Sarah Tee

I have OI with normal blood pressure and heart rate, even during poor man's TTT.

Is that how yours goes too?

I would say it is worth cautiously trying any medication that can help OI, can be stopped rapidly, isn't contraindicated by your medical history, and is unlikely to have lasting negative effects. That has been my approach with my specialist. I have tried just about everything listed below, bar a couple that are too risky, not available in Australia, or contraindicated for me.

Some of these will not be suitable for you given that water retention is a problem. Even something seemingly harmless like raising your bedhead might affect water retention. Just listing everything anyway because if your specialist raises unsuitable options you can be ready to say "Nope, not for me".

1. Increase fluid retention and/or blood volume

• extra salt and water
• raising bedhead
• fludrocortisone (Florinef) – is a steroid but different class; can cause water retention
• desmopressin
• saline infusions
• more obscure options such as EPO, albumin infusions (see list linked below)

2. Increase venous tone (vasoconstriction)

• midodrine
• some others (see list and lecture linked below)

3. Stop blood pooling in lower body

• compression garment that covers the abdomen up to the waist, e.g. tights, leggings
• clenching and unclenching large muscles (thigh, buttock) before standing and when standing
• strengthening those muscles with exercises
• more obscure options such as Octreotide (stops blood rushing to gut after meals; rarely prescribed because of risk of gall bladder damage)
• smaller meals
• lowering carbohydrate intake

4. Assorted other things

• stimulants
• Mestinon
• see video lecture Pharmacological Treatment of POTS (ignoring those treatments related to tachycardia)
• see list POTS What Helps? (ignoring those treatments related to tachycardia)
• immune treatments

Dr Peter Novak seems to be the leading person who publishes on "plain OI" (not POTS, not OH, etc.) at the moment. He has identified some new types/features of OI, called OCHOS and HYCH. (I am sure other specialists know about it and treat it as well.)

He has a textbook of case studies that includes various kinds of OI. He mentions that for "plain OI" he sometimes has success with calcium channel blockers or ACE inhibitors, which are both blood pressure reduction medications. Reason that these help some people is unknown.

If you would like to read his research, let me know and I will link to it. He did do a lecture on OI syndromes for Dysautonomia Intl but unfortunately the audio isn't very good.

https://vimeo.com/479624617

Having said all that, trying medications can be disruptive and tiring. I seem to be sensitive to things too, and trying so many has taken me a long time and been very disruptive. The only things that have worked for me so far are prednisolone (coincidentally given as a short course for allergies; docs do not want me to take it longer term) and albumin infusions (only help me a bit and inconvenient to access). I've still got a few more to try.

I can completely understand someone saying that trying medications is going to be too disruptive for me and I don't want to risk bad reactions and possibly setting my health back or going into a flare.

It might be a balancing act between:

• running it in slowly enough that it "sticks"
• but not so slowly that it is escaping from the blood circulation at about the same rate it goes in.

How about trying it over two hours first thing in the morning? Did the two-hour infusion seem to stick, at least until bedtime? (I mean, in regards to not peeing it all out quickly as well as feeling better.)

Was the trainer referring to GI (glycemic index) in labelling oatmeal as a healthy food? I'm not an expert in this area, but I recently watched a program about this subject in which an expert (probably an endocrinologist) pointed out that diabetics need to be looking at their total carbohydrate intake, and that GI being high or low was irrelevant in that particular calculation.

Glad to hear peanut butter and toast worked out. That is my breakfast of choice!

And it may be a coincidence but porridge for breakfast seems to worsen my symptoms, which is sad because I am part Scottish. (I can eat it later in the day with no trouble.)

Lauren Stiles, who started Dysautonomia International, has Sjogren's syndrome plus POTS. I remember watching a video lecture in which she spoke about her experiences, but can't remember which one it was. Maybe someone else can help.

I think it was on the Dysautonomia International Vimeo channel.

