bombsh3ll

That's great, I'm so pleased your sister is able to access IVs and that they are helping!!!

Slowly, this practice is making its way across Europe! Maybe one day in the UK!! I will definitely try a slower rate next time I get a bag. 

B xxx

13 hours ago, Pistol said:

@bombsh3ll - in my case hyperPOTS runs in the family and we have found great relief from IV fluids BUT - the trick is that they have to run no faster than 150 ml/hr or the fluid ends up outside of the blood vessels ( causing edema ) and the just gets peed out. Faster infusion will also cause high BP. 

Yes I wish I had regular access to IV fluids & could experiment. Even paying to go to a private place though they wouldn't want you there 7-8 hours just to get a litre. I don't even get edema, I can start a 1 litre bag having just peed, and before the bag is done my bladder is full again (and due to chronic polyuria I have a large bladder capacity of over 1 litre), hence it is literally coming out faster than it goes in!

You mentioned your sister starting IVs at an outpatient infusion clinic - how slow are they willing to run a litre bag? It is really only practical to run so slow either at home or as inpatient given the length of time 150ml/hr would take.

B x

I take half a teaspoon of mag citrate powder most nights mixed in a drink. It is tasteless when added to something like chocolate milkshake or a smoothie. 

I understand that both high sympathetic activation (typically the case in POTS) and licorice root/florinef (I took the former until recently) can deplete it. 

The only symptomatic difference for me is it relieves constipation which I am prone to. It can certainly lead to diarrhea if you are sensitive or take too much. 

Some people also report it helping with palpitations although I haven't found that personally.

B x

I know, I just wish there was a permanent method of increasing PV! Even when I have been lucky enough to get IV fluids it is mostly in my bladder before the bag is done!

B x

11 hours ago, yogini said:

High BP can be a symptom of low blood volume.

that is what i think is going on with me for sure! When I feel best, my blood pressure is lower, but taking drugs to lower my bp artificially makes me faint. 

10 hours ago, toomanyproblems said:

My blood pressure usually goes up with my heart rate  upon standing, especially the diastolic. I've also had a mild polycythemia (high red cells) for over a decade. I believe this is a relative polycythemia due to low plasma volume making it appear as though my hematocrit is increased. 

I have this too! In fact when I had the BV test I looked more polycythaemic than lance armstrong, red cell mass was reported so high it was incompatible with life - 200% of normal - however this was determined to be down to the faulty test due to length of time to transport the blood during which time most of it had clotted so couldn't be reinjected properly, skewing results. my haematocrtit on standard testing is always high though.

B x

14 hours ago, Trying said:

If CBC shows normal sodium, does that mean aldosterone is being produced normally and there is no need for fludrocortisone? 

 Not necessarily. You can have normal sodium but still have low blood volume. My sodium tends to run low end of normal/just below normal, and I do not produce any detectable aldosterone. 

What happens if you are losing sodium due to low aldosterone is you also lose free water with it, so they balance each other out. 

This is because a) where sodium goes, water follows and b) if the body detects low sodium it will also lower production of the water retaining hormone ADH, so less water is conserved. 

Under normal conditions I produce no detectable ADH, BUT under a hypertonic saline test with an infusion of very strong saline solution, I did produce it, indicating that I CAN produce it, I just don't because I don't keep sodium. 

If there is a malfunction in one system the body will try and compensate to keep the sodium level in range as too high or too low is dangerous.

The only way to properly measure blood volume is with a specific nuclear medicine test designed for this purpose. Standard blood tests will not show this. 

It would have been best to measure aldosterone BEFORE starting fludrocortisone in order to know if it was deficient or not - did she have it measured? Even in people with POTS/OI/OH etc whose aldosterone is normal, some still benefit from fludrocortisone though. 

14 hours ago, Trying said:

Our daughter's CBC sodium has been normal. She has taken fludrocortisone .1mg for 3 weeks and she has had no improvement in her debilitating lack of energy and her blood pressure declines 30 (SBP) upon standing 3 minutes

I am sorry your daughter isn't feeling any better. I understand though that it can take some people weeks or even months to feel the benefit from fludrocortisone. Unless she has had any significant side effects it is probably better to persist with it in order to give it a fair trial. Does she have a follow up arranged? Some people benefit from a higher dose so it may be worth discussing that. Also do ensure her potassium is monitored regularly, especially if it is not being supplemented. 

I hope she manages to get some relief.

B xxx

19 hours ago, toomanyproblems said:

I guess you'd have to be off the licorice at least another month if you want a true reading on your aldosterone.

