POTS / hEDS - Continuing issues

[mattyb_uk]

Hello folks.  Posting here asking for some advice on behalf of my partner, who is in her 40s and we are based in the UK.

She has been diagnosed with Hypermobile EDS, as have our kids. 

About 2 years ago she suffered from flank pain and ended up fainting in the bathroom.  She was taken by ambulance to hospital and they couldn't figure out exactly what was wrong with her.  She was in the hospital for about 5 days. her white blood cell count was off the charts at about 90,000mm3 and they weren't sure as to whether she had a kidney or bowel infection. her bowel was inflamed so they pumped her full of antibiotics and after seeing negative autoimmune tests, they discharged her. 

She emerged from the hospital about 15lb lighter but about 2 weeks afterwards, she developed thunderclap headaches and the symptoms we now now of POTS / Orthostatic Hypotension.  She couldn't stand up; her blood pressure would crash.  We ended up seeing a Dr Adrian Morley-Davies who was fairly sympathetic and was familiar with POTS/EDS comorbidity and put her on Midodrine, Ivabradine and Fludrocortisone. 

What followed were months of random symptoms such as flank pain, intolerance to extreme changes in temperature, fatigue, near-syncope episodes,  gastroparesis and neuropathic pain. 

Things have levelled out a touch now but she is still on the medication and I swear that the medication is just treating the symptoms and not the cause. 

I've read on the various EDS forums that magnesium is good so the missus is on about 100mg a day, along with CoQ10.  She is suggesting she has lots more energy and less joint paint on the magnesium.   I have read the Driscoll Theory and she's trying Alpha-GPC.  Seems ot have been sorting out the gastroparesis problem to be fair. 

I was just wondering if there's anyone here who has suffered something similar and if it's worth pursuing any other avenues, such as Vitamin B1 / L-Carnitine etc or seeking out any specific specialists? we have spoken to Dr Valeria Iodoci who wants to run lots of Autonomic Tests - just wondering if anyone has had any experience in that area too? Just don't want to go through all of these tests and end up with the "Idiopathic POTS" diagnosis.   

What I'd love is to get her off these meds. 

 

thanks all

[MomtoGiuliana]

Welcome to the forum. 

I am glad she is getting better.  Dysautonomia can include so many symptoms and unfortunately can be "idiopathic".  However I am interested that you mention flank pain bc the same thing has happened to me during POTS flare ups.  The last flare up I had the pain was so severe I needed to take strong pain relief.  It felt just like a kidney stone (which I have had) but according to doctors I did not have a kidney stone.  I even had blood in my urine.  But nothing was ever diagnosed.  Eventually the symptoms receded.  Many tests and all came back normal.

I was found to have low B12 and I do feel better I think on oral B12 supplement.  

She may eventually be able to wean off the meds.  Many of us have periods of remission and they can be quite long.  I have gone years at a time without significant symptoms.

What helps me the most when I am in a flare is IV fluids, drinking fluids, extra salt, low dose beta blocker, small meals, avoiding hot showers especially in the AM,

[Pistol]

13 hours ago, mattyb_uk said:

Things have levelled out a touch now but she is still on the medication and I swear that the medication is just treating the symptoms and not the cause.

Hello @mattyb_uk - welcome to this forum! I am so sorry your partner is going through this - the symptoms are very scary! --- Unfortunately I would like to point out that currently there is no treatment or medicine that CURES dysautonomia, symptom relief is pretty much what we can hope for. And if the underlying mechanism is EDS - well, that is of course also not curable. Both medication and supplementation are commonly a hit-and-miss ; there is no ONE substance that works for everyone and it usually requires a lot of patience and experimentation to find the individual treatment. 

 

13 hours ago, mattyb_uk said:

What I'd love is to get her off these meds. 

Most dysautonomia patients are on medications, but I know of several people with milder types of dysautonomia that were eventually able to get off the meds and just use the usual treatments of extra salt/fluids, daily exercise to tolerance, compression garments. I also would like to point out that in most cases the symptoms wax and wane, and there are times we are better and other times we get severely symptomatic ( referred to as a flare ). Lifestyle adjustments can make all the difference - including diet, exercise and avoiding long periods of standing. 

