bombsh3ll

I have three stools placed around my kitchen as I'm not able to do anything standing. Chopping veggies in advance and freezing meals also helps like others have said (we need a bigger freezer!) and I only cook hot food when there's another adult in the house. I also have to remind my family not to make demands or block my path when I'm moving between seats. I tend to be most able in the evenings so that's when I do things where possible. B x

Regarding clonidine, this may not be the best option to counter undesirable effects of northera as it only blocks your body from releasing its own noradrenaline - it will no do anything against exogenously supplied NA. A beta blocker or benzodiazepine would probably be better but see what your doctor suggests. B x

Hi Andy I get a lot of these too. Harmless but uncomfortable and worrying at the time when you're getting them frequently. I find that keeping my potassium in the upper end of normal range helps ie over 4 and preferably over 4.5, also supplementing magnesium helps. I don't tolerate beta blockers due to resting bradycardia and chronic presyncope (beta blockers decrease cardiac output and cerebral blood flow even in healthy people) however many people can tolerate these at least in small doses and find them helpful. B x

Same question here from me! I am currently waiting to have my catecholamine levels checked and may then depending on results get the opportunity to try this med if they do not rise when upright. As I am only able to be upright a couple of minutes, I don't expect a rise to be detected, so may be in the running for this unless levels or BP are super high to begin with (which they may well be after a 400 mile trip to the hospital and no sleep!) I also have symptoms suggestive of sympathetic excess so am not sure if it would make POTS worse in this regard. There are some studies coming out in relation to Northera in POTS patients, one showed 27% of POTS patients reported improved quality of life. I would definitely try it if offered. I am of the attitude that I don't want to leave any potential treatment untried when living with such a miserable condition. Given that it is typically dosed 3 times daily the duration of action is short should you encounter any side effects, and these would also probably be able to be dealt with by having a beta blocker and/or benzodiazepine on hand. If you don't try something you will never know if it just might have been the thing that got you upright again. I had a year pretty much on my feet (although never able to stand in one place) on licorice root, which I wouldn't have done had I not tried it. I have also tried many other drugs and whilst not helpful and sometimes downright unpleasant, am glad I've crossed them off. If I am really nervous about trying a drug I go and sit in the waiting room of the local A&E department near the desk (I don't check in) to take my first dose, with my medic alert bracelet on (ID & condition) & a note on me about the drug I have taken, dose and time. That way if I end up unconscious or with a dangerous reaction I am already in the right place. That has never happened but it allows me to feel safer when trying something the first time. I also never start with a full dose of a drug but break the lowest strength tablet into quarters and start with that. If it is capsules you can often take them apart and remove the desired amount (check with a pharmacist as some long acting/slow release meds are not suitable for this). Good luck if you do decide to try it. Please let us know how you get on. B x

It's great you found something that helps but I'm sorry it is so expensive. I live in the UK so I am not entirely sure about the situation re drug funding in your country however I have come across other patients online who have been able to access Northera under some kind of compassionate subsidized schemes. It may be worth contacting the manufacturer of Northera and asking if they currently have any such scheme that you may be eligible for. It is in their interest to get their drug out there because the more its effectiveness is shown the more doctors will prescribe it. Good luck, B x

That's fantastic I so wish I could get a port! I am going to start regular IVs soon peripherally but have to pay privately in the UK and it is very expensive. May I ask how much you get and over what period? Are your bags 500ml or 1000ml? I am going to be starting with 1 litre over 1-2 hours. There may be some scope to pay for IV fluids privately in Canada, I am not sure what it would cost - I think their healthcare system is similar to the UK where if you have a life threatening emergency, cancer or a well known condition, then the treatment is good but if you have something that's poorly understood, rarely diagnosed and predominantly affects women like dysautonomia you are basically on your own. B x

I do take in far more salt & fluids (in the form of electrolyte mix drinks & just a little salt added to food as I don't really like the taste of salt) than the average person (approx 6 litres/10g per day). I wouldn't say it helps me with lightheadedness/presyncope although how much worse I'd be without I don't know. I don't produce any renin or aldosterone though, these are the hormones which help your body hold onto salt or fluids, so my situation may not be typical. How come you were salt & fluid loading previously if you've only just developed orthostatic intolerance now and not before? There may be a clue there. Perhaps all the salt & fluids were actually treating an issue you didn't know you had, and it has simply become apparent in their absence - in which case reintroducing them with an eye on your BP may help. B x

No, there is virtually no clinical value in measuring urinary sodium or potassium in dysautonomia. Any attempt to estimate blood volume from this is based on the presumption of normal fluid and sodium handling, which is often defective in us. There is value in collecting urine to measure the volume - large volumes may indicate diabetes insipidus (but is more often due simply to how much we drink), or to measure catecholamines, to help exclude a phaeochromocytoma. Plasma renin & aldosterone are useful, for example I produce neither, hence the likely reason I am clinically volume deplete. B x

