Sarah Tee

A good first step would be to keep a log of your relative's fluid and salt intake and the dehydration episodes. Record their heart rate, blood pressure and symptoms as well. This should help their doctor work out what is going on and you may identify a pattern that is bringing on these episodes. (I wasn't quite sure when you said "no diagnosis" whether you meant POTS hasn't been diagnosed or the high blood pressure hasn't been diagnosed. Either way, best to speak to doctor/specialist about it all before trying anything that could affect their electrolyte balance or BP.) Best wishes for getting it sorted out.

Thanks @MikeOand @Pistolfor your replies. I was quite surprised today to hear from my specialist that he has arranged for me to try one session of albumin plus saline. There's so little documentation on it out there that I expected him to refuse. It will be on Monday week. It is a long shot. I will report back. Even if it doesn't work for me, at least my specialist and the registrar at the hospital now know about it as a possible treatment. Who knows, they may use it in future on someone with really bad refractory OI/POTS. If it does work for me, I will ask my specialist to write me up as a case study! Here's a story about a young woman in Canada being treated by Dr Siddiqi with albumin: https://www.cbc.ca/news/canada/saskatchewan/katherine-kay-diagnosis-genetic-testing-1.4260019 I wonder how she is doing now. *** My auto-immune panel came back normal, but it was a basic one. Thank you for the study link, @Pistol. I will need to read up a bit more. I have heard of the innate immune system but my knowledge in that area is sadly lacking. It's hard trying to learn about all this without much background in biology or medicine. I noticed that the video lectures from this year's Dysautonomia International conference are trickling out. I should have signed up, it wasn't expensive. (Maybe I still can.) There might be some more on the immune aspect. *** Hope everyone has a decent weekend. I am going to help my dad with a small DIY job. I sit nearby and pass him things :-)

I have been searching and searching to try to find information on this topic because my saline infusion trial sadly failed to produce any improvement. It's a long shot, but maybe the fluid is leaving my blood too quickly to be helpful. As I understand it, some people with refractory POTS respond to albumin infusions after not responding to saline infusions. The reason for this is probably that the albumin keeps the extra fluid in the blood (for a while at least). So here is my question: Do people receiving albumin infusions for OI have low serum albumin, or is that unrelated in this context? I can see it might be unrelated, because if you add saline plus albumin, you are not actually increasing the proportion of serum albumin because you are adding it in tandem with extra volume. I looked through a cancelled trial that was supposed to test albumin infusions, but there is no mention of low serum albumin in the inclusion criteria. They were going to test people with severe POTS who had abnormal sweat testing results and who had not responded to the usual treatments. https://clinicaltrials.gov/ct2/show/NCT03365414 I am wondering whether to push my specialist for this. I found going for the saline infusions exhausting. Then again, if it works, I won't find it exhausting! I have another avenue to explore, which is auto-immune testing / seeing a rheumatologist. (You might remember I posted recently that I felt completely back to normal while taking a five-day course of prednisolone, and that my symptoms were reduced for about two months afterwards, with fatigue gradually returning.)

I agree that it sounds like the type of visual disturbance that comes from not getting enough blood supply to the brain or optic nerves. I think people experience a wide range of visual disturbances caused by slightly low blood supply. For instance, I have the sensation of not being able to see very well when I'm symptomatic, but it's a sense of not seeing well with my brain rather than my eyes not working. Recently I had a very unusual (for me) remission during the day, and it was like I could suddenly see the world again properly. It was very disconcerting. It was like when you are tuning a newer car radio and it is off station and a bit fuzzy, then suddenly it snaps onto the right frequency. I suppose the medical term would be mild cognitive impairment. Even though it's a year's wait, the docs at Brigham seem to put out a lot of good research and they seem to do some types of testing that are not offered elsewhere. Dr Novak at Brigham seems to be the only person publishing on non-POTS orthostatic intolerance (although he covers POTS as well). I also noticed his name on a couple of recent studies looking at mast cell disorders, SFN and autonomic symptoms.

