Review: 'Through the Shadowlands’ describes Julie Rehmeyer's ME/CFS Odyssey
I should note at the outset that this review is based on an audio version of the galleys and the epilogue from the finished work. Julie Rehmeyer sent me the final version as a PDF, but for some reason my text to voice software (Kurzweil) had issues with it. I understand that it is...
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Ehlers Danlos Type 3 Hypermobility - Do you have it?

Discussion in 'Connective Tissue Disorders/Ehlers-Danlos Syndrome' started by Eeyore, May 9, 2015.

  1. Eeyore

    Eeyore Senior Member

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    I think that for people with mild hypermobility, it can probably be ignored. I think it is more interesting that there is a connection to hypermobility, as it is not immediately obvious why there should be in ME. It is a clue to pathophysiology.

    My understanding is that in severe cases of hypermobility, it is not a benign disease and needs treatment. Type IV (vascular) EDS, for example, is often fatal due to rupture of arteries or other internal organs (e.g. colon) Some things can be done to monitor for these problems and possibly treat them, extending life or improving quality of life. Even people with more severe classical EDS can use various types of braces to prevent excessive range of motion. There is no cure - but learning how to manage the disease can reduce the incidence of side effects - the main one being arthritis. Vascular EDS is quite rare, and I'm not sure if it's at all associated with ME. If we are talking about type 3 hypermobility, there is possibly an increased risk of valvular disease, which should be monitored probably (although again, as Dr. Edwards mentions, there really aren't any good studies to measure outcomes).

    So I think it depends how severe it is. For most ME patients with hypermobility, it might make sense to learn what not to do, and to make use of braces and such, so as to prevent the development of osteoarthritis, at least until such time as we know a lot more about these diseases.

    Hypermobility is a spectrum. My dad has some - and he has had a lot of ortho and joint issues in his life. Tendon rupture has been a major theme. However, he's in his late 60's, working full time in his chosen career (not physical labor), and able to do what he likes (including exercise and physically demanding hobbies / sports) - so in his case, it hasn't been a major problem, more of a nuisance. On the other end of the spectrum you see 25 year olds who have had multiple surgeries, are in constant pain, and have bad osteoarthritis. The latter is rare though - most people are more in the former category like my father, who still has no major health issues.

    Dr. Edwards also mentions that he didn't see many hypermobile patients as a rheum - I think this may also have to do with some referral bias. My dad has seen MANY orthopedists and had probably 10-15 orthopedic surgeries in his life, many steroid injections, etc. He has separate, specialized docs for his back, feet, hands, and general ortho (knees, shoulders, other). When you present with these problems, your GP will treat or refer to an orthopedist. You'll see a rheum (at least in the US) only if they suspect autoimmune disease. There may be some exceptions to that, but really rheums in the US seem to be entirely focused on autoimmunity. Non-inflammatory joint disease is treated by GP's and orthopedists, who do likely see a fair amount of hypermobile patients - most of whom are treated with conventional treatments - they just require more of them and more often.
     
  2. Sushi

    Sushi Senior Member Albuquerque

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    I agree that there isn't much that we can do about it, but I learned too late that taking certain precautions would help prevent pain and injury. I would not engage in "bendy" games any more and I am very careful about ergonomics as it takes so little for a joint to get out of line and lead to pain. For instance, I no longer pick up a heavy object like a suitcase with one hand as I will suffer the consequences immediately.
    Agreed. When I used to cycle I needed to wear knee braces to keep my knee-part in place. And I do wear a back brace on occasion for extra support.
     
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  3. Hutan

    Hutan Senior Member

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    Exactly.

    Dr Chris O'Callaghan seems to think that there is a strong connection between orthostatic issues and hypermobility. And he has also noticed that many of the patients he is seeing for orthostatic issues are not only hypermobile but have MECFS and migraines.

    My small sample (my son and I) fits this - we have ME, orthostatic issues, hypermobility and migraines. Others here also seem to tick all the boxes.

    I don't know if Dr O'Callaghan has published on this. I haven't met him yet, only heard him speak. But it seems interesting and, as Eeyore says, exploring the theory might shed light on the cause of ME.
     
  4. Eeyore

    Eeyore Senior Member

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    I know of one other person who has been diagnosed with type 3 EDS - but she isn't really hypermobile either (in terms of range of motion) - but has all of the problems associated with EDS (heart valves, frequent dislocations/subluxations, joint pains, early arthritis, joint laxity, etc.) So what is hypermobility really? How are we defining it, and which is the kind of hypermobility that is associated with ME and with POTS/OI? How does this overlap with EDS? Which is the cause and which is the effect?

