bombsh3ll

Hi Clb75,

I'm sorry the Northera didn't work out for you. Have you ever had your catecholamines tested? I think Northera is probably best for those who under produce noradrenaline, and based on my symptoms of sympathetic excess I don't feel I am in that category either, although I have yet to have mine tested. 

Thanks for taking the time to report back on your experience. I think it is always worth trying something even if just to cross it off. 

Bx

Lieze i am so sorry for what you are going through. I understand how difficult it can be dealing with insensitive, and in your case it sounds like downright selfish, family members.

My husband has been very supportive but I know it wears him down. My dad is also 78, he has features of EDS which i clearly inherited it from but he 's never really had any limitations from it, and he really struggled to accept my illness for the longest time. He helps me a lot practically, but I pay for it in terms of upsetting comments & frequently having to 're-explain my illness.

I think sometimes we just have to be blunt and protect our fragile bodies and hearts. If your dad can date, he can probably shop & do his own chores too. Your kids are certainly of an age they should be helping you, not letting you go alone to buy all their food etc on a mobility scooter. i would feel ashamed if i were an able bodied youngster expecting my disabled parent to do that. My little two are 9&5 they do age appropriate things in the house to help.

Your husband's threats to divorce you for being unwell are cruel - do you get anything out of a relationship with someone like that aside from financial security? It may be worth just for some peace of mind finding out where you would stand in terms of settlement - you may just be needlessly living in fear of something that to an objective outsider would look like a blessing rather than a catastrophe.

I hope you can make your family see they are being deeply unfair. You aren't being unreasonable at all, & you didn't ask for dysautonomia.

Another basic thing sorry if you've already tried or it isn't available where you live, but I'm largely housebound  & order in all our food& household items online. If I have to be disabled, i am thankful to God that we live in a time with such technology. Imagine being like this 30 years ago, no diagnosis, no online anything, never connecting with anyone else in the world with the same disease as you, or even knowing there was anyone else with it!

I hope this makes you feel a little less alone. Look after yourself,

B x

Honestly it is so worth getting it, a slightly sore arm for a couple of days is nothing to dysautonomia warriors like us!

B x

I always get mine. The last thing you want with POTS/dysautonomia is the flu. I've never had any side effects from the vaccine. 

B x

Hi I just recently tried Trioral sachets as opposed to the pleasant tasting but expensive supermarket ORS sachets. Even after shipping to UK and customs, it works out much better priced - I drink a litre twice daily. 

Problem is - it tastes foul!

I know that having had to stop work, my family are making lifestyle sacrifices due to my reduced income, and feel like it's only fair that I switch to the £1 per day rather than £4 per day product. 

I've tried adding blackcurrant diluting juice (the supermarket sachets I like are blackcurrant flavoured) but the effect just isn't the same. 

Wondering if anyone else uses Trioral and if you have any tips for making it more palatable?

B x

Hi Megann,

I have POTS and I have all the symptoms you describe apart from seizures. Checking your orthostatic vital signs and keeping a record to present to your cardiologist is a great idea. I would also tell the cardiologist what you have learned about POTS and why you think you may have it. This is not telling the doctor their job, it is providing useful information that will help guide the consultation. As a doctor myself although no longer able to work, I always found it a good thing when patients said what was on their mind if they were concerned about a particular condition rather than keeping quiet and hoping I'd read their mind. 

Of course, the patient may not be correct, but at least their concern can be addressed and reasonable conditions ruled out. Not every cardiologist is aware of POTS, certainly I had that problem myself when my tilt test was reported "normal" because my blood pressure didn't drop, but a good doctor will be open to learning and/or referring you to an appropriate specialist.

Best wishes,

B x

1 hour ago, WarpedTrekker said:

Is this some type of dysautonomia and should I get retested?