Thanks that's really useful info! I think I will stick it out another month (unless absolutely necessitated by electrolytes or BP) given I have to pay for that lab test so don't want to have to repeat it many times. I tested low prior to starting the licorice though so I don't have great hopes of it magically picking up on its own. 

3 hours ago, yogini said:

Have you been tested for low blood volume?

I had a test but unfortunately got no meaningful result due to technical problems. That was the only center in the UK offering it (not DAXOR technology but the old style version of RBC labelling) and they stopped performing it shortly after my visit as it was discovered that the 4 hour round trip the blood had to make across London to the lab & back was causing erroneous results due to coagulation.

I would love to have a proper test with a DAXOR machine but they are not currently in Europe. 

I base my theory that I am volume deplete on clinical picture: cold pale extremities, standing tachycardia, narrow pulse pressure, dry eyes & mouth, sluggish capillary refill, poor peripheral venous access (used to have plump juicy veins when healthy), syncope, nausea, sense of non-psychogenic anxiety/agitation & the fact I pee ~ 6 litres a day! That plus the studies on POTS showing volume depletion to be common (though not universal), and my spectacular initial improvement with the licorice which is a volume expander. 

I just wish I could get a test to prove if this is the case or not. Have you had this done and if so what was the result? Did it provide a help in guiding treatment?

B x

10 minutes ago, toomanyproblems said:

So how long were you on the licorice root and how long has it been since you came off of it?

I was on it approx 3 years. The effect was miraculous at first, I was close to a normal person in function and it totally transformed my quality of life, however it lost effectiveness after about 1-1.5yrs. I was just too scared to come off it in case I ended up completely bedridden & unable to even sit up. I have been off it 1 month now. 

I am unable to tell if I am that much worse as I am basically couch bound anyway, but my BP is no longer spiking scarily high. It does still go up though when I stand (today sitting 106/67, standing 131/89, HR inc about 30)

One thing that seems strange though is people lacking aldosterone often crave salt - I never have. I did salt load for ages then stopped due to very high BP spikes and switched to just drinking balanced ORS solution to get my salt which is more palatable. I am always very thirsty though and pee loads but I much prefer the taste of plain water or flavoured drinks which is not good with a low blood sodium level.

Did you ever crave salt when not taking fludrocortisone?

B x

31 minutes ago, toomanyproblems said:

I mean you have to have some mineralocorticoid, right? 

I have seen it concluded in a study that whilst rats devoid of mineralocorticoid activity can survive indefinitely on extra salt and water alone, it will eventually lead to death in dogs and most humans. Last I checked I wasn't a rodent, so yes this does bother me!

I am currently having my electrolytes monitored - before starting licorice I had low sodium, high potassium. Normal levels during. Off it my potassium initially went to 5.1 then came down to 4.6 (normal), sodium is sinking from 139 (on licorice), then 136, then 134. Due another check Friday. 

I may end up trying back again on a very small dose to see if it helps symptoms even if my labs & resting BP hold steady, as I remain very lightheaded regardless. I can get free tests for basic electrolytes but the aldosterone test is £69. Trying to decide whether to repeat it before giving the florinef another go, was done previously in 2016 and 2017. 

37 minutes ago, toomanyproblems said:

I'm trying to balance it with clonidine.

How did you/your doctor settle on clonidine as the BP med? I tried it before hoping to calm adrenaline surges & expand volume, but it dropped my HR and BP even at quarter of the smallest tablet. 

Thanks for the helpful info anyway. 

B x

I recently came off licorice root (which works similar to florinef) but had lost effectiveness & I was also concerned about my high BP & the long term effects. Off the licorice, my BP is now in the low/normal range BUT goes up when I stand (although not to the stroke levels it was doing recently on licorice).

I have symptoms of hypovolaemia, cold peripheries, severe OI, also drink loads but don't hold fluids. Labs show undetectable renin & aldosterone. 

Thinking of trying Florinef again at a very small dose (bad side effects last time but taken together with licorice)

My question is does/has anyone with the "hyperadrenergic" type presentation ie significant raise in BP when standing take florinef and have you found it helpful?

Theoretically expanding blood volume should dampen down the sympathetic response to standing, however florinef also seemed to cause worse vasoconstriction in me and little/no evidence of fluid retention.

Many thanks.

B xxx

15 hours ago, JimL said:

I've lost 170lbs

JimL,

There are case reports of other patients developing dysautonomia following massive weight loss (achieved by gastric band/bypass etc). This could be something to look into for you. 

PS best wishes with your surgery

B x

P8d I'm glad you've found a helpful treatment and a good neurologist! I am considering the Celltrend tests but would have to pay not only for the tests but to get the blood centrifuged etc. Also if something did test positive I am not sure any doctor in the UK would be knowledgeable or willing enough to treat it.