    I asked in another thread if the hypermobile people were hypermobile before ME onset, and I think all of them said they were hypermobile as far back as they could remember. This suggests that the hypermobility predates the ME. So we are left with several possibilities:

    1) Hypermobility somehow causes ME/POTS/OI.
    2) Hypermobility increases the odds of developing ME/POTS/OI when combined with other genetic and/or environmental factors.
    3) Both hypermobility and ME/POTS/OI share a common cause or risk factor.

    It seems unlikely that ME causes hypermobility. I believe also that Dr. Edwards said he found this notion implausible based on what we know of hypermobility, and this seems to be the prevailing view.

    It is interesting to me that I have multiple hypermobile family members, but they don't have ME. I have a mother with POTS and ME (largely recovered with some residual orthostatic defects) - but she's not hypermobile! I've had increasing subluxations in the last few years though, which is what has caused me to wonder if I've developed something that looks like hypermobility. I have also heard though that men with hypermobility tend not to notice until much later in life, if at all, as increased muscle mass compensates for joint problems - one reason why it is postulated that women have more problems with hypermobility syndromes and EDS. However, it may not be the real reason that there is a gender difference - it might be more closely related to the reasons that ME has a greater prevalence in women than in men, which might have more to do with the differences in immunity and physiological stress response.
     
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  5. Hell...Hath...No...Fury..

    Hell...Hath...No...Fury.. Senior Member

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    I've had this personal opinion for years. I have all of those things, hypermobility undiagnosed as there's no-one to do it where i live. I was also told that there was no point having it diagnosed as it can't be treated anyway :bang-head: And so i hit a brick wall. I used to score 9/9 on the beighton but now only 7/9 as my little fingers have stiffened up over the last few years.

    But been hypermobile my whole life, then migraines from age 4, which started in bowel, moving to stomache, then to right eye, before settling on side of head by age 7.

    Then ME, then POTS. I think the last two in that order but hard to say now.

    I've mentioned in the past to doctors if there's any possible link between all 4 things after reading about it online but i either just got smirked at or was told a point blank 'no', so i just quietly ponder it now.

    I've not heard of Dr. Chris o Callaghan, sounds interesting :)
     
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  6. Michelle

    Michelle Decennial ME/CFS patient

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    I'm another one of those EDS3ers (diagnosed at age 40; had to go to the Children's Hospital to see the medical geneticist and was by far the oldest patient there that day) who clearly had lifelong hypermobility (9/9 Beighton) I spent much of ages 10-13 on crutches from injuries particularly to my left ankle. Had multiple fractures (especially left arm). Have stretchy skin in some areas and it's somewhat fragile but no problems holding stitches or anything. One of my first words was "constipation" and my mother still bitches about sweaters I ruined by throwing up on them. ;-) I do have a very hypermobile niece (also 9/9 Beighton) but she was a high school cheerleader with ample opportunity for injury and has never had any. No other family members that I know of have hypermobility or EDS. I did not develop ME/SEID until I was 26 after surgery/GI virus (though may have developed a mild form or simply FMS as early as 11).

    Once I was diagnosed, I was shocked at the dearth of research in EDS. It makes the ME/SEID field look well-researched. Indeed the literature in the field feels very 19th-century at times. My insurance doesn't pay for genetic testing so I don't know if I have The Gene. But have wondered about EDS3 as a diagnostic entity.
     
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  7. NilaJones

    NilaJones Senior Member

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    Folks here might like to look into the research of Dr Nussbaum, the head of cardiac genetics (or something like that) at UCSF.

    I have not read his papers, but had an interesting conversation with him at his office.

    He thinks there is a well-defined subset of EDS-3, which he calls EDS-3+. It is basically EDS-3 plus ME/CFS, although he does not use that term. It includes fatigue, gastro issues, POTS I think, etc.

    He also said that EDS-3 people tend to have decreased sensitivity to pain, and problems with proprioception (traced to a gene).

    If you go on the EDS-3 patient forums, people talk a LOT about fatigue and pain. Those were not originally part of the medical description of the syndrome, but they are a huge part of people's IRL experience, especially as they get older.

    @Jonathan Edwards , I wonder if the study you were involved with in the 70s suffered from the same phenomenon that so many ME/CFS studies do --- only people well enough to leave the house were in the study. People with severe pain or fatigue may also have been screened out, as having (presumed) additional health issues that would cloud the results.

    As for rheumatologists, the EDS patient forums warn strongly against seeing one if you have EDS, at least in the US. They say that rheums tend to disbelieve reports of pain and fatigue -- something in their training, I guess -- and that one is better off with a geneticist. So they are staying away in droves on purpose.
     
