rubytuesday

I wear Care Medical (caremedical.com? or caremed.com?). It has a USB device that you put in computer port (can be used on anyone's computer). The metal portion is protected and warranted to be waterproof. You can purchase them at Walgreens I think but I got mine on-line from its website. You can put in all your information and add information/categories that may not be there. It organizes all your medical info, surgical info, medicines, allergies, doctors and specialists, insurance information, contacts, assistive devices you need and so much more. You can update it on your computer and save it and upload any updates or changes at your leisure. I just love it. It has the medical alert symbol on the top (but if I can't speak for myself--everything is there and organized and able to be printed off by anyone who needs it).

I sit down immediately before I drop. I'm older than you but it is still embarrassing but not so much that I don't want to get hurt or killed in the fall (and I'm a bleeder). I always have DH with me and use cart as walker to lean over but if hits me giving me enough time--he helps me or I hurry to bench or seat. I am amazed at the volume of folks who pass me by--pale/pouring sweat/shaking/head down/weak/staggering who offer no help or make no inquiries if I am alright (so I suppose they must think I'm a drinker or on something--and we live in a not so large community). I always tuck a bottle of water with me and start guzzling it in as soon as I get seated, too.

I wish I could tell you. I am a bleeder and have had cavities filled without incident but with me, they have to give me far more numbing injections to numb things than is typical and I still feel the pain (not just pressure, but pain). When having 4 small (adult) teeth pulled in preparing for orthodontics, no joke--dentist had to give me 19 shots and I still felt it--I was exhausted, tachy, hot, cold--I finally told him to just pull it (the last one) and if it's too bad I'll signal you. All 4 bled all day and night despite continued application of pressure (I'm a bleeder) and wound up at his office late that night to have all 4 packed. He was the one who urged me to get blood checked out with hematologist (despite all my other histories of bleed in the past). That was 12 years ago and I am very grateful to have had him pick up on this problem and give me direction. My grandson's and nephew's dentist always told parents to give dose of acetaminophen before going.

Although I have OH, I do still occasionally get POTSY only not near as rapid in the heart rate as formerly. I supposed that was why now that when BP drops and heart rate hangs in low 60s that I was told the ANS is failing.

Add me to the list of vocal cord disfunction (diagnosed yesterday by pulmonology loop functions studies). I also have EDS which is double whammie. Constant nasal drip and GERD not controlled well on meds/diet are factors that will be constant irritant. I've just been living with this since spring of 2007, so I don't think it's anxiety induced (it's everyday). My neuro exam in Apr. 2011 I told I had difficulty swallowing but it was dismissed or overlooked. I'd mentioned difficulty swallowing/sometimes (especially in bed) when I have to sit full up and repeatedly try to swallow as it feels like I'm choking. I had attributed the loss of control over the projection of my voice or the pitch or tone as consequences of repeated sinus surgeries. Just what I want--another specialist added to the list--even further away from the specialist I saw yesterday--3 of them about 2 hours away if good traffic and no bad roads/accidents. Another 2 a little over an hour away. If I have to have speech therapy and I imagine I will, that will be another hour ride to as there is nobody close to where I live. Oh, and I called my pulmonologist (almost hour's drive) with this added dx and asked if he treats this. I was told 'no' (but I have asthma/COPD and if in some kind of respiratory crisis that could be from this I would want to be where a pulmonologist knew me--closer than 2 hour ride to University pulm./allergist/immunologist hospital). I hope my PCP can make transfer arrangements if something arises but I don't know how they'd do that if I ended up on ventilator out here in boonies.

One thing that my cardiac specialist explained to me with the OH is that (seeing my purple feet and vienna sausage toes/swollen legs/feet from the long ride and wait to his office) was that in some cases (like this), the leg muscles/vessels are not strong enough to pump the blood back to the chest to reoxygenate/get to brain. Having EDS makes these structures even worse. So my fluids have to be moved by use of Bumex that I take every 2 or 3 days and sitting semi-recumbent keeping the legs elevated. Hypotonia is common in arms and legs with dysautonomia, so exercising to try to keep them better toned is a must. I did 2 mos. of aquaciser treadmill and now use recumbent stationary bike (per card. suggestion) to try to help toning and moving the fluid--although I don't find it to be of much benefit--I still pool and will need to get the fluid off.