I get it frequently, also tends to be when my bp is high too. I am on long term PPI which completely controls my reflux, and believe it is noradrenergically mediated also. I find a small dose of diazepam helpful for this. B x

I don't think it is a deliberate out and out cold hearted scam in that they seem to genuinely believe in their programme, just that they grossly overestimate/exaggerate what can and can't be achieved and seem less concerned with scientific evidence and plausibility than with getting patients to say they have greatly improved whether they have or not. B x

Hi Ck1, Sorry you're having such a hard time. I'm in Scotland and wish I could say care on the mainland was better but it hasn't been for me. I am definitely interested in seeing that specialist of Pistol's at that price, even including flights I've forked out far more here for sweet Fanny Adams! Bet it is the famous Blair Grubb - he's my hero from reading his research & what I've heard & watched online. Couple of suggestions; I understand your concerns about a possible phaeo despite in-range urine catecholamines, and there are private places at least mainland UK where you can self refer for an abdominal ultrasound or mri. This would exclude an adrenal mass, if it hasn't already been done. Second as a GP myself I agree with putting a line under the thyroid unless anything changes. Your presentation would be v atypical for this (although don't we all long for a thyroid problem to be uncovered with an easy fix!) & with normal bloods and no neck mass, it is extremely unlikely. Third have you had autoantibodies relevant to the ANS checked? I am currently waiting approx 10m to see if nhs place in London will do mine but if it there is a private lab in Germany called Celltrend you can send your bloods to again no referral needed. best wishes, B x

Hi Jessica I haven't gone but I have spoken to them about their distance programme. The impression I got made me very wary, and if you look around online you will find information that shows it in a rather dubious light. The amount of money charged out of pocket is the first red flag. If anyone questions anything or says they have not improved, they are met with hostility. The main doctor running the center was quoted by one patient as saying something like "some people just don't want to get better" in a room full of desperate people who had traveled a long way and spent a huge amount of money to be there. The main treatment they use is a biofeedback programme called Heartmath. The device and instructions for this can be purchased independently for about £200. They are very cagey about divulging the name of this product, probably because they don't want people to know they can just buy it themselves. I do believe biofeedback can have a small role in calming down the sympathetic nervous system & have actually asked my husband for the heartmath Emwave 2 for my birthday, but it doesn't need to cost anywhere near the amount they charge. Secondly, and this to me is the biggest thing, having benefited massively from Licorice Root myself (a herbal florinef alternative that actually works and has no side effects) until the effect waned, is that the Pots Treatment Center give patients a list of supplements, of which Licorice Root is one. If people haven't tried this before and then start on it, this could certainly account for some of the dramatic testimonials of patients being out of their wheelchairs at the end of the programme. The effects don't last indefinitely but they can certainly transform your life long enough to praise the Center as miracle workers, not realizing that the sudden ability to stand is due solely to these little capsules for less than £10 a tub. I don't want to put anyone off but just to give a realistic view of what I have learned about them. Best wishes, B x

H Berly26, I wouldn't worry about gaining significant weight (other than perhaps a very small amount of water weight if it works). I couldn't tolerate fludrocortisone due to several side effects, but weight gain was not among them. You may even find it helps you maintain a healthy weight if it allows you to be upright and active more vs sedentary! Did you have POTS before your weight loss or did it develop since? I really hope you find the fludrocortisone helpful! B x

I'm going to get a hormone profile done including testosterone, and consider bioidentical replacement if it is low. I'll definitely report back on my result and if treatment helps, even a little! B x

Don't worry about taking diazepam, I have all the same symptoms as you but chronically, no virus, and diazepam helps me greatly. I use it sparingly so as not to develop tolerance. You are lucky to have a GP willing to prescribe. And please don't worry about the troponin - if you're the typical POTS demographic (premenopausal female) and don't smoke and your ECG was normal, it would be EXTREMELY unlikely to have a raised troponin. Hope you feel better soon, B x

Here's an idea that I wanted to put out and see if anyone had any experience or thoughts... POTS is around 5 times more common in women than men. Men have larger hearts and blood volume proportionate to their body size. They are less prone to fainting generally. Males in families (my own included) with hypermobile EDS tend to be far less symptomatic and disabled than females, possibly due to stronger collagen. I have come across more than one patient story online of people undergoing female to male transgender treatment, whose POTS massively improved or even resolved when they began taking testosterone therapy. It cannot all be due to menstrual blood loss as I use a continuous birth control product which I did for years prior to POTS and do not get periods at all. Therefore I wonder if testing T levels/supplementing bioidentical testosterone as a female with POTS would be worth considering? Gynaecologists and endocrinologists increasingly prescribe testosterone gel to women for low libido, which is a pretty trivial indication compared to being chairbound. I know there can be side effects at higher doses but I for one would gladly shave my face everyday and sing in a baritone if taking T enabled me to stand up! Anyone tried or considered this? B x

