An ex-boyfriend of mine once said “You’re weird you. Things happen to you that just don’t happen to other people”. At the time I was understandably miffed, and even more miffed that there was more than a grain of truth in his words.
Looking back to early childhood there are huge red flags that something wasn’t “quite right”. One of my earliest memories (I’d be about 4 years old), was being pinned down on the bed, screaming blue murder, while my Mother shoved Vaseline up my back passage because I was constipated….again. I’m now in my mid forties, and I still only poop every 2-4 days! I’m not constipated in the traditional sense, ie. little, hard, rabbit pellety poop that is dry and hard to pass. My poop is usually normal in texture, but sometimes so huge expelling it is a bit like giving birth. This, I’ve discovered, is due to over stretchy collagen in my bowel which expands rather too much and ends up producing crap the size of elephant dung 😉 Add to this what I personally suspect to be a lax Ileocecal valve, where much of my poop gets stuck in my small intestine instead of moving on smoothly to the cecum/large intestine, and you get chronic lower right-sided abdominal pain and poop that backs right up to your stomach, causing reflux, gastritis (stomach inflammation) and chronic nausea. Lovely.
However, I digress. Back to childhood, and my Mother’s favourite description of her only daughter: “you see more of her arse than you do of her face!”. This because I spent 80% of my time doing cartwheels or handstands or backflips – they actually felt more comfortable than my upright self. In other words, I was highly flexible. My party trick was picking my nose with my highly flexible tongue (I’ve since learned this is called a positive Gorlin’s Sign), or rotating my elbows round by 180degrees, usually to shouts of “oh my god, you freak!”. Over the years, lots of medical staff have commented on my “double jointedness”. No-one ever mentioned it could become a big, big, problem.
I’ve always loved to dance. I’m not talking Anna Pavlova or Margot Fonteyn (I live in a remote rural area where, when I was a kid, there were no ballet classes, or after school activities of any description come to that), just your regular bopping along to the Top 40 on the radio on a Sunday night, and later getting tipsy and doin’ ma thang in the local nightclubs. But I always danced on my tiptoes, even when I was wearing 3″ heels. Trying to dance with my feet on the floor felt like someone had put both legs in a plaster cast. I later discovered I had very high foot arches, but when I stood up these collapsed due to weak collagen and made me very flat-footed which means I tend to stomp about like a great Clodhopper. My flat feet also make my ankles turn inwards, which in turn make my knees turn inwards so that I am what is elegantly called “knock kneed”.
High arch at rest Flat foot bearing weight
Non-weight bearing ankle Weight-bearing collapsed foot arch and rolled ankle.
In my early teens I had dental over-crowding which was making my front teeth crooked, so I had to have 4 of my molar teeth removed aged about 11. I can now always joke that, far from having a big gob, my mouth is actually too small for my teeth 😉 Little did I know then that the red flags for Ehlers-Danlos Syndrome were getting redder and flaggier by the day.
I also started my periods at the age of 11, and have suffered from what is medically termed dysmenorrhea to this day. If you want to call the feeling that someone is in your uterus with a blow torch and a pair of pliers, and cramps so excruciatingly painful they have you groaning in the fetal position, vomiting, and running to the toilet for days with explosive diarrhea, ‘dysmenorrhea’ be my guest. I just call it The Curse and can’t wait for the Menopause and the 4 weekly torture to be over.
Again aged 11 (see, there’s a puberty theme developing!) I started having back pain. And to cut a long, arduous, frustrating and infuriating journey short, I was diagnosed with Congenital Spinal Stenosis aged 16, and had laminectomy back surgery. It didn’t cure the pain, which is now so bad it stops me doing almost anything, including sleeping and wearing high heels which I mourn much more than being able to hoover my lounge carpet ;-).
Around the age of 13 I developed daily headaches. They weren’t migraines, but more a tension headache which for some reason always appeared at the same time each day. Because of this, and the era in which I lived (things were different in the 70s!), I was basically told I was bringing them on myself and to stop being such a weakling. Panadol Extra became my new and bestest friend. The migraines started when I was in my early thirties. For ten years, although I still had chronic headaches, I only had Classic Migraines with aura (in my case zig zag flashing coloured lights which obliterated my vision within minutes) but no head pain. Then around the age of forty the Common Migraines began, the kind with no aura but pain so excruciating I wished someone would just chop my head off and be done with it. At my worst I was up to around 2 common migraines per week plus the ever present daily headache. These have settled down now to about 3 migraines per month, and hardly any headaches – I wish I knew why, but I’m clueless!
Throughout my child and early adult-hood I was very sporty. I was on the school hockey, netball and tennis teams, though I seemed to get completely knackered when running, much more so than my peers. I rode horses, biked, did my BAGA gymnastic awards and my bronze and silver swimming medals. Later, I did dance aerobics 3 nights a week, and lifted weights in my local gym. I just ignored the fact my back still hurt and that I always fell asleep on the couch at 10 o’clock at night.
Boyfriends commented on what lovely, soft skin I had, but I just thought they were being romantic. I had no idea that other women’s skin didn’t feel like velvet. I also had no idea other people didn’t wake up covered in bruises with no clue how they’d been conceived – I just figured I must’ve banged into some object the day before and just forgotten I’d done it. Good job my Mom wasn’t much into smacking me as a kid, otherwise she might’ve been had up for child abuse! See photos of how stretchy my skin is on my Skin Blog Post.
