bombsh3ll

Well I always feel my best late at night and then like death again in the morning, I believe it is due to overnight volume loss so definitely a similar thing here. I do have the head of my bed raised but haven't found it helpful. Has the florinef helped?

Mine too, although the drop is too brief to capture on a standard BP monitor, it was only seen on the continuous finapres monitor in the first 7 seconds then it overshoots. I believe mine to be the sympathetic nervous system on overdrive trying to compensate for low blood volume. I am looking forward to getting my catecholamine levels, assuming they are accurate. I have none! I've tried the famous water bolus thing even though my BP isn't low & concluded it was bunkum, so it is interesting to see someone who actually demonstrates this response! I also want to do the Celltrend test. I just don't think anyone in the UK would treat a positive result. Also, from what I can gather, pretty much everyone who does it tests positive for something on it which makes me wonder about the clinical validity, although granted these are all dysautonomia patients not healthy people randomly sending off their blood. If anyone has sent their blood to Celltrend and had a completely NORMAL result, I would really like to hear too. B xxx

Lily, what symptoms/tests did your doctor base the low blood volume theory on? Did you have it measured? I truly believe this is a major issue for me. Regarding the EDS, I don't really feel stretchy veins could be the culprit in my case as my onset was so sudden, triggered by a forceful valsalva. I think more CSF leak or previously asymptomatic craniocervical instability tipped into becoming symptomatic due to the sudden increase in intracranial pressure. Compression of the lower brain structures could then lead to volume homeostasis disturbance. I mean, I've had the same veins all my life, three term pregnancies, no varicose veins, so why would they suddenly all become too stretchy after I tried to blow up a water balloon by accident aged 34? That's not to say others with EDS may not have an issue with stretchy veins, but surely that would develop insidiously over time? B xxx

Octreotide has shown benefit for POTS/OI in a number of studies with no mention of VIP, so it might be worth looking into for you given that you do have elevated VIP. Do you have a decent specialist to discuss this with? Also whilst I am not advocating dishonesty, as a doctor myself (although no longer able to work), if a patient reports diarrhoea, you kind of have to believe them IYKWIM. Although that could open up another whole can of worms, colonoscopy etc. B xxx

Thanks, it is really helpful to hear from someone with experience of the combination. I am guessing she has to have frequent electrolyte monitoring on the two? I also value the uninterrupted sleep desmo gives me when I take it, but I am sorry it is not helping with her main symptoms. My only problem is being unable to stand without severe presyncope though so different symptom pictures. I wish all of us could find something that helped. B xxx

This can only really be confirmed by specialist testing, but you could get a basic idea by the appearance of the legs, for example is there swelling or change in skin colour after standing or sitting with feet down? Does the calf diameter increase? Are her feet cold or warm? Veins plump juicy & visible or shrunken & flat/invisible. Pulses strong or weak/not felt (google dorsalis pedis and posterior tibial pulse points for where to feel). Does she benefit from compression stockings? All of these can give clues but are not an exact science. Personally I feel I am overconstricted - no veins, no swelling, cold pale extremities, weak peripheral pulses, hard to get blood from, no benefit from midodrine etc. but I have never had this formally assessed. B xxx

I'm so pleased your result has given you some peace. If it is mast cell, I don't have much knowledge about that but there appear to be a growing number of treatments that people find helpful. Look after yourself & don't feel you need to reply. Just know that you're not alone in this and people care about you on here even though none of us have met in real life. Losing your mother at the same time as becoming unwell is also a major double blow to deal with, & I am sorry you had to go through both at once. I hope you have other supportive people around that you can lean on. Look after yourself, B xxx

I too have really disliked eating since developing POTS. Prior to that I loved my food although I was never overweight. Since becoming ill I would happily not eat and just drink fluids all day, but I force it down in order to maintain nutrition. Protein shakes are really helpful, an easy way of getting in some of your daily load. For me there are no correlations with type of food, only that small portions are easier. I was tested for coeliac - negative. My glucose has always been normal too even when I've fainted in hospital, so it isn't that either. B xxx

