EDS & me

This month is EDS (Ehlers Danos Syndrome) awareness month. I was diagnosed with EDS Hypermobility in August last year. So I figured maybe it was time to share some of the ways that this random collection of letters has affected myself and my family and to try and shine a light into the wonders of what happens when the NHS is awesome. Because this is a tale of two halves. And the NHS is at fault, but it's also saved my life. A dramatic claim you might think, but one I believe I am entitled to make.

This post will be, I suspect, long. Grab a cuppa. Get settled. I'll begin.

When I was a child...

No wait, I should explain something first. EDS is a lifelong condition. You're born with it. The other types of EDS, of which there are currently 5 major ones, all have genetic markers which have been identified, I think. The type I have hasn't currently had a genetic marker identified but they're working on that. It most definitely can be a genetically inherited thing however, as my mum has it, as does my sister - she's just been diagnosed.

So I've had EDS all my life and never knew it. And oh boy does it explain a lot.

When I was a child, I was very active. I spent a lot of time hanging upside down because that was comfortable. I spent a lot of time being asked if the position I was sat in was comfortable as it didn't look very comfortable. It was. I spent a lot of time with cuts and bruises - most of which we knew the origin of, but in retrospect, a lot of which we did not. I spent a lot of time with either one of my thumbs bandaged and splinted - I sprained each one into double digits in the space of a few years. I fell off a swing one day (I loved swings, a very female Aspergers thing from what I can gather, see also hammocks for side to side swinging) onto cracked tarmac and spent a lot of time in A & E before not getting it stitched. The scar, to this day, is 1cm wide and 2cm long. It looks like no one else's scar.

It goes on. When I was 11 I suddenly developed massive vivid red welts all across my ass. It looked, my mum says, like I'd been sat on a screamingly hot grill wire. I had surgery and a general anaesthetic to remove 5 teeth and have a black stitch cut to get a tooth down from the roof of my mouth. I threw up blood after as the tubing they'd stuck down my nose had somehow failed to do its job. To this day I have a phobia of being sick - you try throwing up blood when you've got a phobia of blood generally.

The 'growing pains' started. Sat in classes trying not to cry at the pain and trying not to show it. I only failed once - the teacher made me feel like a complete idiot and I never lost control of the tears again. I stopped running because it was too painful. I was a good runner before then, anchoring relay teams in the 100m and coming in the top 10 of our 100 people year for cross country. I went to the County games to throw javelin. I stopped all of these things. Too painful. No one asked. I didn't bother telling.

In my teens I developed gastric problems. I wont go into details but I should really have known not everyone had crippling stomach aches which made it hurt to breath any more than a shallow grabbed breath and made sweat run down their foreheads into their eyes. I had to go for blood tests and it took so long to find a vein I was covered in bruises and looked like a heroin addict. It also hurt like hell. I asked around and no one else seemed to be having the same experience, but again I notched it down to 'you're shit at coping, just try harder and deal with it'. And so it went on and on and on and on. More and more systems failed. More and more weird things happened, that I now know to be weird but when you're in the middle of it you just don't pay attention. I started fainting randomly. I had heart palpitations at 14. I had to be careful how I got out of bed and to stand slowly, because otherwise I'd be flat on my face 10 seconds later.

At this point, I feel it very necessary to point out my mother was looking after my dad. He took all her time and all her energy and all her effort. She was doing her best to get help with looking after my dad because she knew we were suffering as a result and she did her best with absolutely zero help from anyone, from the NHS, from charities, from anyone. She was also suffering from her own medical EDS related hell which I wont go into but finally resulted in her getting it sorted 10 years after the problem had started after my birth.

In my 20's, I started spraining my ankles. I can't actually remember how many times I've sprained my ankles but I do know I can now literally sprain my ankle on a kitchen floor in socks if I'm not careful. It's over 20 times between them I do know that much - at least once a year, usually more. I had to have an emergency operation under local anaesthetic again for a spinal abscess . I'd left it too late because I'd simply put it down to 'put up and shut up love, you're just making a drama out of nothing'. And honestly, my pain signals were so messed up by that point that it just didn't hurt. Until the point where it hurt going over speed bumps on the bus and I couldn't sit on the floor any more (it's more comfy on the floor, trust me, I'll explain all this, the why's and wherefore's in a bit) and I figured I'd better mention it to my then boyfriend. Who took one look and said we should go to the out of hours doc on a Saturday, who sent me to  A & E after respecting my wishes of no ambulances. Armed with her letter I was seen quickly, admitted quickly and then left for 36 hours with no food between 8am and 10pm while they tried to find a surgeon. Not that I particularly cared because I had no interest in food. But anyway, I digress.