@MikeO I am so glad to hear that the lower dose seems to be helping you and also not harming you. If you can tell when it is wearing off, that seems like a very positive sign. I do hope you can continue taking it and experiencing the same benefit. Your quote "nothing bad happened" resonated with me. For most people, this is a normal (subconscious) expectation for their day. For folks with chronic illness, "nothing bad happened" is a day that is a gift, even a shock, that they might look back on wistfully for months if it can't be replicated. Re albumin infusions, I found a small Canadian study presented as a poster in which the researchers used albumin to treat refractory POTS (I don't have POTS, just undifferentiated orthostatic intolerance, but similar symptoms apart from the tachycardia). https://www.cambridge.org/core/journals/canadian-journal-of-neurological-sciences/article/p040-efficacy-and-safety-of-periodic-albumin-infusions-in-refractory-postural-orthostatic-tachycardia-syndrome-a-comparative-study/60BFDDF0558BE61F70799DEE202A0AE9 What is interesting in the poster presentation is that it seems that there were some patients who did not benefit from saline infusions but did benefit from saline plus albumin. I wonder what this means. Did these patients lose the volume from plain saline so fast that it couldn't help them, but the albumin made it stick? Or is the albumin doing something else? There was supposed to be a full clinical trial on albumin from the same people that did the poster, but it was marked as withdrawn in July this year. I wonder why. I tried emailing but no response. https://clinicaltrials.gov/ct2/show/NCT03365414 @Jyoti, I suspected that the saline infusion wasn't going to work, but, you are right, it was still a disappointment. I seem to fit the profile of low blood volume, so the fact that none of the volume expansion strategies/medications have worked for me so far is puzzling. At this point, I just wish someone could measure my blood volume so I could know whether to move on or keep experimenting in this area. If I win lotto, I will pay for someone to fly to Australia with a blood volume machine and install it in the nearest capital city. I will give Mestinon another try next week. The bottle I got contains 150 pills, so plenty to try again! Going to get some blood tests done first, just in case diarrhea strikes. I'm so glad it is helping you.

Hello @MikeO, Sorry to hear you are having trouble with side effects. I hope you can get it sorted out. Bleeding is not one of those side effects you can brush off. I finally had my second saline infusion yesterday. It was a fairly pleasant experience compared to the first one I had earlier this year, although a few things still went wrong. The main problem was that it didn't seem to help my symptoms. Maybe a tiny bit, but nothing like what I read people who do respond usually feel. I think I am now officially "treatment refractory". I am going to try Mestinon again sometime in the near future. I would also like to try albumin and octreotide. Do your doctors have any more options for you if you can't continue with Mestinon? I do hope so. My current specialist is making noises about "nothing left to try" (that he will prescribe, at any rate). I do have another specialist to try, so not out of options yet.

Hello @Bailee, Regarding the cancelled appointment – after a week of not hearing back, it seems appropriate to call and ask what is happening. I recently had a similar experience with appointments being cancelled multiple times. Each time when I phoned to follow up it turned out that my appointment had been "lost", even though the person who cancelled it promised it would be rescheduled. Also, I know it's hard when you aren't well, but it's a good idea to keep a diary. Always get the name and contact number of anyone who phones, and ask when you should expect any action, and make a note of it, e.g. "Call from Fred Smith, X Clinic, appt w Dr Jones on x cancelled. Expect reschedule in one week. Call back if you haven't heard by date x." Then make a note a week later to phone back if you haven't heard anything. Regarding the "pass the buck" with your bloodwork ... this is a bit sneaky, but I often find that you will get a different answer from different staff members. On Monday Nurse A will tell you to get your bloodwork done with your PCP, but on Wednesday Nurse B will say "sure, send it in" and help you to arrange it. Maybe worth a try. The admin with dealing with medical appointments can be more stressful than the disease sometimes. I hope you can get it all sorted out. Another option is to phone the patient liaison officer (or similar). I have had great help from the one at my local hospital.

I am so glad to hear that. I have been helping with a petition to improve access to IV fluids for dysautonomia patients in Australia because the situation is so confusing here - even when your specialist has ordered treatment it can be impossible to access. My new chap seemed to think there would be no problem with me getting fluids at his old hospital, and told me at the last appt that it was all organised. I phoned the day unit today, and, sadly, it is not all organised 😞 The people I spoke to were very nice, and they are going to phone him, but I think he may have been a bit naive about how hard it is going to be.

Thinking aloud a bit ... Just searched to see whether corticosteroids have vaso dilatory or constrictive action, and there seem to be mentions either way, as well as references to inflammation, cytokines, peripheral vasoconstriction and vascular tone. Interesting, but well beyond my reading level. But again, I can't see this effect lingering on after you have stopped taking them, unless there's also dampening of the auto-immune system involved. Sure, why not. We know that autonomic conditions are complicated.

