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Ehlers-Danlos anybody? (Hypermobility)

Discussion in 'Skeleton, Skin, Muscles, Hair, Teeth, and Nails' started by Sushi, Jul 28, 2009.

  1. Sushi

    Sushi Moderator and Senior Member Albuquerque

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    This is one that keeps popping up, particularly for the dysautonomia crowd. Sorry, one more syndrome! As I understand it (I have it) it is a genetic collagen disorder, usually making tissues with collagen more stretchy. Many of the dsyautonomia crowd have mitral valve (& other valve) prolapses. This could well be from EDs (as it is affectionately known). There is usually a body type that goes with it--long limbs, slim (at least before ME/CFS), arm-span greater than height, and some other proportion things. Online resources give all the specifics.

    One of the possible implications is OI. Stretchy veins, vein valves that prolapse = blood pooling when standing. Other symptoms are lots of subluxations, joints that are easily dislocated, ligaments that stretch and don't hold things in place, and an unusual degree of flexibility. The diagnosis is almost entirely done by checking a few body things having to do with flexibility, looking at body proportions, and history. Things like stretch marks at age 12 are just gravy. Boy did my GP laugh at that one! And, I am sooo good at yoga!

    There are subtypes of EDs, but the one usually found in this population is called EDs III or simply hypermobility syndrome. Has anyone else explored this? I use exercise (pilates) to strengthen core muscles to compensate for ligaments being stretched, but I'd be interested if anyone has found supplements or other routes to enhance collagen.

    Sushi
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  2. Jody

    Jody Senior Member

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    Sushi,

    I haven't heard of this before, I don't think.

    Not sure I grasp what you're describing. Does it also involve being double-jointed or that kind of thing?

    Also, am wondering if this can affect the hands and fingers? Wondering because I have a new (hooray!) weirdness in my finger joints recently.
  3. Cort

    Cort Phoenix Rising Founder

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    I came across it researching orthostatic intolerance. The symptom set between it and ME/CFS has some real similarities and some people with CFS have Ehler-Danlos either instead of or in addition to their CFS.

    Somebody, ironically, just emailed me about his relief about finally finding out that he had ED.

    Orthostatic intolerance is itself a pretty new field. He did recommend this website.
    Wish I had some suggestions - maybe someone else does; in any case it's good letting people know about it.
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  4. Frank

    Frank Senior Member

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    I have this. I don't know if i have dysautonomia, maybe i should see a heart specialist.

    Anyway you can test hypermobility like this:
    [​IMG]
  5. Sushi

    Sushi Moderator and Senior Member Albuquerque

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    One of the best known experts in this field--Dr. Rodney Grahame-- writes:

    Hypermobility

    Systemic disorders with rheumatic manifestations
    Current Opinion in Rheumatology. 20(1):106-110, January 2008.
    Grahame, Rodney; Hakim, Alan J

    Abstract:

    Purpose of review: Joint hypermobility is widely prevalent in all communities yet its clinical effects are poorly understood and often overlooked by rheumatologists worldwide. They may observe the hypermobility but fail to appreciate its significance in terms of overall morbidity and, more specifically, its strong link with chronic pain, fatigue, dysautonomia and the adverse impact on quality of life.

    Recent findings: This last year's publications shed further light on this fascinating and, as yet, largely unexplored terrain within rheumatology. Perhaps the most compelling new knowledge is the finding that hypermobility, if sought, is the most common finding amongst patients presenting to a rheumatologist; more often than not, it is being overlooked. There is an urgent need for rheumatologists to accept the challenges posed by hypermobility-related disorders, which have, in the past, fallen by default to clinical geneticists untrained in rheumatology.


    A correlation between hypermobility and osteoporosis has also been reported.

