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Thumbs no, pinkies yes, feet twisting no. Elbows yes, hands on floor yes. I definitely think it would be worth you getting checked out. Whilst EDS isn't treatable unless you need surgery, and nor do I believe it causes POTS, being diagnosed with it helped me retire on disability grounds as EDS is not reversible, whereas doctors are reluctant to state that POTS is permanent (despite mid-life onset and progressive course in my case). This may hopefully never be relevant to you, however I definitely plan to have my son evaluated before secondary school, as contact sports are best avoided in EDS. (I always had a note or hid in the toilets so sport didn't come into it at school for me, I think I instinctively avoided it). B x

My son who I think also has EDS does that too! I can't, never have. It looks gross & makes me wince when he does it! You can look up the Beighton scale on google with pictures and see what you score - 5+ out of nine is diagnostic. It doesn't include wrists though. B x

If I can stand long enough to get a BP reading, it tends to be high. I have POTS which is diagnosed on heart rate change rather than what happens to the BP. I also have random severe hypertensive spikes >200 which are not posture related - I have been investigated several times for a pheochromocytoma which was negative. When I went to get diagnosed with POTS, I had a continuous BP monitor on my finger which recorded a large drop in BP in the first 7 seconds which then corrected & would never have been caught with an arm cuff, before it shot up. Sometimes, you can get a drop then an overactive sympathetic response to compensate for it, which overshoots. This tends to happen if you are volume deplete (which I am) although the autonomic nervous system is a strange thing and can misbehave for many different reasons. Perhaps you are getting a drop followed by an overactive compensation over a slightly different timescale so are picking up both low and high readings depending how long your cuff takes to inflate or how long it takes to settle. Looking at a typical POTS tilt reading in a presentation by Satish Raj, the tilted BP readings were all over the place, systolic up to 200 at one spike, with generally much wider excursions from the baseline than the control patient. I found this comforting to know (although sorry for the patient!) I tend to focus more on how I feel though than numbers - I never checked my pulse or BP before I was ill. If you are having symptoms, these are what your doctor needs to address if possible even if they are happy with your readings. B x

I am so sorry your son has POTS, the one thing I can imagine to be worse than having it yourself must be watching your child have it. Getting a diagnosis and taken seriously is a really positive step though. It is great that he is going to have an echo (I recently had one too and knowing you have a structurally normal heart gives valuable peace of mind) and getting to try fludrocortisone. I hope he has a positive effect from treatment. Statistically, teens and males have the best chance of remission so there is hope in that he ticks both of those boxes. Do you or any family members have features of hypermobility/EDS? Others in your family may have these but be lucky enough to escape orthostatic issues. My dad 78 clearly has it and son 10 looks like he has it, but my dad is as fit as an ox and has never had problems standing. I was diagnosed with hypermobile EDS since developing POTS, but the other features of it are minimal in me & nothing I would have ever seen a specialist for. I know there is an association with POTS and EDS, but I will also never stop searching for an actual cause and treatment. People are born with EDS but not POTS, so I don't necessarily believe it is causal. I highly suspect the fact that I produce no renin or aldosterone hence cannot retain adequate fluid has far more to do with it in my case. Best wishes for you and your son, B x

Hi Derek, My circulation is rubbish too, immobility creates a real vicious cycle. I just posted about this on another thread which I recently purchased and am finding helpful. https://www.amazon.co.uk/dp/B00EIC5KDS/?coliid=IM8I3FIHVBBAP&colid=211Q3UM5J3UUQ&psc=1&ref_=lv_ov_lig_dp_it B x

I too struggle with temperatures - I believe at least in my case it is due to low blood volume as I seem to sweat, vasoconstrict & vasodilate appropriately, however the mechanism can vary from person to person. I live in Scotland, UK, so feeling cold is commoner for me - however I do not tolerate heat so when family turn the heating on, I suffer. What I find best is to just warm the parts of me that are cold ie hands and feet rather than my whole body. These are the things that help me, some of these things look really geriatric, especially as you are young, but I love them; For warming: Hot water bottle with fluffy cover - for hands Electric rechargeable Cookey hand warmer. Beurer plug in electric foot warmer. Electric blanket on my bed. For cooling: Hand held fan Ice pack (wrapped in cloth) Spray bottle with water (this is really simple, cheap and portable and has staved off many a faint for me) I hope you manage to find some relief. B x

I've had a couple of mini pedallers that I use on the couch during the daytime, but they have generally been poor quality and not lasted long. I recently found this Mini-exerciser called Gym mate Magnetique that works on both arms and legs as well as the cardio-vascular system. It could also be used lying down, placed against a wall. It can be found on-line. I was a very active person prior to POTS & hate how it has forced me into an involuntarily very sedentary life, but this mini bike is allowing me to access some of the benefits of exercise that are otherwise only available to upright people, & I wanted to share that there is such a product. Many of us enthusiastically try exercise then abandon it when the promised improvements in blood volume & orthostatic tolerance are not delivered, but I believe it is worth continuing to exercise sitting or supine for the other benefits such as reduced risk of blood clots, maintained muscle tone in legs (for when that cure does come!), improved cholesterol, inflammatory markers, mood & sleep etc. I just did half an hour and my usually freezing cold, veinless, alabaster hands became warm, pink and boasted plump juicy veins like a healthy person's hand - for a short time anyway! B x