I did find this lecture, but it's with Dr Brent Goodman, not the one I'm thinking of.

https://vimeo.com/359097230

I'm thinking of asking to be tested for Sjogren's (SS-A, SS-B if I have it right) because I suspect my mother had it. I don't have the classic SS symptoms yet, just dysautonomia, plus ulcerative colitis.

I am sorry to hear you are having a bad time with your symptoms lately. I have been getting worse gradually for about 18 years now, but this last year I have felt like I am "losing condition" fast. It's a real worry.

Oh my goodness, have I just discovered something?

Quote

Prednisone decreases inflammation via suppression of the migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

(emphasis added by me)

https://www.ncbi.nlm.nih.gov/books/NBK534809/

Does the prednisolone I took make me feel better because I am losing less fluid from my blood? (In another thread, I described how I responded to albumin infusions; hypothesis is that the albumin, which comes suspended in a liquid, allows my body to hold onto that extra liquid.)

(Prednisolone and prednisone end up the same in the body, if I have it right, with prednisone being converted to prednisolone.)

Have to read this article this evening when my brain is working better!

I saw this BBC program a while ago on Australian tv and was very impressed by the doctors involved and touched by the patient stories. At the same time it made me feel sad and angry because the care the average person receives is so far below what is shown.

Someone has put it up on the website Dailymotion (a more basic version of YouTube). If you go to the website and search "diagnosis detective se1", four episodes will come up. (Only one series was made.)

It is presented by Michael Mosley, but don't be put off by that. It is a much higher standard than his other programs and he only plays a minor part in the stories. The focus is on the patients and specialists.

Without giving anything away, several of the patients are investigated for or end up having dysautonomia issues. You may also spot some of the other diagnoses before they are revealed in the program.

It is a little sad, but most of the people get a resolution of some sort.

Edit: Realised I forgot to say what it’s called – “The Diagnosis Detectives”.

@MikeO, I'm sorry to hear that. You would expect a faint team to cover all types and causes of fainting. Also, anyone medical should be interested and engaged when relevant new information comes up, not annoyed!

I hope your PCP will be more helpful.

So, I had a long talk to my endocrinologist about this situation. She has tested me pretty thoroughly in the past to see whether there was anything endocrine going on (before I got my dysautonomia diagnosis) and has treated me for 20 years for a minor unconnected endocrine condition. So she knows me pretty well and we have a good relationship.

She agreed that as the steroids produced a lingering positive effect it was worth considering an inflammatory/auto-immune angle. Her suggestion was to see a rheumatologist.

She also said that if my condition does have an auto-immune component, I would be much better off getting that identified and then taking a specific medication for that condition (if one is available) than taking steroids. She explained that often there are medications that are more specific and have less long-terms risks than steroids.

Her suggestion was to see a rheumatologist.

I agree with her that this is a good idea but I am not sure I want to get on another medical merry-go-round. The wait to see a rheumatologist could be long, I will have to travel two hours plus for my initial in-person appointment, and I may strike someone who has no interest in investigating. And even if they investigate I suspect they won't find anything. Whatever it is is just not really making itself felt generally yet, or is too specific to dysautonomia to be tested for for another however many years until they get that sorted out.

I was just looking through the reviews for rheumatologists in Melbourne and it was exhausting.

I think I will go on Facebook and ask on the Australian dysautonomia support group.

Can't quite remember why I started this post ... I think my point is that trying to find another specialist is exhausting and may be a waste of time but there is no way of knowing beforehand, which is annoying, and you all know this already!

...

Oh, but I think I'll ask for Sjogren's blood testing, because I suspect my mum had Sjogren's and it's worth a try.

A Facebook support group that includes your new area can be a good source for local recommendations. I don’t use Facebook, so I had a family member join a support group and have a search and ask questions for me. Ended up being quite helpful for finding specialists. (The group also had a PDF list based on member recommendations that you could download after joining.)

Albumin infusion no. 2 happened on Friday.

It was a long day – we left the front gate at 10.30am and got back at 5.30pm, no stops apart from quick toilet breaks.

I am finding it takes about two days for me to recover from the trip to the hospital.