I have low gamma globulins, do make antibodies but not enough. Both infectious disease doctor and immunologist/allergist/pulmonologist told me I need to take IV Ig for the IV serier then home sub-cutaneous infusions. I have to go back to ID doc tomorrow so guess will 'get this party started'. My luck with holidays and needing ins. auth--may not be til Jan with higher deductibles, co-pays and max out of pockets. I'm leary as it's a long drive and I'm really sensitive (even to tape they'll use to hold tubing in). It doesn't take much to give me headache that may quickly/with little warning escalate to migraine. Should I take someone with me when I go? Thanks everybody. I'd be a bonus if it helped with the dysautonomia on the side (but with cooler weather I wouldn't really know as my episodes are so much more freq in summer and they've just up'd my Midodrin for a third time, which could help too).

For those of you who were watching and waiting for an update, being referred to 2 more Cancer Center specialists by the first on I saw in Feb, I just saw the 2nd. Interesting findings on pulmonary flow-loop studies show I have vocal cord dysfunction (my neurologist and PCP dismissed my complaints of difficulty swallowing, sometimes feeling like choking and not getting in good breath). I read that this is found in EDS (which I have) and also associated with Autonomic Nervous System in some way--so I am not sure but suspicion the lax structures vocal cords/laryns may be more EDS related, but some of breathing issues could with the shortness of breath with near syncopes (from dysautonomia) make things worse. I was found with yet another new allergy--latex. (YIKES--telemetry electrodes caused me rash with intense itchings, blistering and 2nd and 3rd degree burns, plus tape give me itchy rashy, blistery welts and now I read latex is also in intubation equipment. Having history of pneumonia/asthma/COPD, if I had to be on ventilator with IV tubings taped to me, heart monitor pads on, latex within me/around mouth and such from the intubation and not being able to speak--I will be miserable inside and out. I can't tell them how imperative it is to keep rotating the electrode pads and watch skin integrity and such). DH tells me not to cross that bridge til I come to it, but I tell him, he has to know as he may be my voice. I was told weight loss (down to 89.4 lbs)/nausea could be coming from food allergies or from some autoimmune dis. with my skin issues (vitilogo/rashy). So had food panel, and immunophenotyping (looks for molecules associated with lymphomas/leukemias and other things), C3, C4, more IgA, IgG, IgE, Ig subclasses, a test to pick up on autoimmune/angiodema/inflammatory issues/diseases--forget what it's called. Eight tubes of blood in all. Now doc didn't really latch on to my question about MCAS. But I don't know if any of the testing she's done may lead to that as she said, depending on results more testing/aggressive treatment might need done. She questioned why my ID doc had not put me on IV Ig. I told her we'd talked about it but I see him Thurs. and we were to discuss it again. She said it was clear that I make antibodies--just not enough and that I need 'transused with IV Ig'--however they do it--I thought she said for weeks and then eventually home subcutaneous infusions off and on. I read somebody's post here that IV Ig has helped their dysautonomia. I have to see her again in a month, an otolaryngologist even farther away in a couple of weeks and the 3rd hemeonclologist for the Delta Granule Storage Deficiency a week after I see this doctor back. So lots of long rides to the city so sorry for those of you who love the snow, but am asking all to pray for me for clear roads on my travel days.

Count me in.

My first EDS III diagnosis was made by a rheume who specialized in fibromyalgia that my orthopede referred me too. Cardiac specialist in dysautonomia concurred with the EDS III. Altho there is no genetic testing for Classic EDS I or EDS III, genetecists who are well versed in EDS are considered to provede the 'Good Housekeeping Seal of Approval' expert opinion/diagnosis. Depending on you health insurance plan, you may be able to contact genetecist office and ask for appointment (without a referral from your PCP).

That's not true. Joint hypermobility is a necessary feature for the diagnosis of classical type, whether type 1 or 2. It's true that as a hypermobile person ages they stiffen and often develop arthritis thus losing their flexibility but Classical with no history of hypermobility can't be diagnosed; it's one of the major diagnostic criteria. There are types with much less dramatic joint hypermobility like the Vascular type, where it can be limited to the small joints (like the hands). This type is rarer and comes with more severe manifestations as rubytuesday was saying. Life span is often limited because of the aneurysms that result from the weakened type III collagen. Dysautonomia is not as common in this type. Yes, you are correct sugartwin. I stand corrected. Thank you.