I take 5mg diazepam for this. I cannot take BP meds as my blood pressure is normal the rest of the time and I am chronically presyncopal. B x

My POTS started suddenly after a forceful Valsalva - blowing up a water bomb that I thought was a balloon. I was previously completely healthy. I was since diagnosed with hypermobile EDS but the other features of this for me are trivial/non-noticeable and I only pursued it due to it being a potential cause for POTS. That said, EDS and other connective tissue disorders increase the risk for spontaneous CSF leaks, and I am finally having an MRI brain and spine with contrast next Monday to look for signs of a CSF leak. I live in the UK so do not have access to myelogram unless the scans show signs, but according to the neurologist Dr Tyagi who is knowledgeable in CSF leaks, the MRI with contrast picks up over 80% of significant leaks. Personally in your case I would jump at the myelogram, but if you do not want the risks of this, the MRI brain and spine WITH CONTRAST could be a reasonable safer alternative. Best wishes, I too long for a treatable cause to be found! B x

Hi Megan, No it has to be the non DGL licorice, the DGL form has the active ingredient removed. The brand I use is Swanson's, which is sold by various online outlets including Amazon. Glad you're feeling better on less propranolol. B x

Hi Megan, Sorry you're suffering. I just wanted to add that I'm another with normal (or highish) BP and HR sitting and can feel absolutely awful with lightheadedness and presyncope with normal readings. O2 is also normal. The reason, I believe (I am a doctor but no longer working due to POTS) is that superficial readings of pulse and BP tell us nothing about cardiac output (for that you need to know stroke volume which is not easily measured), or cerebral blood flow. If you want the science - blood pressure is the product of cardiac output and peripheral vascular resistance. Two people could have exactly the same BP and HR, but vastly different cardiac outputs (and therefore cerebral blood flow). Person A could have a high stroke volume and therefore cardiac output, and a low vascular resistance (ie how hard the blood vessels are constricting), whilst person B (like us with dysautonomia) could have a miserably low stroke volume & cardiac output, with a high vascular resistance (cold hands and feet anyone?!) Unfortunately beta blockers make person B's situation worse in terms of reducing cardiac output further and increasing vascular resistance, although they can make the actual numbers look better. I am still looking for the answer myself. Something that helped me greatly but wore off after about a year was licorice root capsules by Swanson - they boost blood volume. Best wishes, B xxx

I don't understand what florinef has to do with latex. I would speak to your doctor for reassurance but I am sure they are aware of any allergies you have. Also if your allergy just causes a rash, there is no reason to think that something with no connection to latex whatsoever would cause worse. You could always take an antihistamine before the first dose, or go and take your first dose at a medical facility. Unfortunately I haven't found anything else that's helped after the licorice root wore off, but it is an alternative for you if you end up not being able to take the florinef & it really made the toddler stage with my daughter much more enjoyable. I still couldn't stand in one place but I could WALK, & sit comfortably. B x

Hi Potsiebarbie, I am similar, normal cortisol but undetectable renin, aldosterone well below bottom of lab range yet sodium usually low end of normal despite salt loading. On paper, florinef looks like the perfect fix for me but unfortunately it didn't help & caused intolerable side effects, but most hypovolaemic folks with low aldosterone seem to do great with it so don't be put off! What did massively help me for a year or so until my body got used to it was Licorice Root (Swanson's caps, 2 per day) which basically does the same job but without the side effects. With florinef, my advice would be start on a very low dose and build up gradually, don't expect instant results as it can take weeks to show effect, take after food if you have a sensitive stomach, and make sure to get your electrolytes checked regularly - it can lower potassium. Best wishes I hope it gets you back on your feet! B x

Hi Derek I am sorry you are going through this. It is bad enough being unwell without having to deal with all the financial stress and having to prove your illness that goes with it. I am currently going through the same process in the UK. In order to get my pension released early on disability grounds, I have to get a specialist to commit to saying my condition is permanent. The problem is they are so lacking in knowledge they are looking up POTS and seeing that some people recover - yes teens who get it during a growth spurt might, but women with underlying connective tissue disorders who get it in their 30's don't. It is so frustrating and I hope you get the financial assistance you need and deserve soon, and with as little a fight as possible, but at the same time be prepared to put up a fight. Educate yourself, keep copies of all medical letters, test results etc. Best wishes, B x

