of help to anyone.