Eds Question

[roxie]

I went to a new specialist this week and he strongly believes from the timeline of my illness and flexibity that some form of EDS if the root cause of my POTS. It'll be a few months before I can get to his preferred geneticist.

Anyways, I don't know a lot about EDS and from what Ive recently read some forms are fatal and that scares me. Does anyone know if the form that causes POTS is progressive & fatal?

In the 14 years I've been sick I haven't gotten worse I've been perpetually stuck. He says it's because no one has address the collagen problem of EDS so until that is helped no POTs med will help.

But I'm just nervous about this shortened life span and anuerism stuff now. Does anyone know?

[louloutinks]

Did your doc mention to you the vascular type as you mention aneurysm?

It is mainly the hypermobile type (EDS3/EDSH) that is prone to POTS.It is mostly not fatal but a minute percent there maybe issues with vessels in the brain (thye checked mine the other week due to headaches) and all was well. There also can be an overlap of some of the symptoms from the other types mixed in and it is not always 'cut and shut' to the profiles. EDS3 causes me lots of subluxes, joint pain and easy tiring. I have EDS3 but I also have marfan habitus.

Are you being referred to someone to get it checked out? How flexible are you - very..as in most joints or just one or two?

[roxie]

He only asked if anyone in my family has died of an anuerysm? No one has that I know of.

He is going to do more testing over the next few weeks including a transcranial Doppler on the TTT, that show brain problems right?

He is also very interest in my pelvis and pelvis congestion.

I'm very very flexible. From the little testing he did so far he said my Beighton score was a high 7 possible 8. With 9 being the highest.

[rubytuesday]

I have EDS III (but possibly Classic) and was diagnosed by both a rheume who specialized in fibromyalgia and a cardiologist specializing in dysautonomia--very well versed in EDS. I see genetecist for the 'official' diagnosis in about 3 weeks. As a youth I was always super flexible--bending backwards with hands touching floor, bending forward with palms to floor, Chinese splits, regular splits, touching thumbs to arms and some fingers to arms, hyperextend hands, fingers, bend fingertips to 90 degrees with fingers otherwise straight, kick over my head, put both feet behind head while sitting, was very athletic--climbed trees, played baseball, basketball, gymnastics, cheerleading, drill team, could do headstand by having hips out and then up was easier for me than with legs just behind me and up but I could do either way, flips and probabably more that I'd took to be a normal kid. I always had problems with GERD but never knew what it was as a kid. (Our family was large and as one of older kids, parents were busy watching younger ones and we were not wealthy--never went to doctors). I had asthma (listened to the whistles and squeaks coming from my lungs when I'd be sent to trek for Saturday mornings or to grandma's house when it was cold outside. It was not diagnosed until I was an adult because I never knew to mention it to parents, thinking it was normal. I was always with a tissue--cold/sinus infection, hay fever (again as adult diagnosed with boatload of allergies). I was a bleeder--fell and tiny cut inside lip left my clothes soaked in blood, a blood trail from schoolyard to our home (DS was with me and people were coming out of their homes to see if I was ok with this look of horror on their faces that scared me more). It took forever to get it stopped. I had migraines (again not diagnosed altho my DM and DGM had migraines) and could always count on being sick Thanksgiving and Christmas holidays and spring. I think the former 2 might have been related to live Christmas trees. I always dreaded the smells of Christmas because I knew it meant headaches. I would nick my leg when shaving and bleed like the dickens and DM scolded me saying that I didn't know how to use razor and would not be allowed to shave if I cut my leg one more time (cuts were very superficial but left large white scars for years). When menstruating, it was heavy (as was my mom). As I got older, (out of the home), I had to have injections to stop the hemorrhaging when I would menstruate. I had preemie and 40 hour, difficult/complications with labor. I had a miscarriage. DS was born with heart murmmer and now that he's in 30s after injury that was slow to heal they'd found congenital dilated aorta. DGF died of ruptured aneurysm--but he was not a doctor goer and was elderly by the time they found it--had dementia and didn't want to put him through such a surgery to repair it. After my preemie was born and miscarriage, I was found to have a pituitary tumor (microadenoma) that my sister said neurosurgeon said could be 'hereditary'. I also was found (sometime assessing CTs for migraines or sinus infections a possible lacunar infarct in the brain--sent for MRIs to have Chiari I). I'd always had motion sickness (even with merry go rounds on play ground, spinning rides at fair, riding in the seat of the stationwagon that faced backwards). I had the tendonitis, chostocondritis, pleuritis, tenosynovitis, gastritis, pericapsulitis, diverticulitis, RSD following tendon surgery, surgery on both shoulders and feet, arthritis (early onset), early onset cataracts, early onset osteopenia, scoliosis, had critical vitamin D deficiency, off and on iron def. anemia, off and on one of the B deficiency, Delta Granule Storage Pool Deficiency, osteomyelitis, and just found with T cell, T helper cells, IGg1, IGg2, other IGg subset defficiencies and low gamma globulins. I've had terrible trouble with IBS and have had polyps removed from colon and have persistent gastritis despite 2 meds and diet. I have alway run a low normal blood pressure and temp until the past (almost 3 years now), I had POTS about a year ago until this summer (when taking no meds for it while awaiting cardiac specialist to clear things up since so many meds had tried and failed). Now I am told by this specialist it is Orthostatic Hypotension and that my autonomic nervous system is failing. With the bleeding problems, I did ask the cardiac specialist if I could have the vascular EDS and he said no, that EDS affects the veins/vessel structure, too (explaining the hypotonia in my arms and legs) and that if I had vascular type I would already be dead (am in my 50s). DS mirrors all of my tricks and health issues (save the fibro/chronic fatigue/chronic myofascial pain/hemorrhaging) and with his dilated aorta I'm certain he has inherited this gene but he is in denial. He has chronic sinus/ear/throat infections/bronchitis, had more than the 'normal' growing pains as a child, was very colicky and has bowel/GI issues all his life, migraines, asthma, allergies, early onset arthritis, broken bones, sprains, pulled muscles/ribs, nosebleeds from childhood to adulthood that would just start abruptly and had to go to ER a few times to have vessels cauterized to stop the bleeding. He has kids by 2 different moms--one going into healthcare so she'll listen, but other doesn't even have GED and wouldn't have a clue to what I was telling her--would be like talking to the wall. Males are less symptomatic w/ EDS than females and the mother who is educating herself has son, but the one who is uneducated has daughter. All I can do is try to explain to them what to watch for and when to do what.