Anyone you ask with EDS could have very different manifestations as there are 6 categories but a continuum of connective tissue disorders, some of which include Marfans (like manifestation of congenital dilated aorta) or autoimmune. As a lot of us age, our once very hypermobile joints become racked with pain and very restricted in mobility. Not everyone with EDS is hypermobile. However hypermobility EDS III can also be exhibited in EDS I (classic). But not all EDS I (classics) are hypermobile. There are currently no genetic tests explicit for EDS I and EDS III. EDS IV affects the vasculature structures much moreso than the other EDS classes altho EDS affects veinous/arterial structures. My cardiac specialist in dysautonomia told me when I questioned if I could have EDS IV and III (and I) because I am a hemorrhager, he said that I could not have the EDS IV or would have been dead before mid-50s. My hemorrhaging it was found stems from Delta Granule Storage Pool Deficiency. The hemeoncologist I was referred to for that had lot of questions about my parents/immune deficiencies before he referred me on to 2 more of his collegues. So I am wondering if at least with the immune component is genetic as well (many EDSers have problems with immunodeficiencies but that is not in and of itself exclusive to EDS. One can be hypermobile without having EDS (i.e. an acrobat or a dancer or a gymnist--someone who conditions and exercises to stretch those musles to control those musles, to get the long lines and the funkey or beautiful moves)

It would show impeded flow in arteries with things such as aneyrysms, arteriovenous malformation, blockages that interfere with blood flow in the arteries/vessels, or search for a source of bleed. It evaluated the carotid arteries, the basilar artery (two arteries that arise from the chest into the vertebrae) and the circle of Willis (group of vessels at base of skull that feed the brain)' A CSF flow study is an MRI of brain or spine that evaluates the cerebral spinal fluid flow around the brain, brainstem and/or spinal cord (depending on which type is ordered). I hope that clarifies a little better?

Naomi, an MRA looks at the arterial structures in the brain that the MRI does not.

I believe that it would be a pertinent finding on seated/standing MRI that would then lend medical necessity justify the need with health insurance carriers to authorize the brain flow studies. I think it is a process of one finding at a time.

It not only affects joints. Connective tissue holds all things within the body together and even affects the veins. In some, not only veins but the vascular system (in those the life span is less than my age I was told). It also affects the GI, manifesting in IBS, delayed gastric emptying, gastritis,GERD, hernias, adenomas, subluxations or dislocations of joints, tendons and ligaments. Some exhibit widen scars and surgical wounds that dehicse (sp)--split open. Wounds are often slow to heal. There is a common thread of immune compromisation in many--especially sinus. On one continuum of connective tissue disorder is Marfans with dilated aortas (congenital) and such. Skin is often very stretchy and has velvety feel/transluscent in places where veins are very visible. In hypermobility, the joints that were once able to move beyond normal limits (like 90 degrees), as one ages they become very stiff and less mobile, exhibiting more pain and complications. Another not so uncommon feature found in EDSers is Chiari I malformation. The glue that is holding everything in the body (even the eyes) is very lax and the body fights to keep itself held together (to put in simple terms). It's a genetic defect in the connective tissue. that provides structural support to most of the body including muscles, joints and ligaments. Collagen is the proteini that acts as the glue giving the connective tissue strength and elasticity. The fragile skin and faulty joints found in EDS results from faulty collagen.EDS is a varied group of heritable connective tissue disorders that are characterized by articular hypermobility (joints that move or bend more than normal), skin softness and stretchiness, and tissue fragility.There are at least six major types of EDS which are classified by their manifestation of distinct signs and symptoms. Each EDS type is thought to involve a unique defect in connective tissue, although not all of the genes responsible for causing EDS have been identified.EDS affects men and women of all racial and ethnic backgrounds. To learn more about EDS, please visit our Website at www.ednf.org. The later was taken from support group forum to which I belong.