It could be, but is complicated by your other issues and medication. Have you had a tilt table test done? Do you have any symptoms with your fast heart rate? What is your blood pressure like? A lot of patients with POTS seem to focus primarily on their heart rate, but to me what's always mattered is how I feel rather than numbers. My main problem is lightheadedness & presyncope. I know I have POTS because I've been tested (and now wear a Polar heart rate monitor) BECAUSE of my symptoms, but I would've never been checking my HR in the first place if I felt well. Also how bad I feel doesn't necessarily correlate with my HR. 

I think it is a good plan to see a dysautonomia specialist & hope your appointment is helpful. 

B x 

Thanks for sharing your experiences - it sounds like each place does it differently!

I can sometimes walk for 10 min but not stand in one place - I think the walking option for me is the best chance at getting an upright result even if it's not perfect.

B x

19 hours ago, p8d said:

Yes, I stood for 10 minutes. If remember correctly NE hit somewhere in the upper 600s, range was to 400.  

Did you have it measured supine first and do you remember what that level was?

B x

I have to say I do not like the term "functional" particularly when used by neurologists. It carries vaguely offensive connotations and is often used or interpreted to infer the patient's symptoms are psychogenic. 

When I was a young and healthy medical student, I remember such terms being bandied about with a bit of a wink and a snigger, along with "supratentorial" or "non-organic". 

I suspect a lot of things currently assigned such descriptors will, as science advances, be identified as having defined underlying pathology, similar to how epilepsy was misunderstood in previous centuries. 

B x

Nothing really worked for the headache for me other than stopping it, however I had no benefit from it to weigh up against. I very definitely experienced splitting headaches within hours of the first dose, so if your daughter's already started taking it and has no headache, she may not be one of the people who gets this side effect at all. Starting low and building up the dose slowly are also advocated for minimizing headaches. 

I hope your daughter does well on it!

B x 

3 hours ago, Pistol said:

I had my catecholamines taken early on ( after just 2 years ). They took them after reclined in a chair for 30 minutes and then I had to walk for 30 minutes around the hospital. Then they took it again. I was elevated reclined ( above 600 ) and walking ( almost 900 ).

Was that at Dr Grubb's clinic? If so it might help me argue for a similar adapted test in London. At least your supine levels alone gave useful information in that they were elevated. I've decided definitely to ask for walking or at least independent standing. I have an absolute phobia of being strapped to another tilt table, that was the worst experience of my life when I had it done in Edinburgh, and it was of no benefit to me whatsoever as it was reported as "normal" despite a HR of 154 and BP of 170/110. They then deleted the test data (or said they had) so I couldn't take it elsewhere for a second opinion, delaying my diagnosis of POTS by another year until I got to Prof Newton. 

B x

11 hours ago, p8d said:

I had to stand the 10 minutes at a separate appointment from the tilt.

So it was 10 minutes standing to get the upright bloods, that was enough? If I do get the funding to go back to London for the test I'm going to ask if they can be more flexible and allow me to walk about a little bit in order to have a better chance at being upright for 10 minutes. Some places like their very rigid protocols though! Do you remember how much your NE went up by? Symptomatically I feel that mine is high even sitting and rockets if I try to stand, and I would like to capture that, but I am not sure it would lead to any new treatment options in my case as I've already tried pretty much everything (NHS won't treat POTS with IV fluid).

B x

55 minutes ago, Crazycatlady said:

If you can only stand to stand (punny...) a minute or two shouldn’t that be enough?? I hope you get moved on the waiting list for some answers 💕

Thanks, I think the consultant said less than about 10 mins isn't usually enough. I'll give it all I've got though! I just wish it hadn't taken so long to get to see an autonomic specialist - earlier on in my illness I may have been able to stand for longer, especially when licorice root was still working for me. I've thought about asking if I can walk about instead of being strapped to a tilt table to get the upright blood test as I may be able to do that for longer. 

I know when I was at the hospital getting renin and aldosterone levels, they asked me to stand for 15 minutes.  I pointed out that if I could stand for 15 minutes I wouldn't be in a wheelchair, in fact I wouldn't even be there, I'd be running about somewhere jumping for joy!

