If you’re suspected of having Ehlers-Danlos Syndrome you’re likely to be tested via the Beighton Score. This looks at certain joints in the body and how hypermobile they are:
- Elbow bends backwards more than 10 degrees (1 point per elbow)
- Knee bends backwards more than 10 degrees (1 point per knee)
- Thumbs bend inwards to touch your forearm (1 point per thumb)
- Little finger bends up more than 90 degrees from the back of your hand (1 point each pinky)
- Hands can be placed flat on the floor with your knees straight (1 point)
I have big issues with the Beighton Score. My knees don’t bend backwards, my thumbs don’t bend anywhere near my forearms and I’ve never been able to place my hands flat on the floor with straight legs. So according to this I’d only score 4/9 and not really be classed as hypermobile at all!
Why do they only look at these particular joints? What about floppy wrists (which was one of the tests my Rheumatologist did on me), hypermobile shoulders, a hypermobile neck (before mine got stiff and painful I could turn my head round like the Exorcist 😉 ), hypermobile spine (ie ability to do backflips), hypermobile pelvis (ie ability to do the splits), hypermobile ankles or toes – all of which I have. Plus my thumbs might not bend inwards to touch my forearms, but they do bend outwards at 45 degrees and my wrists bend outwards at 90 degrees.
I have no idea why all the joints in the body aren’t tested, and in various directions. If you come across a Doctor, Physio or even Rheumatologist not particularly clued up on EDS they stick rigidly to the Beighton Score and are probably missing people, like myself, who are obviously hypermobile but just not in the particular joints listed.
The Beighton Score has to be coupled with the Brighton Score to diagnose EDS:
- Beighton score greater than 4 (if there has been an injury or surgery affecting range of movement, this can be considered historically)
- Arthralgia (joint pain) in more than 4 joints for more than 3 months.
- Beighton score 1-3 (or 0-3 if over 50 years)
- Arthralgia in 1-3 joints for more than 3 months
- Back pain, spondylosis (spinal arthritis) or spondylolisthesis (spinal subluxation) for more than 3 months
- Dislocating/Subluxating (partial dislocation) in more than 1 joint, or the same joint more than once.
- More than 3 soft tissue injuries, tenosynovitis (inflammation of sheath around tendons) or bursitis (inflammation of the fluid-filled sac in a joint)
- Marfanoid Habitus; a characteristic appearance including being tall and slim and having long, thin fingers.
- Thin, stretchy skin or abnormal scarring (cigarette paper scarring or easy scarring)
- Droopy eyelids, short-sightedness, double vision.
- Varicose Veins (particularly at a young age)
- Rectal or uterine prolapse
In order to be diagnosed with EDS you should have:
- Two major criteria or
- One major and two minor criteria or
- Four minor criteria or
- Two minor criteria and a first-degree relative (parent/child/sibling) who has been diagnosed with EDS-HT.
To be honest, I also take issue with the Brighton Criteria because I barely fulfill the Beighton score and don’t have “joint pain” in the traditional sense of the word – my pain……..actually there’s so much to say about my pain it needs a blog post of its own. At the time of diagnosis I’d never had a prolapse, hernia, varicose veins, droopy eyelids, am 5ft 2″ with short arms so hardly a Marfans body type and no dislocations or subluxations (although some of these have manifested in recent years) so I only really fitted 3 minor criteria.
The Score doesn’t take into account my:
- Positive Gorlin’s sign
- GI issues (reflux, nausea, early satiety, constipation, GI pain)
- Chronic Fatigue
- Low blood pressure
- Dental issues
- Easy bruising
- Excessive bleeding & poor wound healing
- Menstrual pain
- Blue Sclera or
- Mast cell disease
which, coupled with my history and hypermobility, point very strongly towards EDS. All of which are the reasons I was careful to choose a Rheumatologist who knew what to look for in a suspected EDS patient.
I know good Rheumatlogists who specialize in hypermobility will do a full history, know what questions to ask, not stick rigidly to the Beighton Score and expand on the Brighton Criteria but many doctors will not – they tick boxes on a form and if you don’t tick the box then you can’t possibly have EDS. It has to change. I fell through the net for 42 years because a full clinical history from birth wasn’t taken and no-one joined the dots of my mish-mash of symptoms. When I was finally diagnosed my Rheumy said I was such a “text book” case of EDS she was amazed it hadn’t been picked up sooner. But looking at the diagnostic criteria it shouldn’t surprise anyone because they’re really quite flawed.
If I hadn’t read up about EDS online, realized myself I had it, researched the most knowledgeable Rheumy to see and paid for a private appointment because my GP practice wouldn’t fund an out of county referral on the NHS I’d still be undiagnosed. It shouldn’t be that hard.