Anyone With Hypermobility Syndrome And Pots

[jodie watson]

Hi to Everyone

I am new to the forum. I am an Australian mother of 4 (daughters) and I have had orthostatic issues all my life since my early teens. They didn't know what it was then of course. I was diagnosed with Orthostatic Hypotension 8 years ago after the premature birth of my third daughter. I was up and down since then and have had a particularly bad relapse which doesn't seem to be getting any better; worse if anything!!

I ended up in hospital in March last year and was diagnosed by a Cardiologist with POTS. I was prescribed Coralan (drug is Ivabradine) and it seemed to help for a short while and then stopped working, it was increased recently and my symptoms got so much worse after 3 months on the increased dose that I have been off it now for 2 weeks.

The reason for the title of this post is to find out if any of you have or know about joint hypermobility. I have done some research of my own on the internet and there seems to be a lot of medical articles that point to a correlation between this and POTS.

I am waiting to see a Dr in Australia in Melbourne which is a 3 hour flight from me, although I have spoken to him on the phone for about 45 mins. He is convinced from our initial conversation and answers to his questions on the phone that I have joint hypermobility syndrome and he believes it goes hand in hand with POTS. I will know more of course when I see him, but that may not be until the end of November at the earliest.

So I guess the members on the forum would know about this if they have it themselves or perhaps have more information on this connection. This Dr believes that this joint hypermobility causes the veins to be stretchy and that they do the opposite of what they are supposed to do when we are upright, which is to constrict. He believes that this causes the blood vessels to dilate and stretch much more than they are supposed to as part of the effect of this hypermobility syndrome.

Does any one who has both know about, or have been told this???

Look forward to your replies and help. I am glad I found this forum during my research into POTS and dysautonomia as it is wonderful to be able to talk to and question others who have this rare condition.

Kind Regards

Jodie

[sugartwin]

Hi, Jodie and welcome. I think you'll find quite a few of us on the forum who have both Hypermobility Syndrome (or Ehlers-Danlos Syndrome, prevailing attitude is they are the same disorder) and POTS. It's a fun combination of disorders, never a dull moment.

But yes, there is a MASSIVE correlation between the two. One study found that 78% of people with hypermobility syndrome displayed some form of autonomic disruption. As to why, there is no consensus yet. The three major theories seem to be:

1. Abnormal connective tissue in the veins causes them to stretch when people assume the upright position, thus allowing blood to pool and causing a reflex tachycardia.

2. Patients have unstable spinal columns which allows the brainstem, which is the 'control center' of the autonomic nervous system, to be compressed when in the standing or sitting position. This is effectively a kind of Chiari Malformation, which even has its own name, the syndrome of occipitoatlantoaxial hypermobility.

3. Patients have abnormal connective tissue in the small un-myelinated nerves that control the autonomic nervous system. This abnormal tissue is either attacked by the body as foreign or weakened by nature of its flawed design. Over time, autonomic dysfunction occurs.

Personally, I tend to lean towards theory three because patients with other connective tissue diseases such as osteogenesis imperfecta (brittle bone disease) and Marfan Syndrome also are prone to dysautonomia, although not as prone as we are!

I have a HUGE file of medical journal articles which discuss the relationship between EDS and POTS and other forms of autonomic dysfunction. I can e-mail them to you if you like. Most of them were free, some of them were accessed using my grad school's (admittedly crappy) permissions on medical databases. Some of them I just bit the bullet and paid for. You can use PubMed or Google Scholar to pull up all sorts of abstracts from various studies.

[jjb]

I would become familiar with all of what Sugartwin posted.

Here is one link (with translations) on hypermobility & chiari

http://www.chiariconnectioninternational.com/Hereditary_disorders%20_connective%20tissue.php

Also .... it is VERY important to be sure your doc has the right DX for your hypermobility as some types are more serious than others.

Here is a link to an overview of EDS:

http://emedicine.medscape.com/article/1114004-overview

J

[Chaos]

I agree with Sugartwin. That 3rd theory seems to make more sense for me personally as they said my veins, while "too floppy" when lying down actually responded very well when they tilted me.

Welcome to the forum Aussiemum. Sorry to hear that you fit in with our group so well but glad you found us. You'll find a lot of us here have EDS-HM or Classical type EDS in conjunction with the dysautonomia issues.