There is a new publication in the American Journal of Medical Genetics that addresses this issue. The journal article is titled: "The lack of clinical distinction between hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome)."

It is written by Brad T. Tinkle, Howard A. Bird, Rodney Grahame, Mark Lavalee, Howard P. Levy, and David Sillence (some of the foremost experts in the disease).

I am not sure if this link will work, without some sort of authentication, but here it is if you need to know where to find it.

http://www3.interscience.wiley.com/cgi- ... /HTMLSTART

A 1-day viewing access costs $30.00 !!! I paid it-anyone else who wants to read it PM me with contact info, and I will let you read it.

I am sorry you had to pay that much... I got access through work without having to pay... I have a print-out of it. I can let people read my copy too if need be.

I'd like to read it...should I PM one of you?

Excerpt:

And THAT is not promising at all, if what I've seen of all the suggested psychologizing is still part of the picture in Grahame's mind. Plus the fact that HEDS has got a clear case definition that already distinguishes it. Plus the fact that he doesn't seem to have demonstrated any definite pattern of heritability, as is the case with HEDS.

I'm by no means saying that these other cases shouldn't be treated, or even that the two groups don't have plenty to teach one another. But this guy has an agenda of his own - and I'm not sure this latest move serves either group well. I'm catching a strong whiff of what happened to CFS/ME/CFS-ME/and-god-knows-the-next-nametag. They played the game with case definitions and words, he's clearly still playing with it, and we all lost big time. I am not for one instant forgetting that a bunch of us zebras got caught up in that one, too. Politically, it's been a bad spot for us. Medically, it's been worse. And it's not about ego - his, mine, or anyone else's. It's about the human suffering that results.

I'm still looking forward to seeing the entire article. I'm totally hoping I'm dead wrong and end up having to soften or correct some of what I just said... by all means, weigh in an whip up on my arguments if you can. If I'm wrong, that's pretty much how I'll find out.

Push - 13+ years, and every time I think I've seen it all...

I'm confused, PushCrash. Are you trying to exclude HMS-diagnosed patients from EDS care? Don't we want good treatment for all hypermobility patients along the spectrum?

For what it's worth, I'm diagnosed HEDS "equivalent to HMS" by a geneticist.

Not at all! I want us all to receive good care. Heck - GREAT care. The problem I see here is that there's nothing convincingly showing yet that the two conditions ARE equivalent, from a physical standpoint.

Here's the thing - how can you have HEDS "equivalent to HMS", when they are A) two different things as far as anyone knows (so far); and B) HMS doesn't even have a standing recognized case definition? HEDS isn't a maybe. It's already got a fully objective diagnosis. Either you have it or you don't. If GHS is something new, then fine - a new subtype of EDS has been discovered. That definition alone carries certain benefits for patients with the dx.

When they play around with case definitions like this, what tends to happen is not that additional people needing to be included and get care are suddenly given care. What happens is that X number of people suddenly meet the 'new' proposed case definition, while another X number of people suddenly don't. After some appreciable length of time, while the practitioners are getting their heads around the new definitions, and after new med students are trained differently, the activists and advocates manage to tool up for it - THEN there's a shift.

But up until then, all hell breaks loose with the patient population. Grahame's a Brit, right? So I won't go there - I don't know the system as well as any Brit likely would. (Though I know parts of the ME biz that even make the US look semi-decent by comparison.) But in America, it means your insurers are free to play games with BOTH case definitions (and they will!). That hard case def for HEDS is about all some of us have going for us, legally. It means the evil of exclusionary pre-existing conditions can be used against a whole new batch of people. It means that if you need to be on disability benefits through an insurer, everything that can possibly be done to deny your claim will be done. If you need to get on Social Security Disability, you can expand the confusion in the bureaucratic community by 10-fold at a minimum - more delayed claims, more people go without, and nothing is solved. If the claims people and judges don't get it - patients don't get benefits. The bureaucrats don't get on board nearly as fast as the medical community. So, worse delay.

I don't say these things because I'm paranoid or permanently tuned to the p.o.'d position. Just the games played with CFS, EDS, LTD, and Social Security alone cost me well over a million dollars I can now never collect or re-earn. That's not a guess or a gripe. Those are hard numbers. I may have to live with it - but I don't want to see anyone else go through that!

Nothing in Congress now is actually geared to fix that mess, except for a fraction of the general health care exclusions. (Those exclusions aren't being banned from LTD plans as far as I can tell. And you can still lose your LTD benefits when Social Security is delayed or denied due to such confusions.) So, even if you limit the fallout to this side of the pond, the natural result is most likely more human suffering - not less.