That's interesting - I would really like to have the testing offered by Vanderbilt, particularly the blood volume test. We just can't get that in the UK. I believe my "hyperadrenergic" presentation is secondary to hypovolaemia as when I have been volume replete those symptoms, the chest pain, pounding heart, freezing cold extremities, sense of being full of adrenaline/impending doom, tremulousness are all reversible. It will be interesting to see what my blood catecholamines show, although I was only tilted to 60 degrees which isn't really close to standing. I would love to have tangible evidence of the blood volume deficit though. I am also no longer hypertensive now off licorice, although my BP and HR still go up when I stand, just not to the same levels. I have no reason to suspect neuropathy, although due to the sudden onset they did test for AChR antibodies. Have you identified the specific gene in your family? Clearly there is something heritable with that pattern. Nobody else in my family has it, although my dad and son have minor features of EDS like me. I don't believe EDS is causing mine although I am also being assessed for CSF leak and craniocervical instability. Those would be the only ways in my opinion that EDS could cause such a sudden onset. It must be hard knowing you could have passed it on. My one consolation since becoming ill, & something I reassure my children is that it is neither contagious nor heritable so they will not end up like me. I hope that's true. I'm thankful my youth wasn't blighted by knowing what was in store! B xxx

Hi yes I have seen one, he wasn't actually that helpful although he did give me two hypertonic saline tests for diabetes insipidus. The first was inconclusive, the second negative. He advised me to only take desmopressin in low dose and very occasionally at night as I tend to run hyponatraemic. He offered me a 3rd hypotonic saline test saying there was still about a 20% chance I have some degree of central DI, but I declined as it was brutal and I don't see how it would change my situation if I can't take very much of the treatment for it. I have had polyuria all my adult life, but I had my first major postpartum haemorrhage at 18 and another life threatening one 14 months before the onset of POTS, so there is the potential for some pituitary damage (Sheehan's syndrome). My MRI of my pituitary was normal however and other pit hormones also all normal. He was an elderly professor, very old school and at one point suggested the benefits I initially derived from licorice root (which promotes volume expansion) could have been psychological - I later found out he had personally published several articles many years ago on its mineralocorticoid effects, along with a no longer used drug carbenoxolone. Well yes I was euphoric taking it because I was no longer lightheaded & passing out & could walk!! He also wrote off my undetectable renin and aldosterone as the result of a "typical Western diet" - without asking me anything about what I ate. I asked why everyone else eating this presumed diet can stand up except me, for which he had no answer. I haven't been back since because I got my main objective from his clinic which was a referral for health board funding to attend the autonomic clinic in London. I do think that most if not all of my problems stem from volume depletion, but the problem is I had horrible side effects with fludrocortisone, the only drug available to replace aldosterone, and without mineralocorticoid activity it is dangerous for me to take desmopressin. I am going to have my follow up call with the autonomic unit next week with the results of the tests they did, and discuss a retrial of fludro +/- desmo at a very low dose. I never tried a quarter of a tablet and always had licorice root in my system when I took it previously, so it may be worth revisiting. Still, my problem could be autonomic as it is the ANS which tells the kidney to produce renin, leading to the volume retaining RAAS cascade, and similar hormonal deficits have been found in POTS patients in various studies, but it is definitely an angle I would like to pursue further. B xxx

Next week I will hopefully receive the results of my autonomic testing from UCLH in London. I had a stand test, and a tilt table test to 60 degrees, both of which confirmed POTS. Pulse pressure narrowed to 20 with increase in diastolic BP to 102 on the stand test, BP went up to 150/98 initially on the tilt test then gradually came back to around 125/90s, still jumping up and down a bit. Max HR was 120, with increment of about 40bpm. I also did some breathing tests lying down but NOT a valsalva as that is what precipitated my POTS onset. I do not know the significance of those but believe it relates to sympathetic/vagal balance and heart rate variability. My blood catecholamines were tested supine and after 10 mins of tilt. I was surprised I lasted that long but I was allowed to keep on my compression tights, was well hydrated, unfed and had taken 5mg diazepam prior. I am a little unclear despite a lot of reading about how subtypes are determined. I strongly believe I am hypovolaemic and narrow PP with raised diastolic would fit in with that, but am not sure if this is considered a subtype on its own. I have read in some places that hyperadrenergic pots is determined by a BP rise on standing/tilt, which mine does, and in other places that it is identified by catecholamine levels. Some authors refer to high levels supine, and others to either a defined increment or certain cut off level on tilt. The team were very nice but less knowledgeable than the average patient, so I would really like to hear from anyone diagnosed with a particular subtype by any of the top clinics/specialists how that was arrived at. TIA, B xxx