Everyone else seems to find the chairs comfy, but I've tried a different way of sitting each time, plus lots of toilet trips and pacing up and down when I can (limited), but I always end up with horrible DOMS that evening and the next day. Not to mention being sweaty from being partially encased in vinyl.

Anyway, the infusion did help me feel a little better, but I'm thinking that this is not going to work for me long term. It is good to know about; for example, should I get worse or catch a nasty bug, and when I have to have bowel surgery next year.

I still think it is worth trying if no other treatments have worked for you and you have no contraindications for blood products.

I've had no direct side effects, unlike with stimulants which improved my energy levels but caused nightmares and muscle pain, for instance.

There should be some helpful content at Dysautonomia International's Vimeo channel:

https://vimeo.com/dysautonomia

(The lectures aren't organised by topic or in playlists, unfortunately, so you have to page through them to find relevant ones.)

You may have already seen this, but I find video content doesn't come up in searches very well so it's worth mentioning.

I recently saw that Brigham and Women's Hospital has a clinic for connective tissue disorders inc. EDS, and it also has a specialist clinic for dysautonomia. I don't know whether the two clinics co-ordinate care for people with both, but you could enquire and see. Not anywhere near Pittsburgh, unfortunately.

I’ve come up with a low-tech workaround – cycling with one leg at a time, keeping the other leg bent at the knee and foot flat on the ground to give the support missing from my core muscles.

Then I swap between legs every 20 moves or so.

Not as smooth as cycling with a gadget, but seems to do the job.

Another option is holding onto my PJ pants to take a bit of the weight so I can do both legs. Not very elegant!

@Pistol, that is a good idea about physio. We have tried a few times with not much effect. A famous expert on my surgery (j-pouch bowel reconstruction) apparently tells his patients not to do strong abdominal exercises ever. Physio has helped me with lots of other things, just not my abdominals. Oh well. And I have to have more surgery soon so who knows what state they will be in after that!

There is something beneficial for me about mobilising my legs while lying flat when my OI gets bad - sitting and cycling wouldn't do it for me.

Has anyone tried supine cycling, either at physiotherapy or with your own pedal exercise gadget at home?

I don't have a gadget, I just cycle my legs in the air while lying down, but I find it useful.

I don't have very good core strength (had several abdominal surgeries in my 20s), so I can't keep it up for long.

Was thinking of getting something like the Excy (search "Excy exercise bike" and it should come up), although a less-expensive option would be good.

There is one model that can be used in bed and the other models can be used on the floor.

I imagine you could use a much cheaper one providing you could stabilise it to use while lying down. The only problem might be adjusting the height. (I'm guessing cheaper ones wouldn't be adjustable.)

@Pistol, that's interesting. Maybe I have never actually been hypoglycemic and it was OI all the time, as I can't identify two distinct patterns and snacking doesn't really fix things. (I think it's the sitting down and resting while having the snack that creates the small improvement that I've noticed.)

I'm glad to hear you've got this aspect under control with diet.

I had the same question for many years. As well as occasional episodes on standing/exerting myself, I also had "slumps" each day about an hour after eating breakfast and lunch. I wondered whether I had reactive hypoglycemia or whether my blood sugar was dysregulated somehow.

My old GP (now sadly retired) gave me the same advice as MikeO has written above. I also purchased a basic blood glucose meter and checked my levels just to be sure. In my case, the levels were always in normal range or just near the lower end of normal. Having a carbohydrate snack helped a little but did not resolve my symptoms as it would have with actual low blood sugar.

I think that orthostatic intolerance can feel a lot like low blood sugar, either when you are exerting yourself and your circulation can't keep up, or after meals when blood is diverted to the abdomen/bowel leaving the rest of the body a little low.

I have plain old orthostatic intolerance, so when I have these episodes my BP and HR are normal. I have read people with POTS say that they sometimes have an episode like you describe even when their HR is normal.

Having said all that, there is no harm in purchasing a basic meter and checking for yourself. And your GP/primary care doctor could order some blood tests to look into it too.

