I have a 'modest' Beighton Score of 5, possibly 7 but I can't tell how far 'back' your knees are supposed to bend to 'count' [Edit: I got Mr A to have a look at the charts and then my legs, he says my knees definitely look like the ones in the picture. He also says my elbow is way more severe than the pictures of 'backwards' elbows. So that's a solid 7, meaning I only fail the pinky test!]. I am in pain almost all day, every day. I am always exhausted, I have extreme trouble with sleeping. I can't get off to sleep and then I can't wake up. I can't keep my weight up no matter what I do. I have had mysterious heart problems for years now, wherein my heart will start having palpitations or 'dips' or my chest will hurt or I'll have what feels like a heart attack. These are repeatedly dismissed either as panic attacks or without any answer. I pass the ECG, the nurse/Dr shrugs their shoulders, I am ushered out of A&E with no answers, still terrified I'm dying. This has become so bad that even when these 'attacks' are incredibly painful and even temporarily affect my vision, I no longer seek medical help.
I show SO MANY of the characteristics for people with joint hypermobility related syndromes. Even a quick browse through a list shows that. Lets have a look (I've italicised the applicable criteria):
People with Marfan syndrome tend to have several physical characteristics, including:
- being tall (I'm almost 5'7 and would have been taller had I not spent my teen years starving myself.)
- being slim (I have never in my life been a 'normal' weight. I have always been extremely slim)
- having long, thin arms and legs
- having loose and very flexible joints
- small bottom jaw
- high, arched palate (roof of the mouth)
- deep-set eyes
- flat feet
- breastbone (sternum) that either protrudes outward or caves inward
- crowded teeth
Then we have Ehlers Danlos Syndrome, which is what I was convinced I had up until recently.
Ehlers Danlos Syndrome:
- skin problems
- soft velvet-like skin
- fragile skin that bruises or tears easily
- stretchy rubber band-like skin
- easy or severe bruising
- poor and slow wound healing (usually taking weeks to months to heal)
- small harmless bumps under the skin
- joint problems
- loose unstable joints causing frequent dislocations usually occurring in the shoulders, knees, hips, collar bone or jaw (see figure 1)
- double jointedness (hyper extensible joints), extreme in some cases
- eye problems
- nearsightedness, occasionally extreme
At the base of it all, this has been a horrific year. From January when I started this blog, when I was first realising that something just wasn't right and that maybe it wasn't all in my head, to my rapidly degenerating physical health, to the highs of getting a referral to a Rheumatologist and the lows of waiting to see one, to the emotional confusion of that appointment itself.
Today I watched my 14month old do the splits on a shop floor while the assistant looked on in wonder. "Wow, is she supposed to be able to do that?" she asked, clearly shocked. My heart lurched and I felt sick. I have to find out what's wrong with me so that when the time comes, Bug doesn't have to live in pain while waiting to find out what's wrong with her. As soon as she's old enough I'll be Beighton-Scoring her. She's not living like this, and she's not living with the thought that she must just be fucking crazy. She's always going to be able to say 'Actually, it really does hurt, it hurts because I have [whatever], which means [whatever].' She's never going to endure having her spoons snatched away from her by people who are forcing her to do things because she can't explain to them what's wrong in language they accept. She's never going to leave a doctors office crying and humiliated because some patronising prick with a PhD has told her that her pain is all in her head. She's never going to consider killing herself because she believes the alternative is to live in pain and the limbo of being labelled a hypochondriac forever, with all the lack of sympathy, empathy, and compassion that comes along with that. It may be too late for me, I may have done all the damage already, but she will go through the same struggles over my cold, dead body.