  8. Hutan

    Hutan Senior Member

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    The link to Dr Chris O'Callaghan's presentation is above in the thread. It's an hour or so and slow to get going. But yes, interesting, I think.

    He suggests that hypermobility results in flexible blood vessels. Flexible blood vessels result in OI issues. And this produces MECFS symptoms.

    It has to be more complicated than that though. I have always been mildly hypermobile and, when healthy, had slightly low blood pressure and a tendency to get dizzy on standing rather more than the average person, and occasional migraines. But something has clearly happened after ME onset. The OI issues are much more severe, as is the frequency of migraines.

    I think it is interesting that for me and my two children and others here too, we noticed the OI issues some months after ME onset - not immediately.

    So, I don't know. Perhaps hypermobility is a predisposing factor for OI and perhaps ME. Or it makes symptoms of OI and ME worse?
     
  9. Michelle

    Michelle Decennial ME/CFS patient

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    My personal experience would concur with comments above about EDS/hypermobile patients being seen more by orthopedists as opposed to rheumatologists. I've see many orthopedists over the years (unfortunately) but didn't see a rheumatologist until I was 29 and my PCP (GP) suspected Fibromyalgia.

    As his name was mentioned earlier in relation to Orthostatic Intolerance, I would note that Dr. Peter Rowe has his own theory about the connection between EDS/hypermobility and ME/SEID.

    In Part 2 of the series he states:

    In his webinar for SMCI, he goes into more detail, including a discussion of the paper his group published on impaired mobility in a group of hypermobile ME/SEID patients, basically suggesting that people with hypermobility are stretching/straining more due to ligament laxity but that nerves don't stretch like ligaments. The constant strain of muscular-skeletal instability in the hypermobile creates the aforementioned diminished neurodynamics. At least as I understand him. ;-) Not sure what to make of it yet, especially as some of it seems a bit woo-woo, but it has gotten me even more interested (as if I wasn't already) in paying attention to basic muscular-skeletal mechanics.
     
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  10. Valentijn

    Valentijn Senior Member

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    Another possibility is that some of the non-flexible people getting diagnosed with EDS3 have a different collagen disorder instead. EDS is just one of more than a dozen such disorders, and dozens of genes are involved in creating collagen. I haven't looked into them, but I imagine that at least some might have similar features to EDS, even while not resulting in hypermobility.

    Rowe's hypothesis regarding nerve damage is interesting. Similar models that rely on central (brain) sensitization pretty badly fail to explain most symptoms, but if nerves are physically being damaged, that could account for both pain and some broader regulatory failures, such as is seen in OI. Though I still don't see how that could tie in with PEM, unless it somehow completely boils down to a failure to clear metabolic wastes after exertion.

    Though in the case of the nerve damage theory, I'd have to wonder how it could account for ME who patients manage to have significant remission.
     
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  11. Jonathan Edwards

    Jonathan Edwards "Gibberish"

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    It looks very woo-woo to me. I cannot see how this can possibly explain the vast majority of people's ME. And I have looked after hundreds of people with damaged joints from rheumatoid arthritis with instability and soft tissue changes and it never produces brain fog or PEM or unrefreshing sleep or any of those things. It just hurts the way you would expect.

    The real problem with this is area is that the 'experts' sound off about all sorts of things without having actually done any proper research. People say that hypermobility can be managed with things like braces - but where are the studies that show it makes any difference - there are none. Moreover, hypermobility , as measured by a Beighton score has pretty little to do with needing braces. Beighton's score measures increase in range of movement in the normal planes. Braces are used by and large to deal with instability in abnormal planes - like severe lax knock knees or maybe shoulder dislocation (there is no such thing as 'hypermobility' of the shoulder since it is designed to move in all directions, dislocation is something quite different where the joint surfaces separate).

    Hypermobility as judged by Beighton scores of 5+ or by 'being double jointed' is extremely common. I would guess one person in twenty. We all had someone in our class at school who could do clever things with joints like put their hands flat on the floor and bend their thumbs back to their forearm. These things are perfectly healthy. The sort of joint laxity problems that are actually a health issue and now get called EDSHT occur in about one person in ten thousand. How many people at school had to wear knee braces for ligamentous laxity? - I would guess for nine out of ten schools none.

    And the difficulty is that the 'experts' muddle everything up and produce generalisations without any scientific data. I am even doubtful that there is any good evidence for an association between hypermobility and ME. Where is the epidemiological study that shows this? Th e experts think there is because they say there is and so they get sent people with both. It would be scientifically very intereting if there is a real relationship between the two, but until we have some data on that I am a bit doubtful it is worth scratching heads over it to be honest.
     
