sugartwin

Sugartwin- I think I remember you saying something about a theory of it being auto-immune somehow? That the autonomic fibers aren't recognized correctly and the body starts trying to destroy them? There was some connection to EDS with this theory but can't remember what it was. Do you?

Thank you for reminding me, because I fully intended to list it when I started writing my reply but totally forgot about it sometime between then and when I finished! I blame the heat

Anyway, in EDS occurrence of autoimmune disorders is 30% more common. Some doctors speculate that this is because the placenta is more permeable in EDS, allowing the cells of the mother to mix with those of the child. Later, the immune system would be prone to attacks of its own systems. The un-myelinated fibers of the autonomic nerves might be particularly susceptible.

I haven't heard as much about this theory lately though.

There are no clinical diagnoses there because the doctors are true by-the-book scientists, so everything has to be confirmed with testing and numbers, and I think a lot of patients are a little turned off by that kind of culture.

That can be good and bad. It's good when doctors go ahead and treat symptoms anyway, even without a likely diagnosis. The problem with EDS is that for the Hypermobility and Classical types, the types that are most likely to be affected by dysautonomia, the diagnosis is almost always a clinical one. There is no genetic test for the Hypermobility type and for the Classical type, the test is only 50% accurate. The test for Vascular EDS is 98% accurate however and is sometimes done via biopsy. Is this the type they suspect you have? In any case, I strongly suggest requesting a geneticist (rather than a rheumatologist) to confirm the diagnosis.

As to why up to half of the population with Classical and Hypermobilty type EDS are affected with dysautonomia, the leading theory used to be that stretchy tissue in the lining of the blood vessels allowed blood to pool, causing a reflex tachycardia. However, after thoroughly testing the blood vessels of people EDS and finding them normal, this idea has been somewhat discredited.

Some people with EDS suffer from a Chiari Malformation, a result of laxity in their cervical vertabrae. This can entrap the cerebellar tonsils and lead to dysautonomia since this is the part of the brain that oversees the autonomic nervous system. This is a proven cause of dysautonomia and can sometimes be reversed by surgery to free the cerebellar tonsils.

Dr. Diana Driscoll, a therapeutic optometrist with EDS has proposed that subclinically high CSF pressure might be causing symptoms of dysautonomia, sometimes in concert with mast cell activation and chronic cerebrospinal venous insufficiency (CCVI). The details can be found at Dr. Driscoll's site, prettyill.com There are clinical trials in progress to test the integrity of this theory.

Dr. Thomas Chelimsky, a neurologist out of University Hospitals in Cleveland proposes that most if not all dysautonomia is a sign of mitochondrial dysfunction and that this is also the case for EDS. Clinical trials have just begun.

I think I got them all, lol. This is an area of real interest for me, and very, very few studies have been done, leaving most doctors completely unaware of the link between the two.

anyone here with it that just did the supplements vs the testing?

I'm just doing the supplements. The testing seems expensive, isn't fully conclusive, and in my area I would have to go to the Cleveland Clinic, something which I'm loathe to do unless I get a referral to a doctor I trust.

I pulled up this page over at UMDF. They have a lot of good info on doctors and hospitals too.

Getting a diagnosis of mito

Now if she refuses to eat, even for reasons of valid discomfort, she only plays into it. Is there a family doctor who can refute this ridiculousness? When I was in the hospital for constant nausea and vomiting and subsequent low potassium, they told me I was anorexic as well, even at 5'3 and 140 lbs.

Twenty pounds is a lot of weight to gain, especially for someone who is sick. If she is unable to gain 20 lbs, what do they intend to do about that? If force-feeding her like a goose doesn't work, what then? If she does gain this magic 20 lbs, will they suddenly have a breakthrough about her condition?

K. is lucky to have you as her advocate. Keep giving them a hard time.

I did look at the symptoms of EDS and I'm not double jointed or have loose skin. Sometimes my skin is sparkly and/or thin with enlarged veins, but the sports guy said if I had EDS I would have had broken bones or sprains as a kid.

From personal experience, it's not a good idea to try to gauge your own hypermobility and/or stretchy skinned-ness. Pictures of hypermobility tend to be extreme examples, making it easy for someone checking themselves to miss a more subtle presentation. And like Sarah said, a diagnosis of EDS or other HDCT is best sought from a geneticist specializing in those disorders. Other doctors are likely to have notions of EDS that are questionable, outdated or just plain wrong.