Hi does anyone out there have any experience combining these two meds for volume expansion/retention, particularly if you are known or suspected to be hypovolaemic or deficient in the relevant hormones on testing? I am wondering about this combination as I clinically fit the picture of hypovolaemic POTS, pee ~6L per day and my aldosterone and ADH are both well below range. I can produce ADH under hypertonic saline test conditions, but do not do so in real life as an (appropriate) compensation for mineralocorticoid deficiency which makes me tend towards low sodium. I have had bad side effects with standard doses of florinef, whilst desmopressin tabs at very low doses really help reduce the polyuria but not my orthostatic intolerance. These do not cause me any noticeable side effects but drop my sodium further on blood tests which makes regular use too dangerous without mineralocorticoid replacement. Taking extra salt doesn't help because my body can't retain it. I was therefore thinking that a very low dose of the two together may help safely expand my volume with reduced side effects, and would really like to hear from anyone else who has been on both of these meds at the same time. How did it work out for you? B xxx

Have you been to the autonomic unit at UCLH? They specifically do a meal test on patients due to the known effects of eating on autonomic disorders. It may be worth it to get this documented then you can present it to the next (hopefully more enlightened GI dr you see). I declined this during my stay as the only advice for it is eat small meals (who with POTS eats big meals?!) & some patients get to try Octreotide which I have already tried and wasn't for me. Pyridostigmine (Mestinon) can be helpful with GI motility and may be worth asking about. I have heard Professor Qasim Aziz is good and knowledgeable re autonomic disorders. He practices privately but may also hold some NHS clinics in London. I have no GI issues per se other than reflux and slow transit which were lifelong prior to POTS, possibly made a bit worse by immobility now, but they are still easily managed. I still struggle to get enough calories in as being so lightheaded makes you not want to eat and struggle to prepare meals, & supplement with protein shakes to keep my weight up. best wishes, B xxx

This is also the case with me, we've chatted before about this. Toomany, have you ever tried desmopressin? I am still hanging in there re licorice root withdrawal, BP usually about 105/70 now, electrolytes steady but sodium low end. Still permanently presyncopal, just the same as when my BP was dangerously high, so for me it is def related to low cardiac output than BP. Planning to retest aldosterone after 2m. I am wondering about combining low doses of florinef and desmopressin. The latter causes me less side effects and noticeably reduces the polyuria, but I can't take it alone due to the risk of hyponatraemia with mineralocorticoid deficiency. B xxx

Hi there are some studies by Satish Raj and others documenting reduced blood volumes of up to 20% in POTS patients, with the largest deficit noted to be 27%. That would equate to stage 2 hypovolaemic shock, but in our case it is chronic. There is also an older paper by Fouad & co on chronic idiopathic hypovolaemia. The underlying pathology is not entirely clear but appears to relate to a lack of fluid retaining hormones ADH and aldosterone. My own levels of these are virtually non existant, and I pass up to 6 litres of urine per day. This results in some or all of the above symptoms, with which we survive but are miserable. Have you tried any treatments aimed at increasing blood volume and have they helped? B xxx

Hi Megan, I am sorry you are going through this. The way I would look at it though would be to be fervently hoping and praying that it IS carcinoid, which is highly amenable to treatment & could resolve your suffering, than for it to be negative & another dead end, just left so lightheaded & limited with no relief. I have thought about having the chromogranin test myself as I dream of a treatable cause, however I have no flushing or diarrhoea, & the test is very expensive. Pistol I am sorry to hear this too, waiting for a 6 month re-scan is obviously going to be hard unless any of the tumour markers show anything. If it reassures you any, it was fairly common when I was working that a patient would have suspicious nodules on a chest CT that would turn out to be nothing. Resolution is so high that little things often show up like a small fibrotic lymph node that cause a lot of worry but prove benign. Equally though, the prospect of discovering an underlying & potentially treatable cancer may just put your POTS in the paraneoplastic category and hence respond to treatment. How long have you been ill, could the timescale fit? B xxx

This is something that interests me as I have all the symptoms of severe volume depletion & initially responded dramatically to volume expansion with licorice root although the effects of this were not sustained. Symptoms of hypovolaemic shock: Rapid heartbeat. Quick, shallow breathing. orthostasis Feeling weak. Being tired. Confusion or wooziness. Little or no pee. Initially normal or high BP esp diastolic due to increased vasc resistance. More severe volume depletion - low blood pressure. Cool, clammy skin. Note in POTS urine output is increased rather than reduced as the underlying cause of our volume depletion is usually inappropriately high urinary sodium & water loss. Are you able to get a blood volume analysis? I attempted one in the UK but it failed due to technical problems. DAXOR is a company in the US that have an accurate blood volume analyser which is used by many of the main hospitals. I wish they would branch into the UK so I could prove my low volume theory. B xxx