    The Beighton score has been the traditional way to diagnose hypermobility. It was updated to be more diagnostically useful and this update is called the Brighton Criteria: Here it is (sorry for medical language):

    REVISED DIAGNOSTIC CRITERIA FOR THE BENIGN JOINT HYPERMOBILITY SYNDROME (BJHS)

    Major Criteria

    • A Beighton score of 4 - 9 or greater (either currently or historically) (see below for Beighton scoring)
    • Arthralgia (joint pain) for longer than 3 months in 4 or more joints

    Minor Criteria

    • A Beighton score of 1, 2 or 3-9 (0, 1, 2 or 3 if aged 50+)
    • Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.
    • Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
    • Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).
    • Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactily [positive Steinberg/wrist signs].
    • Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.
    • Eye signs: drooping eyelids or myopia or antimongoloid slant.
    Varicose veins or hernia or uterine/rectal prolapse

    Beighton Scoring:

    1. left little (fifth) finger: passive dorsiflexion beyond 90--1 point

    2. right little (fifth) finger: passive dorsiflexion beyond 90--1 point

    3. left thumb: passive dorsiflexion to the flexor aspect of the forearm--1 point

    4. passive dorsiflexion to the flexor aspect of the forearm--1 point

    5. left elbow: hyperextends beyonds 10--1 point

    6. right elbow: hyperextends beyonds 10--1 point

    7. left knee: hyperextends beyonds 10--1 point

    8. right knee: hyperextends beyonds 10--1 point

    9. forward flexion of trunk with knees full extended
    palms and hands can rest flat on the floor--1 point
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  6. Jody

    Jody Senior Member

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    Thanks,

    This information tells me I don't have it.
  7. Sushi

    Sushi Moderator and Senior Member Albuquerque

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    Hi s5044726,

    About seeing a cardiologist... I have seen many and had lots of echocardiograms (which is how they find prolapsed valves). They always find them and keep telling me that I need to repeat the echo every couple of years to see if anything has changed. Well, it never does! They find 3 mildly prolapsed valves each with a tiny bit of back flow.

    Maybe if someone suspects that you have majorly prolapsed valves with significant backflow (regurgition) it might be good to keep checking on it. (A good doc can hear a sort of click that is indicative of a mitral valve prolapse.) But the minor prolapses have no clinical treatment and, apparently, unless you are in the subset of MVPers who have dysautonomia, there are no symptoms and no one is worried about it. About 20% (I think) of the population has one or more mildly prolapsed valves.

    They used to tell us to have prophylactic antibiotics before dental work, but that changed about a year ago and they recognized that the negatives of giving antibiotics routinely outweighed the positives (prevention of heart valve infections.)

    For me, the significance of discovering MVP was that it led me to research it and discover dysautonomia and finally the crossover between dysautonomia and ME/CFS--which has in turn led me to some treatment options.

    Sushi[/QUOTE]
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  8. Frank

    Frank Senior Member

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    Thankyou very much sushi. Did your CFS start with fever or do you have feverish symptoms, because i don't and it's my greatest difference with al lot of patients i think.
  9. Sushi

    Sushi Moderator and Senior Member Albuquerque

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    No, I am also atypical and for that reason it wasn't clear to me or docs that I fit the ME/CFS diagnosis, as the US one excludes me. However, I fit nicely in the Canadian one.

    For many years I knew I had dysautonomia because after reading a description in "Confronting Mitral Valve Prolapse," it was eureka, that is me! I didn't get treatment from docs for dysautonomia because at that time all they were giving was beta blockers and I didn't want to take them. But the book mentioned above had great lifestyle adjustment suggestions and this is what kept me functional (sort of) for years.

    I had had periodic collapse periods when I couldn't even walk across a room, but these had never lasted more than a month or two until 2006, when a series of major stressors knocked me into the non-functional range and I have been slowly climbing out of that ever since.

    I haven't written "my story" because it is not very dramatic or classifiable. I have had weird symptoms since childhood (like the EDs stuff) that are most likely genetic, but I always limped along at about 60 - 70% except for the crashes. I also have very high Epstein Barr titers, for what it is worth, but never knew when I had mono. I also have C. Pneumonica and a couple of other viruses, have traveled extensively and lived in 3rd world countries--which means a lot of vaccinations and parasites. I got rid of the parasites but not the vaccinations!