There are good and bad EDS Facebook groups. They are about as good as how well they are moderated. Any group is subject to be encroached upon by internet anarchy if it is not closely administrated as this one is. There was someone here a few months ago I think who was trying to market something and he was shown the door as I recall. Not that we aren't fab people (we totally are) but there are fab people on Facebook groups as well, when moderators exist to keep the bad eggs out.

For anyone who wants to read the full article, if you message me your e-mail, I can send it to you. I was only able to access it because my sister let me "borrow" her upenn id and password.

I've read the full text. The gist of it was that the doctors involved observed that POTS patients were more anxious and depressed than healthy subjects. And so they decided to test and see whether patients who were diagnosed with anxiety, depression and somatoform disorders had POTS, OH or NCS. And in their own words:

We had hypothesized that the prevalence of POTS and orthostatic [change in]HR response were higher in patients with psychiatric disorders versus healthy sub- jects because previous studies had indicated that POTS patients often experience anxiety and depression [3, 15]. However, this study suggests that this is not the case. It showed that orthostatic changes in HR are not correlated with severity of depression and anxiety, in agreement with a previous study on orthostatic stress in POTS patients and anxiety [5].

At my last appointment my neuro told me there was a strong possibility I had it. Based on some testing he'd done at my first appointment he'd put me on a kind of 'mito regimen' (some of the supplements are listed in my sig.) It did help a LOT with the GI symptoms, but I wouldn't call it the miracle cure or anything.

At the time he just called it supplements for my POTS and two weeks ago was the first time he actually mentioned mito to me, I assume because he was doing a clinical study about mito and POTS and wanted me to participate.

I agree Naomi about contacting the Mito Association. They seem to have considerable resources at their disposal, at least as rare disease advocacy groups go.

And don't give up until you get what you want out your doctors.

I really appreciate all the replies. I used to be a fairly optimistic person, but yeah, now my optimism tank is on 'E'. My symptoms aren't as bad as they were when I was totally untreated, but I'm worried that they are headed in that direction, especially since I seem to be getting bad rather quickly now.

And yes, yuliya, I feel like I'm the only one concerned about this slow moving catastrophe. Watching myself get worse is about as demoralizing as it gets. I feel better knowing I'm not the only one.

I suppose to my doctor's eye I'm just one of many patients, somewhere in the middle, not the worst off and not the best.

I have POTS secondary to EDS and my understanding was that while symptoms hung around for life, they aren't supposed to get worse. At first I wasn't sure that it was happening because the decline was so slow. But I'd think back on things I'd done in the past (say a year ago) and think, 'I'd never be able to do that now!' All the while I'd been keeping up with exercise, 25-30 minutes about four times a week. I'd come to the conclusion that it obviously wasn't going to make me better but maybe it could keep me from getting worse.

Then in March of this year I had a huge surge in my symptoms. I had a constant low-lying nausea, severe constipation, my blood pressure got labile, going from really low one day to high the next. At my rheumatologist appointment I begged him to call my neurologist and tell him about my symptoms getting worse.

(It turns out he called my neurologist and told him he thought I needed to get a job. So helpful.)

My neurologist takes lying and standing blood pressures and heart rates at each appointment so I got out the records from each appointment going back a year and a half. And the standing heart rates had been steadily climbing over time.

I felt so defeated when I saw that. Has anyone else dealt with worsening symptoms over time? What did you do? What did your doctors do?

I got the impression that her POTS was triggered by the parasite, not caused by it. And since the journalist only mentioned it as a "rare Asian parasite" the odds of picking it up stateside are probably low. Plus, what newspaper paper would pass by the opportunity for a little fear-mongering? If she caught it here, they'd have named it. I'm a former journalist, and I sure would have.

Does anyone know if EDS can show up later in life.

Absolutely. EDS often comes a-calling later in life. My mother wasn't diagnosed until she was in her early 50s. I was almost 30, hardly a child or young adult.

To be fair, I can't say I hadn't been having symptoms on and off all my life. The signs were just ignored or glossed over, thanks to general ignorance of EDS and its main secondary syndrome, dysautonomia.

I really want Dr. Driscoll to be right, because someone needs to figure out what is causing the multiple dysautonomias in EDS in order to focus the treatment. However, I had a sinking feeling as I watched the video because I couldn't relate to the symptoms she described except on the most superficial of levels. I wasn't a floppy baby; I walked early and hit my developmental milestones ahead of schedule. The symptoms she mentions don't match my own.