Hi Catlady, This is absolutely "normal" in dysautonomia and many if not most of us find we no longer tolerate alcohol. It is dehydrating and vasodilating, and can cause orthostatic intolerance and passing out even in healthy people. The way I see it is I am not denying myself a wanted treat, I am avoiding something that directly makes me feel even more unwell. For me it is the same with caffeine although some people do tolerate & even benefit from that, but for me it is a strong diuretic. It doesn't affect my social life personally as I am housebound, but good friends should understand and not put pressure on you to drink. B x

No, but (health board funding dependent as I live in Scotland) I will be going back to the autonomic unit at UCLH in the next few months for relevant POTS autoantibody tests. I asked about IVIG, they don't use it but wait for it... if positive they may offer PLASMAPHERESIS which is safer, probably more effective, & often requires placement of a central line so you would be much likelier to be able to get additional IV saline!!! Not getting my hopes up because I have EDS & no sign of any autoimmune disease, but I was blown away at the thought of accessing this in the UK! B x

There are many different medications that have been used with success. If possible I would try and see an autonomic specialist, they would be better placed to treat you than a cardiologist who doesn't understand the condition. It is important though, if you have not already done so, to exclude alternative conditions such as Addison's disease, diabetes insipidus, phaeochromocytoma etc & for that reason seeing an endocrinologist or generalist would be a good idea. There are a number of treatable conditions that can mimic or present similarly to POTS. In the meantime, as well as the above lifestyle measures, one thing I found that massively transformed my quality of life for about a year was Licorice Root capsules. I think these are mentioned on the "what helps" page of Dinet. I take Swanson's brand, 2 x 450mg capsules daily. They can be bought from Amazon & various health food stores. With these, make sure you get the kind with the active ingredient glychyrriza in it. Some brands of licorice have had this removed - these are labeled DGL- and are ineffective. Exercise, if you can tolerate it is also beneficial in minimizing deconditioning which makes things worse for us. I use a recumbent bike. Best wishes, I hope you get some medical help soon. B x

I'm glad you're getting checked for a phaeochromocytoma - everyone with POTS or similar symptoms should have this ruled out. Blood or urine catecholamine levels are also important. I think we all hope we've got one as if you have it is CURABLE! B x

Do you just take it at bedtime then? I find a tiny dose before bed very helpful in getting a full night's rest and feeling less dehydrated in the morning. It doesn't help me stand any but it does improve my quality of life. I would be worried about taking it during the day due to potential hyponatraemia, particularly as I don't produce renin or aldosterone & don't want to have to fluid restrict. Also do you take the nasal spray or tablets? Even one spray was far too much for me - I didn't pee for nearly a whole day and my BP went very high. I split a 0.2mg pill into 6 so take about 0.03mg which works but I believe my body is very sensitive to it due to having no production of my own (except when being infused with hypertonic saline for the test which isn't the case in daily life). B x

Yes this is a concern for me being chairbound as immobility raises the risk for clots. I do take low dose aspirin, obviously hydrate tons & use a recumbent exercise bike which I hope will mitigate the clot risk. I am guessing it is the Mirena you have? I've thought about this too, it might be a good fit ie no or little bleeding but still the monthly hormone fluctuations if it didn't suppress ovulation. B x

Something else I thought of - on another site I read about someone who was drinking gallons of an electrolyte sports type drink for hydration which turned out to have an artificial sweetener in it known to cause diarrhoea. If you are using anything like this check the label for sorbitol or other artificial sweeteners. B x

Has it helped? B x

It's interesting that some people find IVs more effective run slowly. I can only get them occasionally & have always had a litre over about 1-2 hours for logistical reasons. Also, I thought getting it in quicker would be better in order to try and exceed the rate at which I pee it out, but even then most of it is in my bladder before the bag is done so I may as well be tipping it directly into the toilet. Maybe next time I will try a longer infusion period.. Perhaps retention is improved at a lower rate. B x

If you can access it there, Ivabradine may be a good option for reducing heart rate instead of a beta blocker. It doesn't reduce BP or cardiac output like beta blockers do. I haven't tried it as I tend to have a low resting HR but have heard good things from other patients who have tried it. I couldn't tolerate beta blockers at all, made me pass out more. Good luck with the cardiologist. B x

Hormonal BC pills can help by expanding blood volume through fluid retention as well as reducing/eliminating bleeding. Experiences with POTS seem to be very variable, some love them some hate them. I've been on continuous combined hormonal BC since long before developing POTS & have considered stopping it to see if I improved - my periods were heavy but not that frequent & I remember I seemed to retain fluid premenstrually, so I have wondered if my natural cycle might give me some good days vs all bad, but haven't been brave enough to try. How long have you been off them? If it just a few days it may be worth giving it longer until your natural hormones kick back in, but if it's been weeks then it may be worth looking at another brand perhaps. What side effects did you have? I can't imagine many that are worse than POTS! B x