Yes my veins are totally shut down too. It always takes several attempts to get one in me. Before I became ill, I always had really great plump juicy veins, getting blood taken etc was never a problem. Was that the same for you? B xxx

I know, I would much rather be able to get it at home over the duration I choose! Can I ask what you find better - fast or slow infusion & how much you run at a time? Some people seem to respond better to fast, some slow. I am so volume deplete I am not confident this private place will be able to get a cannula into me if they are just used to dealing with misinformed healthy people who think an IV will enhance their appearance. B xxx

Thanks for updating Sue, Do you think this may be connected to your dysautonomia? If the source could be found and resected it may result in a cure for you! Do you get a lot of diarrhoea? Has Octreotide ever been suggested for you? I hope you get some answers B xxx

Hi and thanks for sharing the article! It was this reasoning that led to me trying the SSRIs in the first place, & had they helped then I would have happily continued to take them irrespective of their licenced use. I know a lot of people report benefit and I certainly didn't mean any disrespect towards anyone taking this class of medication whatever the reason if they find it helpful. For me though, I chose to stop due to lack of benefit and secondarily the perception by less well informed healthcare providers on seeing an antidepressant on the prescription list, which sadly I know from my working days happens. I do take as required diazepam which equally carries psychological connotations, but the difference is that does help me, so that outweighs however it might be perceived. I also think in many cases, people with POTS are on SSRIs as a result of initial misdiagnosis, & once their condition is correctly diagnosed they remain on them as either the prescription is never reconsidered or they try and come off and experience withdrawal symptoms which make the therapeutic value of the medication appear greater than it actually is. I have had a look at the study above, & it seems to conclude sertraline showed no symptomatic benefit despite altering clinically measured parameters. As far as I am aware the only evidence supporting the use of SSRIs in autonomic disorders is anecdotal & no formal studies have demonstrated symptomatic improvement, but I could be wrong & would still be really interested to read any studies that do show symptomatic benefit though, if anyone has come across any. B xxx

There is currently a study going on in Alberta to investigate the benefits of albumin which should in theory last a lot longer than saline: https://clinicaltrials.gov/ct2/show/NCT03365414 How I wish I was a patient there!! I am confused about the inclusion criteria though, it states idiopathic POTS but also seems to say they have to have abnormal sweating in feet/legs (which I don't think I do but never tested) to confirm neuropathic POTS. I believe my primary pathology is hypovolaemia rather than neuropathy and surely IV infusions are even more likely to be of benefit in volume depletion. Anyway who knows in years to come if this may become widespread if it proves helpful. A new place has opened up near me doing walk in private IVs with all kinds of vitamins and fancy guff in them. I neither know nor care what glutathione is, (bet most of the celebrity love island types who go there think it's something to tone your butt) - it isn't cheap & it's just the volume I want not all the toppings, but I think I may give it a go as an occasional treat. They have said they can omit the magnesium for me from their cheapest litre, so that's good as magnesium can drop BP. B xxx

Hi Aida, I have always just experienced plain lightheadedness/presyncope rather than vertigo - there is a definite difference, many on here will know exactly what I mean - but lately I have had some vertigo type symptoms as well - a bit like being on a boat or moving when you're not. Is the zoloft a recent addition and do you feel it is helping in any way? It certainly could be a side effect and may be worth discussing with your doctor. I am a bit leery about the use of antidepressants in dysautonomia - I did try fluoxetine and zoloft early on in my illness, not for the depression which is a natural response to being unwell, but because there is a school of thought that they can favourably modulate the ANS - however they did not help me and I came off due to feeling uncomfortable about being on psychiatric medication when we already have enough difficulty with some doctors mistaking our disorder for a psychological one, even after we are formally diagnosed. I would definitely have remained on them despite any stigma if they had benefited me at all though. I hope you find something that helps. I may try the otc remedies suggested above myself. B xxx

Having tried both I would highly recommend the tablets which can be broken into small pieces. With a spray, you cannot really control your dose. Also it is not that easy to administer - you don't know whether to sniff or not, and it all just seems to run back out again, leaving you wondering how much if any has been absorbed. The tablets act quickly, especially if taken on an empty stomach. Sublingual wafers also exist that dissolve under your tongue but these are more expensive and probably harder to access. Good luck I hope they help and would love to hear how you get on with them. PS instead of saline which is short lived have you ever had opportunity to try a colloid infusion? B xxx

I don't know maybe something that might get you access to IVIG? I'm considering Celltrend which I believe is similar. I wish my husband could put an IV in me! Seriously I know you have a clotting disorder but what about with blood thinners? B xxx