The Hypermobile Red Herring

Or why I think hypermobility is a misleading sign… March 2021

Phew! Crazy times we’re living in. I intended to write this post almost two years ago now. Then, I got a pandemic for my birthday in March 2020. And a second and final (I hope!) move within my preferred apartment building to the top floor last November 2020, well away from the barbeque odors, gas-powered leaf blowers and screaming children running literally back and forth around my door in the hall because… kids. 

Along with going a bit overboard serving my local houseless community this past winter under my own steam, here we are in March 2021. Still dodging COVID-19. And waiting oh so patiently for our vaccinations at least in Oregon, among other things. But also finally getting back to the writing board. 

That said, I had a flash of insight upon watching a video of a talk by Jane Simmonds in 2017 (around the 20:30 mark). I’ve been struggling with the word “spectrum” in Hypermobility Spectrum Disorders since they were invented and described in 2017 – because, a lot of people kept thinking it only referred to the hypermobility or bendiness bit being on a spectrum.

That is, we’ve taken it to be a spectrum ranging from least bendy (and so least visible) to most bendy (most visible). And quite understandably, since a) the very word is in the title itself, and b) all of the literature and criteria to date has been hyperfocused on the Beighton 9 point scale, i.e, your bendiness score. And it’s one of the most visible signs, too. But…

screen cap of a two sided arrow showing a spectrum from less sick to more sick
An illness spectrum

(Screen capture above of a slide from Dr. Simmonds’ talk in 2017 to the HMSA showing a spectrum of ILLNESS, not hypermobility from Simple/Acute on the left to Complex/Chronic on the right.)

…this is not always the case! As her slide above alludes, it can be about the symptomatology – or how “sick” or affected you are.

Which rang a clarion bell for me – this is what I’d been trying and failing to convey in my little Oh TWIST group on Facebook for the last two – nay – THREE years!

SOME people are very bendy (aka hypermobile), but… not very or even at all affected! They would just be called hypermobile, but NOT diagnosed with a Hypermobility Spectrum Disorder. (Emphasis on disorder.) Or a form of EDS. (Same difference IMHO.)

Until and unless they became more affected. That is, symptomatic, with issues that drive them to the doctor. Not just bendiness, which may even just be a fun advantage in their life. (Think Sofi Dossi of AGT, Houdini, The Ross Sisters, etc.)

SOME other people might not be nearly so bendy (aka hypermobile) but… they may be mildly to severely affected with issues with their tissues due to a clearly underlying systemic connective tissue disorder. (Raises hand myself now. I also suspect many late celebrities in hindsight, like Michael Jackson, Prince, Marilyn Monroe and even Elvis. But just my hunches to be clear.) 

These people would (or should) be diagnosed with a form of Hypermobility Spectrum Disorder IFF they have ruled out all other possible causes of so many issues with their tissues, including all other forms of EDS, (especially hEDS) and other HCTDs like Marfan, Stickler syndrome, OI, etc. As well as ruled in or out any additional comorbid autoimmune diseases which can greatly mask hypermobility in my opinion.

In other words, you aren’t being diagnosed because you are bendy, in either case! But because you have pain and issues with your tissues in a fairly systemic manner that lends to suspecting a connective tissue disorder. 

“If  you can’t connect the issues… think connective tissues… “ – anon. (as quoted by Dr. Heidi Collins, MD in her talk of 2014 with this as the title)

So while being super bendy may have seemed to be a diagnostic asset up until now, it’s really just been a big red herring. That is, a misleading sign sending everyone down the wrong diagnostic path by both its presence, AND absence.

Some who are quite bendy but have few to no problems or approved signs technically do not have a connective tissue disorder according to the powers that be. Yet I watch both patients and even many smart doctors fall for someone’s extreme flexibility and hypermobility as a sure sign they “have EDS”. All. The. Time!

But we should really just say “wow, that person is really flexible/bendy/hypermobile!” Period. QED. As we truly know nothing else about them just by looking. Full stop.

And conversely, those of us who, like me, may have either a) stiffened greatly with age and arthritis onset, or b) were born semi-stiff or not very bendy like several I know of, but have loads of issues with our tissues are regularly overlooked and gaslit into thinking its’ “all in our heads”. And we’re “just depressed”~! (My fav, ha.) Precisely because we are not very bendy. (That doggone red herring again!)

No, doc, sorry, but depression didn’t split my SI joint in 2012, nor loosen all my fingers, ribs and toes and cause cranio-cervical instability and settling in me. Nor cause my friend’s severe lymphedema, deafness, CSF leaks, hiatal hernias, ganglion cysts, tendinitis, and chondromalacia patella. (Plus loose or weak teeth, severe myopia and astigmatism and so much more.)

And the even more confusing part? From the annals of stuff I just can’t make up: sometimes, you will have BOTH kinds of people even in the same family! No, really! Is it anyone wonder our doctors are confused? Heck, even patients are confused!

Do check your impostor syndrome if this is you, especially if you have a larger platform due to minor celebrity. You may mislead a lot of people inadvertently, ahem. Because no, EDS as a whole is NOT rare, just rarely suspected and diagnosed, by everyone. Not just our doctors.

Yes, some forms are quite rare. I happen to be best friends with one of the few with the newly recognized classical-like EDS. (Not the well-known classical EDS aka “cEDS”, but clEDS. There’s a difference.) It took her over seven years to get she and her very affected son properly diagnosed by the best doctors in the world. Finally. 

But, you would no more say that cancer is rare because some forms like ovarian cancer are, would you?

No. The collection as a whole is not rare. Even if some forms are

You special folks may continue to be special and have your own secret handshakes and meetings away from the madding hypermobile crowds. As well you should! You definitely have some unique issues and concerns us “garden variety” hypermobile folks don’t.

But to continue calling the entire collection of forms of EDS with or even without the new categories of HSD “rare” is a serious disservice in my unprofessional opinion. Again, it puts doctors off the scent. And often some patients too. Like a self-fulfilling prophecy. 

Heck, Castori et al cited as high as up to 2% of the general population may have #hEDS but a majority are undiagnosed in 2012 here. That’s far from rare. (2% equals 1 in 50 people. Look around you, you undoubtedly know someone with this at that rate, I promise.)

And Tinkle, Castori, Berglund et al cited one study showing as high as 3.4% may have hEDS or a proxy for it in 2017! That’s far from rare! If you then add in the other rare forms of EDS, well, at even just 4% total that’s far from rare.

I’m sorry, but I’m just not that special – and I don’t need to be. Unlike some of my colleagues apparently.

But more importantly: I’m no longer very hypermobile. Even though I used to be, even extremely so like the Ross Sisters shown here. (I have no idea if they had a form of EDS. I mildly suspect so, but again, I cannot say they did. Only that they were definitely quite hypermobile!)

Was I “cured” of my hEDS since I was diagnosed in 2012? I don’t think so! (Trust me, I injure in my sleep every night.) Did I stiffen with age and arthritis so I no longer pass the 2017 criteria? Yes. But just like dear Pluto, I did not otherwise change a spot, nor alter my orbit around the hypermobile sun one iota. I’m still a full planet, dammit! (Smile.) We just changed our minds about what to call me.

Start pulling back, and seeing the bigger connective tissue picture with me. Hypermobility is just a red herring…😉

We have you surrounded!

12 Comments

    Add a Comment

    Your email address will not be published. Required fields are marked *

    This site uses Akismet to reduce spam. Learn how your comment data is processed.