What I do know about them having EDS and Chiari I, POTS/OH/dysautonomia is that with chiari I (cerebellar tonsils exit the base of brain into spinal column), a supine MRI shows yes there is a chiari I and it's measurements that are useless. Seated or standing MRI are more useful as it shows the pull of gravity on the Chiari I (since we spend most of our time in upright position). This could impede blood/spinal fluid flow in/around the brain. Dynamic Imaging has website that explains. Chiari Institute also has website videos you can watch. I would presume that whatever it is your doctor is looking at would be the same theory.

When I called my cardiac specialist in dysautonomia to let her know BP was still plunging (Mestinon and Midodrine) but now Mestinon was making me so sick at my stomach, she blurted out 'well some people's BP we just are not able to get up' (goal was to make me hypertensive then work on hypertension). I was confused as the doctor had told me there were lots of combos of meds I may have to try to find a fit. Three weeks later (and about 10 lbs lighter--me), Dr's office called to tell me to stop the Mestinon, let it get out of my system, then they would introduce a different med. I figure this may be meaning at my next visit whenever that may be.

My PCP (IM) will never order MRI on anything suspect of orthopedic or neurologic problem--rather she makes referral or if I already have ortho/neuro tells me to ask them. If she did test, test was abnormal--what would she do with it but send you to ortho or neuro, thus she views it as beyond the scope of her practice.

i hate bananas, The genetecist told me (there is no genetic test for Classic or EDS III) that he would treat me the same as any other EDS III patient and to keep doing what I am doing. He was certain I had it but didn't want to add himself as yet another member of the list of specialist and PCP (IM) who comprise my health care team.

I was wondering about this too, having gastric motility issues and GERD despite Rx meds, but with the state of my autonomic nervous system and EDS I am pooling fluid/blood in feet and legs and have to take Rx water pill to get the fluid off. My legs already feel like daggers are piercing me all through the legs when I get up in the morning--would raising the bed make this worse in situations like mine? Thanks for response and thanks for posting.

My DF and I were always 'blessed' with running BP on low side (I think one of my sister's does as well). My DM and sister (g-parents), son are hypertensive. DF passed with cancer in his early 60's but DM has been passing out for years. (She describes same sx's as me but she attributes it to her 'since my chemo and radiation' that ended in 2001, or maybe because of her BP med). I can't get her to check her VS (even getting her equip when she has 'episodes' to talk to her docs about it. Once with episode the found Pulmonary emboli, so she thought it was from that but she is in late 70's and still gets occassional spells--especially in warm weather when she's outdoors. I think b/c of her age and hx of hypertension and heart disease, they don't really look into things like POTS with her. I would not be surprised if she had it though. I am older of my sibblings by nearly a decade and I wasn't symptomatic with faints until my 50s, but so far, none of them have exhibitted it--altho some of them have similar health issues as me.

In my case my cardiac specialist in dysautonomia told me that had he seen me a year ago (having all the grafts of my BPs and HRs) he would have concurred that I had POTS. More recent graphs from summer and test at his office that day gave dx of Orthostatic Hypotension (under umbrella of OI). My BP typically runs on low side but if standing, bending, stooping, reaching, lifting, heat, etc. may cause me to faint--sometimes with, sometimes without warning. Specialist explained to me that my autonomic nervous system was failing and I would have fewer warning signs so his aim is to get me hypertensive, then work on the hypertension. Salt is a restriction for me as I am now retaining blood/fluid that pools in my legs (guess that goes with the dysautonomia). With my OH episodes, my BP bottoms out still but the heart rate now ususally hangs in low 60s when that happens. Specialist told me I'd probably need pacer down the road. I still will occassionally have a swing where I have the BP plunge with the tachycardia, but the tachycardia is not nearly as fast as it was when I was in POTS state. So I have both, only moreso the OH at this point.