The reason UCLH offered to test my AChR antibodies was due to sudden onset, but mine was triggered by a forceful valsalva & I've nothing to suggest autoimmune disease. I do have dry eyes & mouth but Sjogren's has been ruled out by biopsy, & it is likely anyone with polyuria of 6l/day would have dry eyes & mouth!

B x

7 hours ago, p8d said:

A few years later the Celltrend test also showed a positive for autoantibodies. 

I am interested in the Celltrend panel myself as I was only tested for AChR antibodies at the UK's only public autonomic center. Which of yours showed positive and what was the treatment offered if any & has it helped?

B x

I use Trioral. usually 2-3 litres a day. works out more cost effective than any others I've seen & it is also based on the World Health Organisation recipe for oral rehydration salts which is specifically designed for fluid replacement in places without access to IV fluids. 

It doesn't taste very nice but a small amount of fruit juice or other flavouring can be added. 

I've tried making my own according to various recipes but am never confident in getting the proportions right to maximize absorption. 

What I like about Trioral is 1 sachet makes up a litre whereas many other commercial products make up only small quantities about 200ml which is worthless to someone with POTS!

B x

18 hours ago, WinterSown said:

The biggest help I got for dyssynergia was a script from my EP for Mestinon.

I tried mestinon in the hope it would help my OI, sadly it didn't but it was a decent laxative. I didn't continue with it just for this purpose as I have other laxatives, but for anyone not finding relief with the usual bowel meds it may certainly be worth looking at. It didn't have any side effects for me (and I am very sensitive) so would consider it quite a safe & gentle drug to try. 

B x

Hi Eraena, I'm sorry you are struggling. 

It can be frustrating not getting a clear diagnosis but then even if you demonstrate typical POTS on the test, that is in itself a syndrome and can be caused by a number of different issues, not all of which can be identified. A repeatable diagnosis of POTS like in my case, also doesn't necessarily lead to any treatment that improves quality of life or functioning. 

What matters is that you feel unwell and are having symptoms that are interfering in your life, and even without a specific label there may be several treatment options to help you feel better. Have you tried any medication yet? How about compression stockings if blood pooling seems to be an issue?

Regarding your BP, if it is persistently low there are a number of medications that can be used to raise it. You mention it dropping when you stand, but does it then stay low or come back up again? Mine drops in the first few seconds which is only observed on continuous finger monitoring, but by the time it can be taken manually, it has shot back up above where it started. 

Lack of sweating can also be a sign of autonomic neuropathy, and there are specific tests such as skin biopsy or sweat gland stimulation test. 

Are you able to go to one of the main centers specializing in autonomic disorders? They might be able to give you more answers, and most importantly treatment to improve your quality of life. 

I also use a recumbent bike and seated indoor pedaller, and something I've found really helpful that is so simple & inexpensive is a small plastic spray bottle that I fill with water to spray my face when I feel faint & my body too when exercising. I do seem to sweat appropriately although have never been tested for this, but the water spray still really helps. 

Best wishes

B xxx

That sounds so unfair to have a defined list of conditions and if your particular one/s are not on it you don't qualify regardless of how ill you are and level of functioning. I really feel for what you are going through. I would get the kind of lawyer Kim mentioned, who does it all and takes a fee at the end if & when you are successful.

Here in the UK disability is based not on the name of your condition but how it actually affects your functioning eg if you can walk, cook, bathe, reliably stay conscious, etc.

It makes more sense because you could have someone with cancer or multiple sclerosis who is fully mobile & can work & do everything for themselves but everyone has heard of their condition & it is perceived as serious, whereas the person with POTS who cannot stand, uses a wheelchair & passes out frequently is a lot more impaired but receives no compassion because their condition isn't well known or understood. 

I truly hope you get it approved & get the support you need & deserve. Get your specialists to provide as much info as possible, particularly the one who recommended you apply for disability. 

B xxx

21 hours ago, JimL said:

Dr William Osler who is considered the father of modern medicine said that if you listen to the patient, they will give you the diagnosis. It's lost on medical professionals today.

I totally agree. I hope I was never the kind of doctor we're all too familiar with (I don't think I was) & I wish I was still able to work. I was only allocated 10 mins for each patient though so at 6 minutes when they're still telling you what they had for breakfast...😉

21 hours ago, JimL said:

next week I have more surgery, more cervical fusion.

All the best for your surgery! You never know, some people have improved from POTS after cervical fusion. I hope it goes well for you! Make sure you discuss your POTS with the anaesthetist beforehand so they can be aware that your BP and HR may be more labile, and that you are likely to require more IV fluids than the non-POTS patient to keep things stable.