B x

I'm sure disorders of CSF pressure in either direction can create problems, in fact I repeatedly expressed concern about HIGH intracranial pressure. I have been for repeat eye exams to exclude any signs of this. I am angry that the neurologists and neurosurgeon didn't pick up on the fact that splitting headaches when upright relieved by lying down are characteristic of a spinal leak & consider this possibility, especially when the patient is mentioning intracranial pressure (albeit the opposite concern), and has known risk factors - EDS, precipitating Valsalva. 

Maybe I don't have it, but the diagnosis should be considered and excluded, rather than nobody having thought of it. It wouldn't be OK to go to hospital after a car accident with severe leg pain and unable to walk, & nobody thinks of or looks for a broken bone. Many people won't have one, but it should be ruled out. 

I also don't buy it about lax blood vessels - surely this would have come on gradually. I would have had lax vessels at 16.55 on the day I fatefully blew up that water bomb, not just since 17.00 when it happened. Also my dad who also has EDS is 78 and has awful varicose veins and an aortic aneurism, & he can stand up!

B x

I'm so glad you're having positive results from the fludrocortisone increase (and rather jealous!) 

I am sure the drug's onset of action really is that quick, as that is the time frame in which it makes me feel awful when I have tried it!

Hope it continues working well for you!

B x

Epinephrine is meant to be low, and having readings below the threshold is unlikely to be clinically significant. It is good in a way as it helps rule out a phaeochromocytoma (although actually most of us would probably prefer an operable cause!). Your Ne rise is consistent with POTS (possibly Hyper dep on your center's criteria).

I am on the waiting list in the UK to have supine and standing blood catecholamines drawn if I can get health board funding. The problem is I can only stand (or tolerate tilt) for a minute or two, so the doctor didn't think this would be long enough to meaningfully detect a change. Can I ask how long you were upright before your standing bloods were drawn? 

I hope your results point you in the direction of some helpful treatment. 

B x

It's great hearing about people who were finally cured of a debilitating illness when they just found the right diagnosis!

I could be clutching at straws- don't we all long for a treatable cause to be found - but I am determined now to be fully evaluated for this. I know it probably isn't relevant to a large number of people but having EDS, onset triggered by forceful valsalva, splitting headaches when upright relieved on lying flat - I emailed Dr Carroll & he thought I had a good history for a spinal leak. 

Problem is very specific myelography imaging, read by someone experienced in this field is needed to accurately diagnose it, & even then it can be missed. 

I'll have to wait a while to see the neurologist I've been referred to re this, & I'm gathering info to argue my case for the right investigation.

This study is interesting https://journals.sagepub.com/doi/10.1177/2515816318773774

B x

Yes it's hard having kids as a non-upright person! How frustrating that your specialists don't agree. Have you ever had a CT myelogram to look for spinal leaks? This seems to be the test favoured by Dr Carroll. 

I've been referred to Dr Tyagi, an NHS neurologist in Glasgow who has at least heard of CSF leaks/spontaneous intracranial hypotension, but I am not sure if the CT myelogram is available in the UK. If I don't get anywhere on the NHS I plan to try and get to Stanford to see Dr Carroll. 

What type of EDS do your family members have? I am diagnosed as hypermobile type based on Beighton score, but have never had genetic testing. I understand the other types have genetic tests but there isn't currently one for HEDS. 

B x

How awful it sounds like you've been through a lot! Is your leak cranial then if the fluid runs down your nose? Was it caused by surgery or spontaneous? I do hope you can get some relief. It is encouraging that blood patches helped even if transiently. 

B x

Yes I am pretty sure it is the TOP end of the bed for autonomic disorders - it is meant to be for longer term volume expansion rather than short term comfort & cerebral perfusion.

B x

Hi and welcome!

I can relate to what you're feeling, my situation isn't quite the same as I have a husband and kids, also my dad stays with us a lot to help out, but my biggest (probably irrational) fear is that my kids (9 & 5) will find me dead & be terrorized, say when my husband has left early for work and my dad isn't here. I always sleep easier when I know there is going to be another adult there in the morning. 