I'm sorry you're having such trouble with side effects. I was recently taken off my Mestinon due to side effects. I, too have the boatload of drug allergies/sensitivities. I take Allegra (doesn't seem to do anything for me--nor does Claritan), Zantac was added at bedtime (couldn't do twice/day due to too many meds filtered thru liver) and have been on Singulair for years (for asthma/allergies) taken at bedtime. I've been lucky not to have side effects from that. Did you have tryptase/ and the urine test? Did they do bone marrow to do mast cell count in bone marrow? Just curious as to how yours was diagnosed. I was referred to 2 hemeoncologist by another oncologist (one appointment in a couple of weeks) for multicomplex autoimmune deficiencies. I was wondering (since this hemeoncologist has research interest in Mast Cell activation but is 64 years old) if she will consider this or not. I also take Prilosec OTC but had to cut that down to 1 day again because of all the meds I take that are hard on liver. Wishing you success with your new regimen. Try to hang in there. If it's anything like the orthostatic hypotension, there are most likely other agents they could try.

I sure understand your plight. I went thru to long awaited to somewhat infamous genetecist in our area for official confirmation of my EDS III yesterday (EDS II diagnosed in 2005 and again in 2011 by cardiac specialist well versed in EDS III, attributing some of the blood/fuild pooling in feet and legs from the weakened muscle/venous structures from EDS). I was told genetecist would spend about 2 hours with me, his staff told me he'd examine my scars, measure wing span, etc. He did none of those things--just looked at hands riddled with arthritis (said pain was EDS rather than fibro), fingers were positive as were thumbs on maneuvers. He had me bend over to touch my toes (when I'd told him my pain was high in the hip where glutteal muscles/tendon were ripped off the hip bone 7 years ago and I have the fainting--with fewer warnings and bending at waist is a no-no--he said he knows my cardiac specialist). Still I did it--thankfully not passing out but having to steady myself w/ exam table beside me. He told me it's very difficult to dx someone my age (mid-50s) with arthritis/autonomic nervous system failing as some of the things could stem from that. He said it was most likely yes, I do have EDS III but without more big joint dislocations he couldn't give official diagnosis--then turned around and told me to have all g-kids, kids, sibblings checked whiile young. (What doc is going to 'screen' those test when some close relative has a very probable diagnosis--no ins. would auth some of those screenings without definitive). DS's dilated aorta and GFs passing from ruptured abd aorta was concerning link of ?Marfans. All the wounds dehiscing with surgeries, deep, elongated tears with childbirth and with bowel movements, etc. could or could not be?). Most likely is but can't diagnose definitively. I was told he would need to measure my wingspan and examine transluscent skin and skin that was stark white in large areas, cuts and bruises and rashes. He never had me disrobe. My weight had dropped to 91 lbs fully dressed and without my water pill. Gastric motility issues could or could not be EDS compnent or other autonomic nervous system failure related. He felt autoimmune dis. involved--despite extensive testing repeatedly being negative and he offered no suggestions. He told me with dx of EDS III, he instructs his patients to do what I'm already doing so no need to add another doc and dx into the mix??? I was flabbergasted to say the least. My appt. was at 11:30, staff started with me early, by time they had all their entries in computer and genetecist came in to see me, it was after noon. He said as he walked in the door, without laying a hand on me that 'you're probably not going to like what I have to say here but you fit into EDS but at your age, with the arthritis and ANS failing, it's just too hard to give a definitive diagnosis. I was home before 1:30 and it takes over an hour to get home. Honestly I think I was cutting into his lunch time. So I have to treat myself as EDS III but without his official diagnosis. I was thinking about highlighting exerpts from his book that were applicable to me and send his book back to him along with a letter suggesting he might benefit from a refresher course, unless things have changed since his publication. I was excited to go but didn't have high hopes as I've been referred from specialist to specialist to specialist to specialist--all who think it's nothing in their area, but something going on, or part in their arena, or need yet more opinions. I am so done. I wish you the best on your visit. I had DH with me but he sat quietly as he was just in a hurry to get back to work. He said, well you came for an opinion and you got one. I said, yeah, that it most likely is EDS III but can't give definitive so what does weight does that hold among the medical community who see me. The genetecist told me to keep warning surgeons/dentists/orthodontists of wounds dehiscing, and anesthetists of problems w/ anesthesia and good rheume (been to 3--closest one, longest one retired, 2nd one near retirement, and third I'd seen charges you a fee just to be her patient). No thank you anymore. I can't take NSAIDs and short term prednisone helped the crippling feet, ankle, hand pains. We live in boonies and I have alot of specialists I have to see with regularity and don't need more drives farther away. I just give up.