But I’m a horse!

Update May 1, 2017: This post was originally written in 2014, before the brand new category of Hypermobility Spectrum Disorders was introduced by The Ehlers-Danlos Society in March 2017 for those who are “less bendy” or symptomatic per the new EDS criteria.

Thus, I will be re-writing this entire post to reflect this fact and not the now obsolete and outdated Brighton (with an “r”) diagnostic criteria it used to be based on. And updating the diagnostic references as needed.

Update May 2019: I just rewrote the entire post as But I’m a Horse Redux 2019 here. You may wish to read that now instead. I will keep this post for historical purposes now only.

I will also no longer say EDS is not rare, just rarely diagnosed.

But I WILL say that the newly recognized Hypermobility Spectrum Disorders are NOT rare, just rarely diagnosed now, as I’m positive that well over 2% of the population may have signs that have just been either dismissed or worse: normalized by doctors, many of whom actually have it themselves!

“Oh everybody does that!” they say. No, they don’t. But because birds (or zebras) of a feather flock together, you are probably surrounded by fellow family and friends who do.

[Update April 2019: The latest literature indicates that HSD and hEDS together may run as high as 3.4% of the population. (Tinkle et al, 2017)]

We are highly attracted to each other I find, both platonically and romantically, adding to the difficulty seeing something that’s been hiding in plain sight all along. Just as awareness of autism is slowly but steadily increasing, so more are getting recognized, I believe the same will happen now for both hypermobility, and mast cell activation syndrome.

And maybe, just maybe, we’ll finally start listening to and believing each other when we complain of something, and not just dismiss anything we can’t easily see.

Original somewhat out-dated post from 2014:

If you’re not aware by now, you sure should be that May is Ehlers-Danlos Syndrome Awareness Month.

And you should also be aware that the zebra is our rare disease mascot. But, in case you weren’t aware, I’ll refresh the history behind it a little so as to explain myself.

The saying goes that in medical school, doctors are trained upon hearing hoofbeats behind them, to think and diagnose the more common possibility, a horse so as to avoid over-diagnosing rare diseases.

Needless to say, we turn out to be that less common occurrence, a medical “zebra” (in the western world), which helps explain the difficulty we experience getting diagnosed, as I’ve explained at length in About EDS and When to Suspect EDS. Naturally this leads us to sport lots of zebra themed attire and décor during May, but even all year round for the dedicated.

[Edit April 2019: There is a small but growing movement lead by someone other than me on social media to ditch the zebra as our mascot and color theme. Some find it childish, others feel it’s misleading, since the most common form of EDS and the HSDs together are far from rare, just rarely diagnosed.

No concesus has arisen on what to replace it with however, and this would entail a major rebranding for all the leading charitable organizations which feature it. I’m personally agnostic since I’m not using it myself.]

That said, I’ve also pointed out repeatedly how leading experts are now [in 2014 when this was originally written] citing EDS may run as high as 2% of the general population (Castori et al 2012), or 1 in 50!

And I must say from my own personal unscientific observations, I’m utterly convinced this is true. The thing is, most don’t have such a dramatic or “visible” experience as I have had, not becoming afflicted in any visible way (though they are afflicted in plenty of invisible ways like pain and POTS!), and so remain undiagnosed, and struggling to be taken seriously and heard.

It is my personal, totally unscientific theory that most of us who are most visibly and seriously afflicted and thus more easily diagnosed are likely double allele carriers, i.e, we got a dose from both sides of our families, even if this may not be obvious at first glance. (I personally also think hypermobile people are attracted to each other for a variety of reasons, both physically and mentally making this so). 

Personally EDS runs most clearly on my late father’s side, but I now suspect my late mother in 20/20 hindsight as well. (She had full dentures, varicose veins, phlebitis, anxiety, depression, headaches, weakness, fatigue, very thin skin that tore at a glance, easy bruising, more. Thing is, she left when I was just 10, so I’m working off old memory here.) I’ve watched several colleagues slowly draw the same conclusion in our support groups over time also.

[Edit 2018: I’ve since met a cousin on mom’s side online who was formally diagnosed by none other than geneticist Dr. Brad Tinkle, so guess what? I was right… sigh.]