I hope that makes sense. If Grahame and company were merely seeking recognition for something new (which is what he has said earlier) then ok - maybe an uphill battle, but could be done. Instead, they seem to advocate riding EDS's coattails. Not cooperate, not share info - but create a single group out of thin air? Muddy the waters for existing HEDS patients? I can't find anything physically, socially, or medically factual OR advantageous so far. (If there is some benefit to be found in the UK system, please enlighten me!)

This is not the first time that it has been reported that HMS and HEDS are likely the same thing just differing severities etc. Supposedly Dr Francomano stated this at a HCTD conference about 1-2 yrs ago, they are not 100% certain because the genetics aren't certain for either condition but it is thought to be the same.

HMS does have a recognised diagnostic crtieria which is VERY similar to HEDS except from what I have read in it's severity.

I have NOT read this article but would like to see what they are saying.

I don't know if I am miss reading this but I can't see how more people being diagnosed with HEDS could detract from those who already have esisting HEDS diagnosises.

I have been told at different times that I have just hypermobility, that I have HMS and that I have HEDS which one do I choose? I have no idea, which one do I have ? no idea and until a genetic test is available I am not likely to find out.

From what I know and can find the diagnostic crtieria for HMS is
DIAGNOSTIC CRITERIA FOR THE BENIGN JOINT HYPERMOBILITY SYNDROME (BJHS)

Major Criteria
A Beighton score of 4/9 or greater (either currently or historically)
Arthralgia 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, arachnodactyly [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.

The BJHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

BJHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 (8) and the Villefranche 1998 (9) criteria respectively). Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.

and for HEDS it is
Major Diagnostic Criteria for the Hypermobility Type or EDS

Joint hypermobility, which usually is confirmed by a score of five or more on the nine-point Beighton scale
[Beighton et al 1973]

Soft or velvety skin with normal or slightly increased extensibility

Absence of skin or soft tissue fragility, which is suggestive of other types of EDS. Examples include:
-Spontaneous or easily induced skin cuts or tears
-Spontaneous or easily induced tears or ruptures of tendons, ligaments, vessels, or other internal organs
-Atrophic ("cigarette paper") scars (although mildly atrophic scars are sometimes seen in the hypermobility type, especially
in areas subject to physical stress, such as extensor surfaces and the abdominal wall)

Molluscoid pseudotumors

Surgical complications, such as incisional hernia, wound dehiscence, or sutures tearing through tissues and failing to hold


Minor Diagnostic Criteria for the Hypermobility Type of EDS

Family history of similar features without significant skin or soft tissue fragility in a pattern consistent with autosomal dominant inheritance

Recurrent joint dislocations or subluxations

Chronic joint or limb pain

Easy bruising

Functional bowel disorders (functional gastritis, irritable bowel syndrome)

Neurally mediated hypotension or postural orthostatic tachycardia

High, narrow palate

Dental crowding

"There is disagreement as to whether the "benign familial articular hypermobility syndrome" is identical to EDS, hypermobility type or represents a unique condition. The distinction is subtle and relates to degree of joint complications and presence or absence of skin manifestations. However, first-degree relatives of probands with EDS, Hypermobility Type often have relatively asymptomatic joint laxity and mild or absent skin manifestations. " <- I found this on the net (http://www.ehlersdanlosnetwork.org/hypermobility.html)

I am not taking sides or trying to be offensive or anything just stating my opinion and what I understand from my research etc...

guess firstly I need to state that I am a "grahame fan"
so yep- I'm biased!!

pushcrash- "there's nothing convincingly showing yet that the two conditions ARE equivalent, from a physical standpoint."

the whole "is it or isnt it the same" debate exists because from an easily observable physical standpoint and from the possible symptoms and co morbid array of other conditions- there is NO CLEAR difference-
otherwise why would these guys debate about it

HMS ers like EDSers can and do have:
subluxes/dislocations;frequent injuries like sprains/strains etc; easy bruising;
moderate hyperextensive skin; slower healing, unusual scar formation
chronic pain;chronic fatigue; functional bowel disorders (afaik some 60/70% of HMSers have this)
autonomic dysfunction (which possibly the bowel probs might also be due to) incl. POTS
etc etc etc

the differences between an HMSer and and EDShypermobber dont seem any bigger than differences that can occur between 2 EDShypermobbers....
there's "true" EDShypermobbers (and even classic EDSers) that are able to lead a normal life/are very little affected and there are HMSers that are severely disabled by their condition...
the thought that a "true" EDShypermober is ALWAYS iller, that it's ALWAYS worse than what can occur in an HMSer... is a fallacy.


pushcrash-what have you read that makes you think grahame thinks it's "psychological" ??