I know POTS isn't supposed to be fatal, but having next to no blood flow to your brain, plus all the red flag symptoms you are supposed to seek emergency care with ALL THE TIME eg chest pain, shortness of breath, passing out etc can certainly feel like it! How would you know if you ever did have a real emergency when you feel like that constantly?!

I like the idea of a wearable alarm, although you would have to be conscious to activate that, also a roommate or a service animal are good suggestions. If your building permits animals, I suppose even an ordinary dog as opposed to a trained service dog would help, as it would surely start making a lot of noise if it wasn't being fed or let out.

You could also make an arrangement for a family member to call you at a certain time, once or twice each day, with the understanding you have a phone on you all the time, so they could raise the alarm if you weren't answering. 

I hope you manage to find something that gives you some peace of mind. 

B x

I recently had to put together a collection of my medical info for disability, and I was reading over my hospital letters, did a bit of thinking and online reading and came across a very interesting presentation by Dr Ian Carroll who spoke at the dysautonomia international conference on CSF leaks. 

I have EDS (only diagnosed after POTS, lifelong weak collagen/weird surgical issues but nothing disabling so wouldn't have seen geneticist otherwise), and was COMPLETELY well until a very forceful Valsalva (blowing up a water bomb I thought was a balloon) at which point life as I knew it ended instantly, and I could no longer stand or walk without passing out. Along with this came severe nausea and a splitting headache and neck pain whenever I was upright, yet lie me down and my old personality, IQ & light behind my eyes are back within minutes. 

Like everyone on here, I would so love to find a treatable cause for my POTS. I was tested for Sjogrens due to dry eyes and mouth but my lip biopsy was normal. I have seen 2 neurologists and a neurosurgeon and repeatedly expressed concern that the Valsalva could have caused HIGH intracranial pressure, but none of them (nor I to be fair - I am a GP) ever considered the opposite possibility, that I could have triggered a CSF leak with LOW intracranial pressure. 

Dr Carroll's talk went into detail about a number of POTS patients actually having this, especially people with connective tissue disorders like EDS. I know that EDS is associated with OI generally, attributed to lax vessels that don't constrict, but surely that would come on gradually, not instantaneously with one event that is a recognized trigger for spontaneous CSF leaks. 

There is another video on Youtube by a young lady called Kristin Ann who had POTS and turned out to have a leak and was fixed by this guy. Her symptoms sound like me to a T. None of the standard POTS medications have helped me either. 

I know this is probably clutching at straws, but has anyone else here had, considered or been tested/treated for a CSF leak?

I would love to hear your experiences if you have or know somebody that has.

B x

Hello and welcome Dogmom, sorry you are suffering but you have come to the right place to get support and feel a little less alone. 

I am 38 and have had severe orthostatic intolerance since 34. I fainted a handful of times when I was younger but was well in between. My diagnosis is POTS. I've never really found any medication with lasting positive effects. 

The heart stopping and BP plummeting are quite typical with neurocardiogenic syncope, & unfortunately pacemakers have not shown to be terribly effective in preventing fainting in younger, otherwise healthy people with NCS, but it is good that you have had a work up by a cardiologist. 

Do you have a BP cuff or a heart rate monitor/watch at home that you could use to check your numbers when you feel presyncopal? That would help provide information for your doctor which could guide treatment. 

Frustratingly when I am sitting, my heart rate and blood pressure are usually perfectly normal, but I still feel extremely lightheaded and miserable. 

I hope you manage to get ongoing medical care sorted out soon. 

B x

Hi I'm sorry you're having difficulties getting your much needed med, I know how I would've felt when licorice root was still effective for me if I hadn't been able to get it!

A couple of possible (hopefully temporary) alternatives that you could ask your doctor about are guanfacine or clonidine. Another possible option is to look at overseas pharmacies that you could send your prescription to - the supply problems may not affect all countries. 

Good luck, I hope you manage to get it sorted!

B x