Pots Caused By Eds?

[BeforeTheMorning]

Is POTS caused by EDS actually something gone wrong with the autonomic nervous system or is it simply because of stretchy blood vessels? I guess it probably has to be something more on top of just being too stretchy or everybody with EDS would have POTS, right? Does having EDS just make you more likely to get POTS?

Sorry for the ramble, I'm kind of thinking aloud here!

Lyla

[andybonse]

There is no research to answer this question. I too want to know.

My doctor is researching this soon, looking at the actual tissue, nerves, vessels of someone with EDS/HMS and POTS/Autonomic Dysfunction. I cant wait.

I cant believe its not been done yet.

[BeforeTheMorning]

Wow I can't believe no one's done this yet either! I'm glad someone is going to be researching this soon.

Thanks,

Lyla

[andybonse]

If not, I'll be saving $10000 and fund it myself lol

[~elizabeth~]

The truth is, no one knows what the mechanism is. All I was told is that it's due to 'bendy veins' which frankly I don't really buy as a solution as it doesn't explain why I've had so many odd neurological symptoms and tachycardia present at the same time as the erythromelalgia started, despite having bendy veins and very low BP for all of my life.

The last neurologist I saw diagnosed the facial flushing (previously misdiagnosed as MCAS) as facial erythromelalgia, and thinks the cause is likely to be a mutation in a particular sodium channel. Having spent the last few days researching this, I now find there are links between NaV 1.7 sodium channel polymorphisms and dysfunction of the sympathetic ganglia. It's known that people with EDS are often insensitive to lidocaine, and also have accompanying tendency to experience hyperalgesia in response to painful stimuli, which are both signs of defective sodium channel function.

My guess is that this will eventually prove to be a large part of why sympathetic system is dysfunctional in people with EDS, along with lots of other neurological deficits that have a sodium channel pathology (vertigo, tinnitus etc). My head pain neurologist wants to get funding to do a study on me, which will involve genetic testing so I guess if there is a connection then it will eventually be brought to light. He also thinks that facial erythromelalgia is probably underdiagnosed (not surprising given how everyone on these boards makes an automatic assumption that ALL facial flushing/burning is MCAS). I do find it quite worrying that I had to see a headache neurologist to get this problem correctly diagnosed, having been assured by the autonomic department that it wasn't a channelopathy. If they missed it in me, then they may be missing it in other people too.

http://forums.dinet.org/index.php?/topic/24785-nav-17-mutations-and-sympathetic-dysfunction/

[andybonse]

I find stretchy veins to be a bit unbelievable, especially since I was fine all my life until 22 and it suddenly got worse in a day, veins dont all become stretchy suddenly.

[HopeSprings]

I don't understand why they haven't looked more closely at this either, considering how commonly EDS and dysautonomia go together. Andy, can I ask which Doctor is doing this research and what his thoughts are on the whole issue?

[~elizabeth~]

My blood pooling/POTS appeared almost overnight in my legs quite soon after the erythromelalgia started, they'd been a reasonably normal colour before then, something suddenly caused a widespread dysfunction in small C fibres and autonomic nerves. I'd had dysaesthesia sensations in my face/eyes for about 8 years before that, which is exactly the same time frame during which I'd noticed my muscles were weakening and that I'd become hypermobile (I certainly wasn't as a child, I remember being pilloried by a teacher for not being able to get my hands much below my knees, let alone touch my toes). Personally I think EDS III will turn out to be due to a number of different syndromes, rather than a single disease entity/mutation.

[Chaos]

I think one of the reasons the research hasn't been done is because they didn't recognize how many other problems were associated with hypermobility until recently. I think Peter Rowe was one of the first to make the connection between EDS/hypermobility and the autonomic issues so many of his patients were having and that was just in the last decade or so. Perhaps in the UK there are more advances in research being made, but here in the US, the vast majority of schools are still teaching that hypermobility just means patients have more range of motion than usual and have no clue as to all the other issues that go along with it.

Elizabeth- you may be right. Personally I've been hypermobile my whole life but only developed POTS in 2007.

[kitt]

According to Dr Nancy Klimas, (an immunologist who specializes in CFS), she explained it to me this way when I asked about the relationship between EDS and pots. She summed it up with 'floppy joints / floppy vascular system'. She dx my pots (although not my hyperadrenergic state) but as a CFS expert she routinely screens for pots in her CFS patients due to the high viral load many CFS patients carry. There is a strong relationship, and yes Dr Peter Rowe at Johns Hopkins is responsible for much of the research on the relationship with children who have both EDS and CFS.

[andybonse]

I dont believe its a floppy vascular system, doesnt make sense since people get it randomly over night, just wouldnt happen.

[HopeSprings]

I'm not so sure it comes on suddenly. Many posts I've read on this topic have people tracing problems back to childhood.

[SpinnyC]

I've wondered about this too. Maybe it's a case of something within the mutated gene being "turned on". Or a function of age and changes within the body. I know someone who has a birth defect with their heart that didn't show any symptoms until they were in their late 30's early 40's. It was explained to them that symptoms don't show up sooner because a younger circulatory system compensates for the defect better than an older one.

[HopeSprings]

I've been thinking along these lines too Spinny C.