@MikeO, there’s a really good forum here that has threads on CGMs:

https://www.diabetes.co.uk/forum/

I wonder whether two weeks is too short a time to get a handle on using a CGM properly (even though “technically” you don’t have to do anything, there are still things to factor in such as finger prick tests, recording meals and activity, and possible effects of lying certain ways in bed on the sensor).

That is a very difficult situation for you both.

I first started having symptoms when I was 29, but they were mild to begin with, so I was able to keep working for quite a while. But over the years my symptoms gradually got worse, and I am now unable to work or do much of anything. My father is my main support person, taking me to appointments and helping me deal with paperwork and other things that brain fog makes hard.

Is your daughter happy with her doctor? Although research into dysautonomia doesn't move very fast due to the lack of funding, there have been a few developments in medications and diagnosis in the last few years. Maybe it is time for a review of her situation or a second opinion if her doctor has perhaps not been keeping up with advances.

Does she have the kind of symptoms that IV saline might help when she goes into a flare?

I would suggest putting in writing that you do not wish to apply for FMLA related to your husband and send it by registered post, or create some other proof of receipt such as copying in relevant staff members on an email or even sending a copy to your family lawyer (if you have one).

Make sure the letter is very bald and clear.

I am not familiar with the FMLA process, but it might also be appropriate to send that authority a registered letter saying the same thing.

Plus, does the organisation have a help line? If you can get through, phone and tell them what's happening. This will create an official record to support your side of things.

Also make sure to document every thing that happens.

Sorry you are going through this hassle on top of everything else.

P.S. I would also ask for a copy of the notes from the meeting, and if they misrepresent you, put it in writing and again lodge copies wherever you can.

@Bailee, I found these two resources helpful to see whether there was anything else for me to try:

Lecture by Satish Raj on POTS medications:

https://vimeo.com/486427306

List here of just about everything that has been tried for POTS:

I wonder if your specialist would try you on IV saline given that you are still struggling despite your best efforts. Some doctors view it as a good bridging therapy to get people more active and allow them rebuild their strength and stamina, then slowly reduce the infusions, hopefully leaving them in a better place.

Other doctors are concerned about the risks of having infusions and the complications of access for regular use.

One more thought – you mentioned compression socks. Have you tried compression that covers the abdomen, e.g. tights, leggings or bike short style?

I too have been working through a long list of interventions and medications and trying to exercise at night (when I often feel better). It is very hard when nothing really seems to be working.

@Wanderland, I’m sorry you didn’t get much help when you last tried.

This lecture from Dysautonomia Int. might be helpful:

https://vimeo.com/641527663

I haven’t got round to watching this lecture yet, so I’m not sure how relevant it will be, but just thought I’d mention it:

https://www.youtube.com/watch?v=Cg1-iS59V4o

It’s called “Sex hormones and dysautonomia”, presented by Dr Blitshteyn.

Thanks, @MikeO!

I am honestly amazed that my specialist ordered it, and amazed that it worked. I am also a little dumbfounded that it only happened because I had seen it mentioned here and then chased down some references to its use in Canada and asked whether I could try it. My specialist had said that we were pretty much out of options if saline didn’t work.

DINET to the rescue!

He has ordered three more sessions, so watch this space.

@RecipeForDisaster, I don’t think I’ve had my serum albumin tested in recent times. I’ve been meaning to check through my records to see if it’s there but haven’t quite got round to it.

Is yours on the high end of the normal range? If so, it is hopefully combining with all that nice saline and enabling it to stick around.

I’m so glad your nausea is gone. I hope you can enjoy a few favourite meals/snacks!

My specialist has ordered me three more sessions. Not sure whether they are to be weekly or fortnightly. The day unit at the hospital is a bit booked up pre-Christmas so there may be a wait, but that is okay. They have been very kind and accommodating so I know they will fit me in as soon as they possibly can.

@MikeO, I hope the Dexcom monitor gives you some useful information. I hear they can be very helpful.