I'm not going to be silly and say her theory is wrong because it doesn't apply to me. However, it doesn't apply to me, which makes me sad more than anything because it means that any treatment that comes of it will likely not work for me.

I would doubt it's the cause. I have loss of cervical curvature as well, caused by EDS. I think the formal term is 'reversal of the normal cervical lordosis.' It's not terribly uncommon though.

I used to have some alcohol tolerance earlier in my POTS career, now I have barely any at all. I used to be able to handle one mixed drink, now even a few sips of hard cider is enough to kick off the adrenaline sweats.

I feel sorry for the people who are going to read this article and get the wrong idea.

Dr. Levine is not the first, nor will he be the last to rightly suggest that a course of exercise can be beneficial for certain patients suffering from POTS. When I was first diagnosed, my physical therapist put me on an exercise regime HE had designed for patients with POTS. It was helpful. It continues to be helpful. I'm not cured though.

I discredit his study mostly because it reeks of cherry-picking, that is, purposeful pushing and shoving around of data in order to arrive at a particular conclusion. He also dreadfully oversimplifies a very, very complicated issue and has now taken his oversimplification show on the road, so to speak. So people are rightly upset.

I don't see why it would be rare. I bet it's actually common. If you think about what is occuring in most cases of POTS, it would be unusual for the heart rate NOT to increase upon sitting up. In my case, even my resting heart rate is tachycardic because of how poorly I vasoconstrict. I used to get heart rate increases just from rolling over, because when you think of it, even modest changes in position require an increase in vasoconstrictive activity (just not as much as standing or sitting.)

It would be worth travelling to Baltimore to see Dr. Francomano who is pretty much THE EDS doctor on the east coast of the US. Since you say possible vascular EDS, I assume your friend is looking for a diagnosis?

It's not exactly a short trip though...it took me about two hours to drive and that was from Philadelphia which I think is itself two hours south of NYC.

It's called Paraneoplastic Autonomic Neuropathy and is actually relatively well-known. Not associated with all cancers, just a handful...certain lung cancers for instance and a couple more. You can read more about it here.

Position definitely matters. I might feel fine lying flat on my back with a pressure of 70/40 but absolutely rotten standing up with a pressure of 86/51. In fact, I start to get confused about my surroundings when I'm that low.

I'm not sure what's going on, because I was doing fine on my meds, but now my blood pressure is beginning to swing low again, like before I started taking anything. I'm not enjoying it.

I ended up reporting my primary to the board over the inaccuracies on my medical records. She was my doctor for just over a year and I was surprised when she abruptly discharged me after we disagreed over whether I needed a handicapped placard. After I paid to get my records I found out she never recorded a single one of my diagnoses. Instead she had me listed as a depressed woman possibly "hypochondriac" who enjoyed "doctor-shopping." When I wore (doctor-prescribed!) braces on my knees to keep them from subluxing she recorded that this was for no reason, that "no findings supported" their use.

And I always wondered why the doctors she referred me to were so rude, condescending and dismissive.

Since that, I ALWAYS go through my records, usually during the appointment as I'm waiting for the doctor; if they're good, they won't mind.

Not all POTS patients have low blood volume. Some do, some don't. I take tramadol and oxycodone as needed for joint pain from my EDS. Neither one is particular friendly to my GI tract, but Miralax helps and have Phenergan on hand for the nausea.

That aortic dilatation is found in EDS...were you worked up for that while you were at Mayo? That along with the fact that you mentioned pain bad enough that your doctor thought you needed a pain program.

I take Lyrica. It's not usually an anti-nausea medication so I have no idea why it works the way it does, except that without it I get so ill that I have to into the hospital because I can't keep anything down.

Sugartwin --Ok I don't mean to be dense here. Are you saying that autonomic neuropathy can be the cause of dysautonomia? I guess what I am really getting at is -- would it be worth pursuing going to a Doctor and asking to be evaluated for Small Fiber Neuropathy (hopefully getting a skin biopsy) and then -if that was positive- possibly conclude that this was causing the Dysautonomia?

Yes, that's what I'm saying (You're not dense! I'm just not good at explaining.) And I think it would be worth being evaluated for. In the end it's a personal decision, but it can help focus treatment decisions. I think it's ridiculous that a doctor would not consider searching for the cause of something like POTS...