It certainly helps with constipation but not with anything else in my experience. B x

Sorry you had a bad experience with the cardiologist, I would definitely not go back there - seek out someone who understands dysautonomia and is willing to listen and address your questions, particularly if you are paying for it. Licorice root can be bought without a prescription and it is really quick to take effect so you would soon know if it helped you or not and could just stop if not. I would recommend checking your BP on this just to make sure it is not going too high. Best wishes, B x

I also never found any benefit from extra salt and now drink commercial electrolyte mix instead which is more palatable. I think any benefit from extra salt can only be very transient as the body just adapts and pees out the extra. I do second the concerns about large amounts of plain water though - that can lead to low sodium levels. I don't produce any fluid retaining hormones and consequently drink a lot voluntarily due to thirst - I always have though, long before I developed POTS. What I am curious about though when I see recommendations for POTS patients to drink 2 litres, 3 litres etc, is whether this is your total fluid intake for the day which seems very low, or if it is in addition to what you would normally drink by choice? I drink a litre of electrolyte mix before getting out of bed, and my total daily fluid intake is about 6 litres. B x

I still take them even now, I am afraid of how much worse I might be without them. Also I produce no renin or aldosterone at all, and total mineralocorticoid deficiency is rapidly fatal in humans so I need some form of substitution and would have to take florinef instead which gave me horrible side effects. The brand I take is Swanson's Licorice Root 450mg capsules, 2 daily. They can be bought online from amazon, iherb and various other suppliers and do not cost a lot. It was about a year until the effects started to wane for me. Others have said between a few months and a few years for this to happen so we are all different. I tried a licorice tea once but the taste was absolutely vile. If you do choose a licorice product, make sure it is true licorice root and not the deglychyrrized (inactive) version which wouldn't have the mineralocorticoid effect. Ironically my blood pressure is not low - stage 1-2 of hypovolaemic shock is what I relate to - increased BP in an attempt to compensate for low blood volume, however licorice root is said to raise BP as well for those who need that. I would really recommend trying it, you might just have as good a year on it as I did - I could walk for a couple of miles on it and even stand a bit too!!! It allowed me to get married standing up for which I will be forever grateful. B x

Couple of suggestions re your night time tachy to bring up with your doctor (I wake with this too, usually early morning before my alarm and it is horrible); 1) Ivabradine is worth considering as it lowers heart rate without lowering BP. I have decided against this for myself as my resting HR is low, but it may be a good fit for you if you can access it. 2) Possible tolerance to/withdrawal effects from clonazepam. If you have been taking this every night for several years you may have developed a tolerance so that the dose that once kept you sleeping peacefully is not having the same effect and symptoms you would have had all along but for this are rearing their head, and also you mention trying to lower the dose - this may be producing withdrawal effects. I was also going to ask if you raise the head of your bed as is often suggested, but you've answered this re sitting upright. I do wonder if having the head of my bed raised contributes to this problem in my own case, possibly by causing reduced cerebral blood flow (like when we sit or stand up during the daytime) triggering the sympathetic activation & pounding heart etc. I am spending a few nights away from home next week and will pay attention to whether this happens when sleeping flat. Good luck with your appointment. B x

No, it basically tricks your own cortisol into mimicking aldosterone in the kidney, resulting in increased salt and fluid retention. It does so by inhibiting an enzyme found in the kidney which normally inactivates cortisol so that it cannot bind to the mineralocorticoid receptors. It is definitely worth a try although for me the effectiveness wore off after some time. I have heard this is similar to other patients as well. Interestingly I have also heard the same about fludrocortisone, which I didn't tolerate anyway due to side effects. Maybe if I had just kept the licorice root for special occasions I could have kept it working for longer. The benefits were dramatic and noticeable within a few hours of taking. B x

What kinds of exercise does your PT get you to do, for how long and how often? I have never had access to physio, but I bought a recumbent bike which I use for 30 minutes most evenings. I can't say that it has helped me but my legs are probably stronger than they would have been without it. B x

I don't experience fatigue as I'm pretty much chairbound due to orthostatic intolerance so don't get to do anything fatiguing, but do either or both of those meds help you be upright at all? I would be interested to see how quickly I got "normal person tired" on a treadmill with gravity removed! B x

I took mirtazapine for a while when I first became unwell. I primarily took it for nausea and sleep. I lost a lot of weight when my POTS started so gaining that back was helpful for me, but when I started to get heavier than I had been pre-POTS I cut the dose from 15mg to 7.5mg and my weight stabilised. What dose were you taking? It may be that a lower dose may still help without the weight gain. B x