After pituitary surgery, and always looking 'rheumatoid' (according to my doc), I've had the full gambit of testing and always watching the endocrine function, thyroid function, neuro exams, etc. Forgot to add the very stretchy skin and velvety skin (the rheume specialist said--I never knew my skin was 'velvety', had never heard of EDS hypermobility). I have vitiligo. ANA and ESR are always negative. RBC and WBC are within the normal range but it is the components within the WBCs/platelets that are lacking so now the cardiac specialist has referred me to hemeoncologist to see if there is anything to explain. I see him the day before Thanksgiving. I have multiple drug allergies--some docs tell me it's longest list they've ever seen and I have other allergies/sensitivities to meds and to environment. I have had 2 sleep studies (2 different docs at 2 different centers) when diagnosed with the fibro. I do not get into deep phase of sleep, almost nil REM and what 'little' sleep I got was in stage II with sleep intruded more than 150 time by alpha waves (waves that wake one up in the morning). I bruise easy--large bruises for small hits. I am always walking into things and have proprioception problems. I've had what I had only know to describe as 'fibro fog'--the harder you try to think of something, it would be like staring at a blank page with a brain freeze. Cognition and memory problems are worsening. I'd wondered if brain cells were damaged from syncopal episodes. I had overactive bladder that has since seemed to calm down for now. Now with the dysautonomia more pronounced, I have the purplish black feet when standing and pool fluids in legs/feet even tho I keep them up/flexing as much as I can and can now have no salt and must take water pill. I think I understood the cardiac specialist to explain EDS as a component of this as well as the dysautonomia.

I have TMJ and had to wear splint before they could put me into braces (in my 40s)--too many teeth for such a small mouth and I have high arched roof of my mouth and no little skin flap that attached upper lip to gums that I can feel and only tinsy weensy one that connects bottom lip to gums.

I don't know if any of this helps you or not but this is my experience having dysautonomia and EDS.