    I totally flunked the Tilt Table Test ending up with a BP of 88 systolic and 80 diastolic--that is when I screamed, "Get me down!" When I tell other docs those readings they say, "That is just not possible!" Ha! Lots of other autonomic screw-ups showed on the TTT. I had plenty of gut bugs, but treated them herbally (with expert help) and that area seems quiet now.

    When I took the Vitamin Diagnostics Methylation Panel I was very out-of-range for 12 of the 13 analytes tested. This showed me something that I could actually work on, and that is what I have been doing lately and it has helped.

    So I guess I "told my story" but in the wrong area of the forum! But atypical is just hard to put anywhere.

    Sushi
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  10. Cort

    Cort Phoenix Rising Founder

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    Most Common Finding!

    What an amazing story Sushi. You have ED but you also have some very typical ME/CFS readings. That response - this isn't possible - is something you hear fairly frequently with ME/CFS patients. We're such a strange group! I wonder how many different types of 'ME/CFS' there are. There's a reason its referred to as a 'Wastebasket Diagnosis' after all. I wonder where, if any place, all these different but similar syndromes meet?

    That one statement

    just blew my mind. It's amazing what doctors miss. This is like observational stuff - are you very loose limbed? Do you have longish fingers? Mitral valve problems, joint pain, etc. etc. Don't you think they would start to get it after awhile? It may be that doctors have become really lousy observers and really good test interpreters.

    Do they know if this is genetic? Or does the body simply take a wrong turn at some point?
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  11. Sushi

    Sushi Moderator and Senior Member Albuquerque

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    Cort,

    Anecdotal but telling: Dr. Blair Grubb, who is one of the most respected clinicians in the Dysautonomia world, can watch a new patient walk in the door and recognize: "EDs primary, dysautonomia secondary."

    He has seen so many that the body geometry, skin characteristics, movement (loose limbed), instantly clue him in without doing the actual physical checks. (though he also does them) So, not rocket science!

    I once opened an informal poll on a Dysautonomia site asking people to measure the ratio of "wing-span" to height. Again telling. A lot of that OI bunch had fingertip to fingertip reaches of several inches more than their height. (stretch out against a wall and have someone measure you--or figure out how to measure yourself) The gorilla gene maybe?;)

    And yes, it is thought to be genetic. They have identified some of the genes but it doesn't seem to be the whole story yet. Also, the things that the EDs III--hypermobile people do best (like yoga) makes the situation worse because you stretch those ligaments and joints even more. This is why I have switched from yoga to pilates as pilates will strengthen the muscles that keep things in place. I have had prolotherapy to tighten the ligaments supporting my knees--helped a lot (hurt a lot too!).

    So public awareness would help little EDsies from getting worse instead of doing acrobatic party tricks to entertain their friends and then having to present themselves to a rheumatologist (who will misdiagnose them) a few decades later.

    Sushi
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  12. Cort

    Cort Phoenix Rising Founder

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    I'm really tall (6'6") and slim but I didn't meet the standard indices of ED. I wonder, though if there are varying degrees - just like ME/CFS. Maybe I have a tendency to it.

    I wonder if that great swimmer has it a bit - super long wingspan, super flexible - super strong too as well that's for sure. He's healthy as can be. Maybe swimming, though, with its non-impactful nature is a good exercise for ED.

    Ever tried Prolotherapy? Just put a post on it.

    http://forums.aboutmecfs.org/showthread.php?t=230
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  13. Sushi

    Sushi Moderator and Senior Member Albuquerque

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    Yes, Cort,

    There are degrees of the different forms of EDS. Most people seem to have just a few of the diagnostic criteria, but may be affected in ways like OI without having all the other things going. I think mine is sort of in the medium range. i have a friend who has not only heart valve prolapses but organ prolapses that may require surgery.