So clearly, while few of us are getting diagnosed yet, there are many more suffering sub-clinically among us, and at potentially 1 in 50 that’s far from rare! Yes, some specific types of EDS are rare, some thankfully extremely so. But I feel very strongly the collection as a whole, especially the milder manifestations sure are not.

So I feel justified in declaring myself a medical “horse”! As I told my doctor in 2012, if I’m rare in any way, it’s for being a Portland native with EDS. Or for being into Balkan folk dancing and languages and knowing Pi to 20 digits. But not for having hEDS, by far.

Again, I feel one of the main reasons for this is the hyper-focus on the 9-point Beighton Hypermobility Scale, rather than the rest of the manifestations described in the Brighton Diagnostic Criteria (of which it’s just a confusing part) for diagnosing the most common Hypermobile EDS.

A majority of HEDS patients I’ve met (including myself) no longer “pass” or score very high if at all on the 9pt Beighton Scale! (I used to be very bendy as a child, would have been a 9/9 easily, but am now a 2/9 and know some who are 0/9).

/Update May 2017: The Brighton (with an “r”) Diagnostic Criteria have now been obsoleted by the new diagnostic criteria for hEDS and the HSD category here now. This post will be either completely re-written or deleted soon. So the following information is now outdated, but I’m keeping it for historical perspective and purposes only now Nov 2017./

But all of them (and most with Fibromyalgia) pass the brightonslide2013 Criteria with flying colors when read thoroughly and carefully. (We got “bright” in Brighton, England, right.) I’m seriously concerned that the 9 pt Beighton Scale mentioned at the start of the Brighton Criteria continues to be a red herring that serves only to mislead and confuse BOTH doctors and patients just learning about this complex condition. Dr. Jaime Bravo in Chile agreed with me in an email when I described a very suspect but totally non-bendy friend, he still suspected her based on my description of everything else. He also speaks from personal experience apparently:

August 3, 2012

“Dear Jan :

Your friend probably also has Ehlers-Danlos type III, even though she is not lax. In my studies I have found that 50% of them are not lax, I am one of them. Have a GOOD DAY.

Dr. Bravo”

The problem is, everyone reads the Brighton Diagnostic Criteria from the top down, left to right like good western English speakers, and their minds seem to snap shut upon failing to pass the first two major criteria involving increased hypermobility and lots of joint pain, and proceed to mostly ignore the seven other minor criteria having nothing to do with being bendy! I watch this happen repeatedly, and so have begun running them backwards to that end.  Eyes keep opening as people realize they don’t have to be bendy to have HEDS! (NB, Vascular EDS is not known for hypermobility much either, and it is far more dangerous).

See for your self with my (totally unofficial) Reverse Brighton Criteria:

8 MINOR CRITERIA (4/8 bolded have nothing to do with the joints, 7/8 have nothing to do with being at all flexible or hypermobile since most are no longer, and some never are!)

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

2 MAJOR CRITERIA:

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

You “pass” if you score either any 4 or more minor criteria, or 1 major and any two non-matching minor criteria (the last two minor criteria shown above are a restatement of the major criteria, that is to say “match” them), or both major criteria. Alternatively, you can pass with any two minor criteria if you have a known (diagnosed) first degree relative (parent, child, sibling). Again, this is MY unprofessional unofficial variation on the Brighton Diagnostic Criteria to help de-emphasize the 9pt Beighton Hypermobility Scale, as many stop reading when they don’t pass the first major criteria in the proper Brighton Diagnostic Criteria.

This breaks my heart, since this is the whole reason these were developed: to help smoke out “non-bendy” EDS patients based on all the “extra-articular” (non-join-related) signs and manifestations we can have. But I sometimes feel like I’m beating a dead horse, to mangle a phrase. (Sorry!) I’m also not a doctor, just a very pedantic well-read patient, so please consult YOUR doctor or health care provider for proper diagnosis and treatment.

I’ll stop there. Please feel free to keep wearing zebra stripes to raise awareness though meanwhile as you wish, as I fear it’s going to be a fair while still before the notion of EDS being common catches on. 

I just ask that you help highlight the non-joint-related signs of EDS of all kinds so as to help raise awareness of it for that 50% Dr. Bravo referred to and that I keep smoking out too. Meanwhile back to the therapy pool for this horse, so as to walk again another day. Thanks for reading.

Jan