Thanks, @Pistol. My GP may be persuaded to let me take it for a week once a month, or once in three months. I don't know. She talked about how she looks after patients with severe asthma who take steroids on that kind of schedule. Thanks for the info from the talk. I will ask about vasopressin.

Just wanted to add that I have slightly high blood pressure, although only in the past five years, and my symptoms have been around for 18 years. I don't get low blood pressure, even when I have presyncope or take an active stand test. Also, the prednisolone did not appear to increase my blood pressure. It did however let me feel well enough to go out ... to the dentist. Sigh. Wish I could have done something more exciting on my little "symptom holiday", but I didn't know for sure it was going to happen.

Cortisol has been a dead end for me. I have been tested every which way for years and am always in the middle of the reference range. Is Cortef hydrocortisone? It wasn't a replacement dose, because I've been shown not to be deficient. It was only a small dose, less than 10mg. The endocrinologist who gave it to me was unable to explain why he had done so, despite me asking him repeatedly, and I've come to believe it was a negligent action because of my normal test results and his lack of clarity. Anyway, I won't be taking it ever again! I now have an excellent endocrinologist and she is satisfied nothing is wrong adrenal wise or in other endocrine areas. I trust her completely. I just don't think the benefits I happen to get from corticosteroids are related to cortisol. For example, it is now five weeks since I took the short course of oral prednisolone, and my symptoms are still improved from my usual baseline. But people with cortisol insufficiency have to replace every day, because cortisol gets broken down/used up by the body. It just doesn't fit. I will ask for aldosterone and renin testing. I did ask a while ago but it never happened. I am having a bit of a hard time with my new specialist. He ordered a bunch of tests without talking to me about them at the appointment. Many of them I had already had recently and multiple times over; others turned out to be useless because he didn't tell me what they were for. Thanks for listening - this is all rather lonely and frustrating for me.

Yes, I thought that too about volume expansion. However, I checked and found out that corticosteroids such as prednisolone only have a small effect on blood volume compared to fludrocortisone. (Someone on another forum directed me to a chart that showed the various actions.) I have tried fludrocortisone very thoroughly twice with no improvement in my symptoms. I have tried midodrine and it didn't help, unfortunately. I did get the goose bumps though :-) Basically, I have now tried every drug that specialists here (Australia) will let me have and nothing has worked. I have also tried every non-drug intervention. I am waiting to try IV saline, but, given that I haven't responded to any other volume expansion measures, I am not holding my breath. It is odd that the only thing that has helped me is steroids for an allergy (and I don't have MCAS, just your common or garden allergies). My GP thought it was interesting, but the specialist didn't. Anyway, thanks for listening! It is hard trying to work out these things on one's own. I wish I could fly to the US and see one of the "top guys". Have got my lotto ticket in for next week ...

About a month ago, I was prescribed a short course of oral steroids (prednisolone) to put a stop to some bad allergic itching (just the "normal" kind, I don't have MCAS). My orthostatic intolerance symptoms greatly improved. For example, I was able to stand and wait for several minutes outside a cafe, something I could never normally do. I also felt close to normal energy and went for a walk around the lake near my doctor's and could have kept going if my companion hadn't got tired. I did have some side effects, but they were minor compared to how much better I felt without my OI symptoms. At times during the day I felt completely normal for an hour or so, both while taking the steroids and in the weeks following. This was a very odd feeling for me. It was like a scratchy radio had suddenly been tuned to the right frequency, but that radio was my brain! I often feel better late at night, but have felt unwell during the day for almost two decades, so this was very unusual. I also had a previous experience having my symptoms resolve when taking hydrocortisone (although in that case the side effects were horrible). New specialist refuses to comment except to say he wants to test my cortisol levels again, and do another synacthen test, which I refused because I've already been tested every which way for adrenal problems, nothing abnormal ever found. I am now sure it is not just "anyone with fatigue might feel better on steroids" because it was very specific to my OI symptoms. It wasn't like when I tried stimulant medication, which felt more general. Specialist reluctantly agreed to run auto-immune panel, although it's probably pretty basic. Am not having it done yet because I am sure the steroids could affect the results. All very mysterious and frustrating and I just wanted to get it off my chest. I really feel this must point to auto-immune involvement, especially as the positive effects have lingered after I finished the course (five days). What does everyone think? (I am diagnosed with "chronic OI", basically POTS without the T. Main symptoms are presyncope, fatigue, horrible drained feeling in face and head, and heat intolerance. 18 years' duration. Symptoms initially morning only, but gradually extending to longer and longer until now they last from morning until about 11pm each day. No lifestyle modifications or medications have worked so far. Running out of options.)