    Also the EDs III or hypermobility is the mildest of the EDs spectrum. One (the vascular form) is life-threatening and usually fatal as blood vessels and organs actually degrade.

    Still, for me, it is an alert area to realize that the OI (life-long but mostly liveable with until recently) is likely to have other causes than the usual and that I may have to train muscles to "take up the slack" for veins that don't do their contraction bit effectively.

    Sushi
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  14. Michelle

    Michelle Decennial ME/CFS patient

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    If I had a dollar for every time a doctor has said "I've never had that happen!" to me after whatever he or she did landed me in the ER, I'd have a fair amount of spending cash at this point.

    When I first heard Peter Rowe talk about hypermobility among his patients awhile back and several ME/CFS patients say they had EDS, I looked EDS up and went OMG, that's me! I haven't been able to throw a ball overhand since jr. high because my shoulders pop out of joint and pop back in again (causing inflammation that takes forever to die down). I've always been not just very flexible, but my joints pop out of place frequently (subluxate). The first thing a physical therapist notes is how hypermobile I am. The first thing the nurses in the ER note is how fragile my skin is. I've had surgery on two joints (right ankle and knee) to help stabilize them, though both are still more unstable than they should be. And now my sacroiliac joint is slipping and sliding around like crazy, in addition to my shoulders, ankles, fingers, hips, and even my elbows to some extent.

    I haven't yet approached my doctor because I've been nervous to say "hey I've been reading the internet and I think I've got this rare genetic disorder!" But he's been running out of options to treat my pain and even he's starting to test me for weird stuff (Reiter's Syndrome??). So between that and my SI joint problems, I think it's time. I even had someone approach me last month after reading a blog post of mine about whether I have EDS.

    The only two things that make me question the diagnosis is that one, I've had problems with clotting and people with EDS tend to have the opposite problem. The first time I was on Coumadin I did have problems with bleeding, but I was also post-operative. I'm also the exact opposite body type of the typical EDSer -- I'm short and fat with tiny stubby fingers.

    But then, I tend to present abnormally with just about anything. I presented with a stomach ache when I had blood clots in my lungs and pain traveling down both legs with a urinary tract infection. My nurse practitioner always used to say, "Michelle, if you ever present normally I'll be blown away!" ;-)

    Oh, I should note that while my physical therapist wasn't familiar with EDS, she was the one who recommended prolotherapy to me. Granted, I've got to figure out how to pay for it given that Medicaid doesn't cover it...
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  15. Jody

    Jody Senior Member

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    Well Michelle,

    Don't you feel special. :D

    You are truly one of a kind.
  16. Michelle

    Michelle Decennial ME/CFS patient

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    LOL yeah my nurse practitioner -- at least in the beginning -- would always say when I would complain at how weird my body was, that I was "just really special." But towards the end even she switched to "weird."
  17. Esther12

    Esther12 Senior Member

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    I've got loads of the features of marfan or things associated with ED... but not the hypermobility.

    I think a lot of CFS is related to these sorts of issues. In 50 years time we'll know, but right now it's all pretty confused.
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  18. Uno

    Uno Senior Member

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    There are three or four forms of Ehlers Danlos, I have Type III and have a delightful tear in my gut from the lax tissues plus busted ligaments in my ankle and permanant damage to my neck pelvis and shoulders. I am looking into special supplements that have collegen in to see if it helps. If you are in the UK I'd see Prof Graham, he is excellent. x
  19. Esther12

    Esther12 Senior Member

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    Sorry to hear about your troubles Uno. Does Graham think your fatigue is a seperate CFS issue, or a manifestation of EDIII stuff? To me, it doesn't sound that surprising that you're suffering from fatigue too!
  20. SaraM

    SaraM Senior Member

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    Prolotherapy and Hyaroulonic Acid help some people.

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