I was always a messy eater. You could tell what I’d had for my lunch from what was spilled down my top. This gave rise to the inevitable round of jokes from my family who were amazed that someone with a mouth as big as mine could ever miss it with a fork full of food. For Christmas one year, when I was about 35, my Mum bought me a Pelican Bib as a gift – she was only half joking I should use it ;-). I could also never play games that involved hand-to-eye co-ordination, like darts or snooker. No matter how well I thought I’d aimed, the ball never did go in the pocket. I later found out my Proprioception was shot and my brain had no clue where my limbs were in space, which made judging distances tricky. Saying all that, I can park a car in a tight space like a pro!
The “you’re weird you are” boyfriend comment came on a holiday in Greece, where I’d been bitten alive by bugs (a standard occurrence for me when abroad) and spent days being sick due to the change in food, water and climate (another standard holiday response). I just felt like a total loser as everyone else always had a great time on holiday, but I just seemed to find it an ordeal, was always ill and often came home more exhausted than when I went. Of course I knew nothing then about heat and its effects on dysautonomia and mast cells.
In 1994, when I was in my mid twenties, I was diagnosed with Myalgic Encephalomyelitis (M.E.). My ME story could fill another entire Blog but there is a brief summary here. Miraculously, I started to recover somewhat from ME in 2004, although I do still have the disease.
As I became more active again, I started to suffer from widespread pain – it felt like my muscles were tearing at the slightest strain, along with stinging and burning and all-over-pain I find hard to describe. In January 2009 my ankles started to seize up first thing in a morning, and for a good hour each day I could barely walk. Then 3 months later both my elbows developed burning and stinging pain that travelled down my arms to my hands. I was diagnosed with “multiple point tendonitis” but no-one knew why it was happening. My back was stiffening up – not only could I no longer do a back flip, I couldn’t bend backwards at all. I developed a burning pain between my shoulder blades and my head felt like it was attached to my spine by a red hot poker. My right wrist became so sore I could no longer lift the kettle and, let’s face it, life without tea is pretty much unthinkable 😉 My thumb and several fingers were also playing silly buggers. With not a part of my body free from pain I asked my GP to refer me to my nearest ME specialist Dr Gavin Spickett for an assessment.
While I was waiting for my appointment I became increasingly worried about a chronic, bronchitis-like cough that I’d had for about 2 years. I’d never smoked, lived in a beautiful clean-air environment and, as far as I knew, didn’t have any kind of asthma. Yet I’d start coughing for no apparent reason, and it would go on for as long as 5 months at a time. It was exhausting, and at times my chest felt so tight and I felt such an air hunger that I thought I’d die in my sleep. Several trips to my GP were fruitless, with a brilliant peak-flow reading of 500 (average is about 350 for my age and build!) and a totally normal chest X-ray. Plus I didn’t wheeze or get short of breath like in a typical asthma attack, just had this chronic, dry, unproductive cough and what felt like an elephant which had taken up permanent residence on my chest. I was prescribed various inhalers which didn’t help one iota. The only thing which seemed to shift the cough was a course of antibiotics – only for it to keep returning just a few weeks after I’d finish the course of tablets.
I saw Dr Spickett in the spring of 2010. His investigations into my chronic cough, which included blood tests for moulds and inhaled allergens like dog dander and dust mite, were negative. He said my widespread pain wasn’t ME-related and was probably due to my hypermobile joints. Of course I already knew I was double jointed, but no-one had ever warned me it could cause pain of any kind. He referred me for an MRI scan of my spine, which showed very little other than some mild osteoarthritis. I was gutted to be honest – how was it possible to be in this much pain but have “nothing wrong” with me?! (I’ve since learned that people with EDS need an upright MRI scan. Like with my foot arches, my spine may appear fine at rest – the issue is with weight bearing and consequently collapsing ligaments and tendons and the affect these have on discs and nerves, which can only be seen when the spine is upright and bearing weight). I sat in the hospital car park and cried, knowing something drastic was going on but not knowing who to turn to or what to do next.
I came home and thought I’d look online to see what “hypermobile joints” were all about. I found a disease called “joint hypermobility syndrome” and another that I’d never heard of called “Ehlers-Danlos Syndrome Hypermobile type” (although several other types of EDS exist). Ding, ding, ding folks, we have a winner! Reading through the symptoms for HEDS was like reading about my own life, from the childhood flexibility, constipation, menstrual pain, back surgery and dental overcrowding, to the migraines, bruising, clumsiness, tendonitis and stiffness of my later years. I asked my GP for a referral to a Rheumatologist, Dr Paulene Ho, who diagnoses people with EDS and Joint Hypermobilty Syndrome and was told I was a “classic case” (although I later found I could have seen a consultant much closer to home, Dr Hassan lead Rheumatologist at the Cumberland Infirmary Carlisle). She couldn’t believe it had taken 42 years for the disease to be picked up, but it doesn’t surprise me one iota that I had to diagnose myself and chose a specialist knowledgeable in EDS to have it confirmed, as most of the health staff I’ve talked to locally have never heard of it, including my GP. More about my treatment and the rest of my EDS journey in my blog posts.