I’m not sure whether it’s still going, but at one time doctors could ask for a free copy of The Dysautonomia Project. (Possibly patients could nominate a doctor to have a copy sent to as well?) US only.

My specialist ordered two litres of saline over four hours. I only managed to have it once, but am hoping to resume it soon. Dr Satish Raj has done a bit of research on saline’s effect on POTS symptoms. If you look at the “methods” section on one of his research papers it will probably state how much was administered over what time period. Judging by posts and blog articles from dysautonomia patients that I’ve read over the years, slower is usually better. Half an hour seems a bit fast.

Oh, I really hope it does help eventually. Do you have have it at Dr Novak’s clinic, or can you have it at your local hospital if you are not near Brigham? I hope it’s not too gruelling to access. I am still puddling away in Australia trying to get my basic saline infusions, which were ordered in February.

I hope it goes better for you this time. I may try again at another time. It made me generally unwell for about a week, then that passed, and it seemed to do nothing. I stopped taking it because I needed a mental break. I am seeing a new specialist and it has been hard because he doesn’t know my situation yet. I suppose I am glad that I tried Mestinon, although I am still confused as to why the specialist prescribed it. I recently felt much better after a short course of oral steroids for allergies (unrelated to dysautonomia), so I asked him if that could indicate auto-immune involvement. He said he doubted that. But at the same appt, he prescribed Mestinon, which seems to help people who have auto-antibodies plugging up receptors. I didn’t question him on it, because I had to bring along my carer to the second appt to read him the riot act about a few things, but I will ask him some time. On the plus side, he has organised to get me a trial of saline infusions at the hospital he is connected to. I was supposed to have this trial in Feb and it never happened despite months of wrangling with my local hospital.

@RecipeForDisaster, just wanted to say that I have heard of HYCH 🙂 Were you diagnosed by Dr Novak? I try to read everything he publishes, even though some of goes over my head. He seems to be the only person researching undifferentiated chronic OI. I suspect I have OCHOS, one of his discoveries.

If eating a low-carbohydrate breakfast still causes problems, how about skipping breakfast? I was looking into RH a while ago (turns out I don’t have it) and some people with RH fast at the start of the day, or even longer into the day. One person has coffee with a slug of double cream. Of course this might simply move your symptoms to whatever meal you have first, or make you wobbly, or make your POTS worse. Just thought I’d mention it. Maybe you can try it on a day when you don’t have to go out. P.S. I forgot to mention that I used to have my OI symptoms for about an hour after breakfast. Gradually it extended further and further into the day, until now, 18 years later, my symptoms go until about 11pm, at which point I feel normal. I am still experimenting with skipping breakfast. I have felt better a few times when fasting, but also not. Trying to consistently replicate the benefits I noticed occasionally when fasting before blood tests.

I happened to find a poster presentation on this topic from 2019: https://www.cambridge.org/core/journals/canadian-journal-of-neurological-sciences/article/p040-efficacy-and-safety-of-periodic-albumin-infusions-in-refractory-postural-orthostatic-tachycardia-syndrome-a-comparative-study/60BFDDF0558BE61F70799DEE202A0AE9 (One sentence seems to have a typo.)

Thanks, @MikeO. I see now that Mestinon helps helps acetylcholine receptors to work better (oversimplifying), and these receptors are present in various places in the body, including in the muscles to control voluntary movement and in the autonomic system. Unfortunately it doesn’t seem to have worked for me.

Thanks, MikeO. So it is supposed to work in POTS the same way as it works in myasthenia gravis? I am a bit confused because, in the studies I found on its use in POTS patients, the improvements weren’t just muscle strength.

I am feeling rather brain fogged today. I read the info on it here: Could anyone explain very simply what unplugging these receptors might be expected to have? Is it just “allow your autonomic system to work properly” or is it more specific, like giving you more muscle strength?

I did put a link to the abdominal support, but apparently that isn't allowed. Sorry I can't show you what it looks like because I'm certainly not going to post pics modelling it 🙂 !!! What are you waiting on in the post, medical or otherwise?