There are aspects of being hypermobile that don’t appear on the Beighton Scale. In fact, if I’d been diagnosed strictly according to the Beighton scale I would have failed miserably – my thumbs don’t bend anywhere near my wrist (what they do do is bend 45degrees in the opposite direction) and my wrists also bend outwards not inwards; I’ve never been able to put my hands flat on the floor with straight legs; and neither of my knees bend backwards. At the time of diagnosis I’d never had a dislocation or subluxation in my life either (I’ve still thankfully never dislocated a joint, but have now had several subluxations), which was why it was important for me to see a Rheumatologist who specializes in EDS/JHS. If you see a Rheumy who doesn’t really know much about connective tissue disorders, and who go strictly by the book, I would have scored only 3 out of 9 on the Beighton Score and probably never been diagnosed. As it was, my specialist Rheumy took other hypermobile joints into account (eg. I have a very hypermobile spine, hips, wrists and ankles), plus factors like a positive Gorlin’s sign which demonstrates hypermobility in connective tissue not even mentioned on the Beighton Scale. She gave me a score of 8/9.
I’ve learned a lot about EDS in the 3 years since my diagnosis, including the fact that many of us have symptoms not mentioned in research literature but which appear quite frequently on patient forums. I have my “Sean Connery” eyebrow:
My “webbed” toes (the proper name for which is Syndactyly):
And the fact I can fit 4 (or more if I had more!) fingers in my mouth in a straight line up to the knuckle at the same time. I’m barely opening my mouth in this photo – I daren’t, as it’s very prone to dislocation, so imagine how large it would be if I truly over-extended it!
Thankfully, it’s been recognised in the past 5 years in particular that hypermobile joints are often the least of your worries when you have EDS. It’s a multi-faceted disease, with nearly all patients having degrees of GI complications, autonomic dysfunction and chronic pain. In addition, many patients experience debilitating fatigue, sleep disturbance, migraine/headaches and a significant minority also have mast cell disease. I’ve recently added a link to a 2013 video presentation by Dr Fancomano to the Resources Page, which outlines the systemic nature of EDS beautifully. I wish I’d had this knowledge at my 2010 neurology appointment, where the Consultant basically told me there was nothing wrong with me and I was obviously just sensitive to pain! Quite the opposite is, in fact, true. Most people with EDS live with a daily degree of pain which would floor most healthy people, and they do it without a murmur. I personally live without any form of pain relief, as I have anaphylactic mast cell activation to pain killers (and almost all other drugs). I think that was why I sat in the car park and bawled my eyes out following my neurology appointment. I actually think I do remarkably well to live with the pain (and myriad of other symptoms) I do without any of the medications that most people take for granted. Yet here was this snotty woman telling me I was a bit of a wimp and that I really did need to just get a grip. Needless to say I have been waiting with bated breath for my mast cell results and a firm MCAD diagnosis (if, indeed, I receive a firm diagnosis), then I have every intention of writing to said snotty consultant (and my ME specialist who is also bloody useless) and tactfully educating them in a disease they clearly know nothing about.