B x

10 hours ago, Pistol said:

But after I had my daughter ( unable to go through vaginal delivery despite BEARING DOWN heavily, needing a C-section in the end ) I slowly developed POTS symptoms until I was no longer able to compensate. The realization that the labor process my have been a trigger is just my own theory, no one has told me that this was the onset of POTS for me but I believe it is a possibility. 

Wow yes a lot of women report onset after childbirth - I'd assumed that could be due to blood loss or hormonal shifts & not made any connection to my own case as I never got it after giving birth, but what the forceful straining is implicated in some way as the common denominator?

It would be really interesting to break down whether postpartum onset cases had laboured naturally (even if like you they ultimately had a section) vs just had a section without going into labour. I may start a poll on that. 

Have you ever been evaluated for a spinal CSF leak? I just had an MRI with contrast last week to check for this, as CSF leaks can also be triggered by forceful straining especially in people with EDS like I have, and can produce POTS symptoms in particular the splitting headaches when upright that go away lying down. 

B xxx

I feel like a real weirdo every time I tell my story - I was totally fine until 5pm, 4th August 2014. My dad came through the door with a balloon & my two young kids started fighting over it. I thought right, I need another balloon quick so there is one each. I went to the drawer where I knew we had some balloons, got one out and started to blow it up. It was really hard, not like a normal balloon but I was really determined and blew until I blacked out. 

I came to after a minute or so but still felt very faint. At first I wasn't concerned, people commonly faint after a forceful Valsalva, but as I lay on the floor waiting for the blood flow to properly return to my brain, time went by & I didn't feel any better. I was still down after 20 mins and although I didn't fully lose consciousness again at that time, the overwhelming feeling that I was going to kept coming in waves, despite lying flat on the floor. I still remember watching the time tick by on the oven clock, that's how I know exactly when it started. My dad called an ambulance & I was taken to hospital. 

Everything checked out normal except that I continued to feel very faint, couldn't stand up and was noted to have a high heart rate when I tried,  although it took a further 3 years to be officially diagnosed with POTS. I later discovered that what I'd tried to blow up was a water balloon that you fill from the tap. It was never going to be inflated by mouth. 

That one act in that split second was the onset of severe full blown POTS for me & the end of my lovely life as I knew it. I would give anything to have that day again.

I was since diagnosed with HEDS but my other symptoms of it are very trivial & I doubt it would ever have been diagnosed at all if not for POTS.

Of course it could have been waiting in the wings for me anyway and triggered by something else at some point but I will never know, and could have had who knows how many more active years with my children if not for that. 

B xxx

6 hours ago, JimL said:

After my spine surgery last October my bowels seemed to slow down a lot and never got better. 

Are you still on opioid painkillers that are affecting things? It could also be that you're less mobile due to POTS which doesn't help the bowels.

When I first got POTS my GI system seemed to get much worse at the same time, I was worried I had gastroparesis. However I have since come to the conclusion that for me the nausea is related to chronic cerebral hypoperfusion/presyncope - nausea is a common symptom of people about to faint -  & the slowed transit & hard stools due to immobility and dehydration due to polyuria.

At least with the bowel side of things unlike the orthostatic issues there are a range of effective medications that can help. Do you take anything currently?

B x

39 minutes ago, JimL said:

What's that like?

Bliss! I take movicol, magnesium citrate, bisacodyl and glycerine suppositories as needed. The latter are little miracle workers. My son had to get one yesterday & he was a different child afterwards 😊. I have EDS & I had bowel probs my whole life though long before POTS. 

B x

That's a good idea to run it past the cardiologist first, as they have more experience with ivabradine.

I hope it helps if you do decide to start on it.

B x

21 hours ago, KiminOrlando said:

I went from constipation and feeling spasms to no spasms and regular movements. It only lasted about 24 hours, but it was nice. It had been so long I had forgotten what 'normal' felt like. My stomach didn't hurt. My muscles settled down. They suspect that I have small fiber neuropathy but I didn't feel that crampy feeling. It was almost like total relief.

A normal BM is something to be celebrated for sure! 

B xxx

Loving it so far! I am doing about an hour a day whilst watching TV. It comes with a year's warranty from amazon.

I have used a proper recumbent bike in the garage since becoming ill but this is a lot more comfortable & accessible particularly in cold weather and allows me to be with my family at the same time. 

Cycling hasn't helped me stand any but exercise has independent benefits such as maintaining muscle tone (My steel buns are wasted in a wheelchair😉) & reducing the risk of blood clots - important in sedentary people. 

I would highly recommend one, they come in different colours and wasn't too hard for my husband to put the pedals on. I would stay away from the cheaper ones though as they don't last long. 

B x