as he was the one who dxed me after some years of worse and worse "inexplicable"pain and fatigue- which my gp could find no reason for and gp suspected at times that it was due to psychological reasons..

and it was grahame that said : nope there are physiological reasons for this!

for many many many of us who have seen him, he was the first to categorically tell us that "nope, you are not making this pain/fatigue up, this IS real and not imagined and it has a physiological reason- and if you do have psychological problems (as many do have)- these are secondary to the physiological problem- NOT the other way round"

he does however agree, like just about anyone in the med sector, that psychology plays a big part in how you DEAL with/MANAGE chronic pain- and that this can make a big difference to your quality of life and to levels of pain you experience.

he also thinks afaik, that it is possible that psychological trauma, as well as physiological trauma can maybe trigger the onset of probs like the chronic pain/fatigue/-
but that's also not such an 'odd idea'- psych trauma/stresses even make you more likely to get colds/flu etc...

and saying that a psych trauma might possibly trigger/facilitate a physiological illness- is not saying that the illness is psychological.

and grahame has been battling for decades to get proper recognition of this 'possibly new or possible same or possibly forme fruste" genetic connective tissue disorder- has worked together with c t d specialists around the globe for decades..
and he sees not only HMS patients... he sees all types of people with probs that include hypermobility (yep he also has EDSvascular patients... )
and in these decades... he seems to have found more and more similarities rather than differences


well..I think them possibly finally agreeing to look at and treat these "possibly 2 different things" as "equivalent"- big positive step forward!

all being put "in one pot" (and of course they will still look at each case individually, as no 2 cases of HMS or EDS are exactly alike) tmm has the advantage that suddenly there are many more peops who have these probs= it isnt so 'rare' anymore=docs might actually learn about these things= saving us endless probs!!!

and of course once the 'sufferers pool" is bigger there's more chance for monies/research and all the good that might bring
(and then they might one day actually precisely find out what/if there is a difference
)

as for pre existing conditions and insurance... afaiu HMS is also a "pre existing condition" - you are born with a fault, having usually inherited it (though just like with EDS spontaneous occurrence does occur) and this fault may or may not lead to an incredible amount of problems and disability-
so no change in the chance of insurance screwing you...

and from what I know of grahame... he is def not a "divide and conquer" nor a 'fame/name seeking" guy- the man is a lovely human being.
(so is prof bird btw- we brits are lucky )

nope - I see it as he and the other guys who got together for this paper- (although they actually vary in their precise opinion about this question of "same or not"!!!- and I LOVE the fact that peops of different opinion managed to write a paper TOGETHER)
do want to UNITE for the purpose of sufferers getting better understanding, care and treatment.

although whether this will actually happen... that's not down to just these guys who wrote this paper...
xxg

Yes please. You can PM me.

I agree with Gila. Also, Dr. Brad Tinkle is a kind compassionate person too. He was already saying that he believes these two conditions are the same (it is in his book on the hypermobility syndrome).

I found his words comforting at a time when I was diagnosed with Benign Familial Hypermobility Syndrome. Now that I have been "re-diagnosed" by Dr. Francomano as "Classical EDS", I no longer have to fight that battle, but it is much easier for me to get appropriate care with the EDS diagnosis, than it was when I had the HMS diagnosis.

Thanks Dr. Tinkle and Dr. Grahame, as well as all the doctors who participated in this endeavor.

Lots to think about. (I'm not ignoring - just super busy day, and I want to be thoughtful about my responses.)

I still haven't gotten to read the full article yet. If anyone who has it could PM me?

I do not like that passage. I'm just basing my answer on this passage.

This is just my opinion, but HMS/BJHS is crap. You either have HEDS or nothing in my book (unless it's Marfan or whatever)

I also tend not to agree with most of what Grahme writes as well..."Pain syndrome"??? Here in the US it's a PSYCH DISORDER and is diagnosed when physical causes can't be found or when you're "in too much pain warranted for your disease" which is what my somatization dx was based.

I just recently heard about Dr. Tinkle...is he the new neurosurgeon Dr. F is sending people to?

I was "diagnosed" with BJHS when I was 16...when I was diagnosed with HEDS respect for me as a patient drastically changed. I mean TOTAL FLIP...I was able to get disability related things that I couldn't get before from my state before. There were a few drs like my old rheumy who thought HEDS was just that you're flexible (um no).

I'm really no longer in the debacle as I don't have HEDS but I'm interested in how this turns out...

I think that's their point, that HMS/BJHS may turn out to be HEDS, and that because they present with the same signs and symptoms, they should be considered the same for the sake of treatment. What's not to like?