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  12. Hutan

    Hutan Senior Member

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    Indeed, where is the study? It wouldn't be hard to do. But it takes funds and acceptance that ME is actually an illness worth investigating...

    Dr Chris O'Callaghan is a pharmacologist, he runs a hospital blood pressure disorders clinic. He is well-respected. My GP nearly fell off her chair with happiness when I suggested I get referred to him for POTS (finally something and someone she didn't have to google before forming an opinion). She told me he writes national clinical good practice guidelines. He's been the head of the Victorian Medical Safety Committee (Victoria = Australian state).

    He gets sent people with orthostatic intolerance, lots of them. And he believes there is some link between hypermobility and orthostatic intolerance and ME/CFS.

    My son is first up to see Dr O'Callaghan in July about POTS. The extensive questionnaire we have had to fill out covers, among other things, personal and family history of EDS symptoms. I'll ask about what evidence Dr O'Callaghan has for his belief when we see him and let you know what he says.
     
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  13. Michelle

    Michelle Decennial ME/CFS patient

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    Quite.

    What's remarkable to me having both ME/SEID and EDS3 (and I was one of those with a knee brace in school -- and an ankle brace from when I was 10 that I still use from time to time as I stopped growing at 12) is that since I got my EDS diagnosis, my symptoms are taken far more seriously by clinicians than my ME/SEID symptoms are even though there is more evidence to support the abnormalities seen in ME/SEID. I'm amazed at how clinicians will make sweeping proclamations about EDS when there's no damn evidence for what they're saying (Vitamin C as a "treatment" for EDS? Really?!). Yet when you show them decent (not great, but decent) research about problems with, say, aerobic exercise and ME/SEID, they completely blow it off.

    With regard to Rowe, I share your skepticism (though with less capacity/context for adequate critique). I should note, his group published their findings in the Journal of Pediatrics last year ("Impaired Range of Motion in Limbs and Spine in Chronic Fatigue Syndrome" Rowe, et. al. August 2014) so his theory has been subject to some form of peer-review (for whatever that's worth). ;-) If I remember correctly (which is highly dubious on a good day) he argues that PEM is caused by the increased metabolic load required to accommodate poor movement mechanics that result from hypermobility. He also argues that infection can cause neural edema that would have a similar effect to the neural edema caused by hypermobile movement mechanics (I'm not sure "movement mechanics" is the right way to explain what he was saying but my brain is not forthcoming with the correct terminology and I don't feel like watching his webinar again).
     
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  14. Jonathan Edwards

    Jonathan Edwards "Gibberish"

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    But hang on, he now seems to be saying ME/CFS patients are HYPO-mobile - that they have less mobility rather than more. I am a bit confused!
     
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  15. Valentijn

    Valentijn Senior Member

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    Yeah, that bit really doesn't seem plausible.
     
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  16. Valentijn

    Valentijn Senior Member

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    I read it to mean that we have to work harder to keep our legs from falling off and such. But the extra work wouldn't even remotely explain either the small amount of exertion which triggers PEM, nor that whacky symptoms that manifest during it.
     
  17. Jonathan Edwards

    Jonathan Edwards "Gibberish"

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    But he is saying that PWME have restricted joint movement rather than excessive laxity it seems. Nothing to do with exertion in this case.
     
  18. Michelle

    Michelle Decennial ME/CFS patient

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    Yeah, that threw me initially too. But in the paper they argue for impaired range of motion in specific places in ME/SEID patients compared matched hypermobile controls.

     
  19. Jonathan Edwards

    Jonathan Edwards "Gibberish"

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    Yes I missed that they were matched for hypermobility. That presumably means matched for Beighton score features. So it seems that for people with similar Beighton scores PWME have more trouble with a rag bag of tests vaguely related to neural tension signs associated with root entrapment. I cannot see why they are testing foot dorsiflexion, except that it is a component of the scietic stretch test, although on its own it would not have much to do with nerve stretching, similarly for prone knee bend and the rather dubious femoral nerve stretch.

    I honestly find it difficult to know what one could make of this> It seems to suggest that if PWME have nerve tension problems it is not due to hypermobility because other people with hypermobility do not have these problems. I think the basic problem is that there will be a huge confounding factor relating to sampling bias.
     
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  20. Hutan

    Hutan Senior Member

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    From Ros Vallings report on the 2015 IiME conference:

    Amolak Bansal (Surrey, UK) gave an overview of the diagnosis and differential diagnosis on ME.
    ...... Other physical signs include: joint hypermobility (20%)


    So this doctor has identified an association between hypermobility and ME. He is seeing hypermobility in 1 in 5 people with ME. Chris O'Callaghan suggested the frequency of hypermobility in the general population is 1 in 20.
     

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