[Pistol]

@mattyb_uk here is a link that has a lot of valuable info on the subject of dysautonomia and EDS: 

Dysautonomia in the Ehlers-Danlos syndromes and hypermobility spectrum disorders-With a focus on the postural tachycardia syndrome - PubMed (nih.gov)

[mattyb_uk]

@MomtoGiulianaand @Pistol, thanks for the lovely words, the warm welcome and the advice.

@MomtoGiuliana - interesting that you mention the flank pain. My missus since 2017 (before her 2020 collapse) had been into the doctor's on and off complaining of suspected potential infections and they would always find leucocytes in the Urine but wouldn't be able to culture anything. It was bizarre - we ended up going to see a specialist who found some suspect nephritis in the kidneys but nothing further.  I wondered whether there were inflammatory mechanisms at play here but we never got anywhere.  The only thing that works for her is Naproxen to relive the pain.  Nothing else touches it.  She suspected that something in Eggs would cause it as it seemed to coincide with her eating boiled eggs!

Completely understand on the "flare up" too and waxing and waning of symptoms - I started to wonder if it was diet that was impacting this but again, we never have explored this and would love to also.

Also, wholly appreciate getting her off the meds may be a long shot / and/or unreasonable. 

@pistol , funnily enough that paper you send over is co-authored by Valeria Iodice, consultant who has recommended a battery of tests for her.  Guess we should go for it and get tested!

 

 

 

 

[MomtoGiuliana]

2 hours ago, mattyb_uk said:

they would always find leucocytes in the Urine but wouldn't be able to culture anything

Yes this has been my experience as well.  I would be told I had an infection they would start me on antibiotics.  Then would say I had no infection as the culture didn't indicate anything.  This has happened to me with almost any severe POTS flare up.  And on some occasions I would have blood in my urine.  The last flare up--over two years ago--it was the most severe ever.  I was admitted to the hospital as they said I had a kidney infection.  But then a day later said it was not a kidney infection.  The pain was intense--I really thought I had a kidney stone and they missed it, but I had ultrasound and other imagery and claimed they saw nothing unusual.  I had so many tests and all came back normal.  I am interested her experience is similar bc I have not met many people who have experienced this with POTS.  The only diagnosis I came up with was LPHS.  I have read it can coincide with POTS.  It's (of course) not well understood and many drs not aware of it.  Anyway at this point it's all a mystery for me--and fortunately I have not experienced it again for over 2 years.

[mattyb_uk]

Oh wow. Yeah. The flank pain basically is where this all started. She had an episode in 2017 where she fainted and thought she had a fit and had severe flank pain. She didn't get admitted to hospital. She had follow up neurological tests, kidney scans and all sorts but it showed nothing up. 

Then in 2020 was when the flank pain returned and she fainted and was hospitalised and then the POTS occurred. I have never heard of LPHS. 

At first we were wondering whether she had Lupus but she tested negative for it.  She has tested positive for AnAs in her blood but they have never been able to find anything.  She is HLA-B27 positive though. 

She does have occasional urgency and the feeling of an infection but it seems to subside. 

That extreme flank pain is really what I dread as it generally means that we have a problem!  The only thing that seemed to work when she was at her worst in hospital was diazepam!

Will go do some googling for LPHS. Thanks for the nudge in that direction!

 

 

 

 

 

 

[Pistol]

@mattyb_uk @MomtoGiuliana - have they ever done a cystoscopy to check for Interstitial Cystitis ( IC )? I have had bladder and flank pain as well as blood and/or Leucocytes in my urine every time and once I saw a urologist he did an exam and immediately suspected IC, which he then confirmed with a cystoscopy. 

[MomtoGiuliana]

@PistolYes I had a cystoscopy.  All normal.  That is what the urologist insisted on and insisted that was the problem.  My intuition was that it was not the problem.  Anyway, I was right...and still no answers.  GP, urologist, nephrologist, ob'gyn all stumped.  Fortunately it has never lasted longer than a few weeks at a time.  But it's scary and can be excruciatingly painful.