That wound - the nurse said it resembled a knife wound (we were in Hendon in North London, I guess she'd treated some?) - took 3 months to not need daily packing. It went on and on and on and on. And on. My notes got bigger and bigger and bigger. She wrote up what she'd done every day. In the end my wound started to heal from the top down and they had to burn some of the cells away with no anaesthetic. Yeah. To this day, I have a scar which looks like a 50p piece with a dent under it. It's ugly and horrid and I feel sick every time I feel it. I haven't ever looked at it. I'm nearly 40. This happened in 1999.

Whilst all this was going on, my anxiety was becoming more and more of an issue. Not helped by dumbass stunts like my then boyfriend leaving me in the hospital, visiting once and completely failing to tell anyone I was there - like my mother - and not even bothering to be there to pick me up, leaving that to my housemates, nevertheless, other peoples dickish behaviour aside, I wasn't well mentally either. I fought and fought and fought against it, and mostly managed to hide it and never let it show - that incident with the teacher had taught me well that public humiliation by authority figures was only ever one careless show of emotion away - but occasionally it would break through. Like the time someone thought it was a brilliant idea to send someone with a blood phobia on first responder training and they showed us videos and I ended up on a heap in the ladies toilets floor hyperventilating. Or the time someone in another job though it was a good idea for my confidence building to become a fire marshal and they showed us that video of the fire in the footballs stadium and I went home and bawled for half an hour. Try as I might, trying to keep a lid on everything sometimes just didn't work and it came out occasionally in grumpiness, irritability and curtness. I was icy cold sometimes because that's all I could show in fear of completely and utterly losing it.

Into my 30's. Still no diagnosis and no explanation for why I seemed to find life just a little harder than everyone else around me. Still beating myself up that I was shit and not trying hard enough and I was intelligent and smart and should be doing better than this damnit. Things got slightly better in the first half of my 30's before completely and utterly crashing out.

It started with a dislocated knee. Which dislocated 6 more times in the space of a month. The pain from one dislocation would ease and another one would happen. I went to the doctors. They offered my weight loss surgery. I went away again. I went back two weeks later and was finally offered an xray and some physio. By this point, a dear friend was reading my Facebook posts at the time and alarm bells were ringing. The words hypermobility and EDS started filling my timeline and my internet browser history. It was like being given a key.

It would take another year, possibly 18 months actually, I can't remember, before I was finally, finally, referred to a Rheumatologist and another 3 months to see him and be diagnosed. Inbetween, I would dislocate my left knee a further few times, the forefinger and little finger on my right hand would dislocate, I would start to have bladder issues which were no longer ignorable and minor, and have major issues with heart rate, dizziness and bending down and standing up again without passing out. I would read and discover than not everyone could feel everything the dentist was doing during their root canal because the local anaesthetic didn't work, that not everyone threw up after fainting, that not everyone had major temperature crashes every time they had general anaesthetics, that not everyone could see every blue vein across their chest and down their arms and in their feet, that not everyone could cross their legs then wrap their feet around their legs again, that not everyone got a tired neck which meant they had to read with their head resting on their right shoulder, and not everyone cracked and clicked and had joints which seemed to slip and side at will, especially their ribs.

So, what is the cause of all this? Why does this litany of seemingly unrelated and frankly bizarre information all add up to one label and one diagnosis?

Collagen.

Yes, yes, I don't look my age, well done. And yes, it is that kind of collagen and yes that is probably why I don't look like I'll turn 40 next year, and also why my mother looks nothing like her 62 years either. Genetics, remember?

But collagen isn't just, unfortunately, a thing that affects how old your skin looks. Oh how I wish it were. How I wish I could slap some anti-aging cream on my face and be done. That would be so nice. Nope.

Collagen affects everything. It's kind of systemically crucial. It's the main component of connective tissue. There are different types of collagen and which types are affected depends on which type of EDS you have. Hypermobility type, the most common of all the types, is affected by faults in type 1 and type 3, Unfortunately type 1 is the largest type of collagen in the human body.
From Wikipedia:

This is the most abundant collagen of the human body. It is present inscar tissue, the end product when tissue heals by repair. It is found intendons, skin, artery walls, cornea, the endomysium surrounding muscle fibers, fibrocartilage, and the organic part of bones and teeth.

So EDS affects the structure, processing or production of collagen and collagen is present in all these different places in the body. Which is why my medical history looks like a car crash of confusing symptoms to absolutely everyone I've ever come into contact with in the NHS apart from one chap who is my Rheumatologist and who thankfully studied under Professor Bird, one of the leading brains in this area - or was until he retired. Which is an increasing problem, but anyway.

So my Rheumatologist. Is amazing. That's the first thing I want to say. I saw one of his doctors first and explained everything and within 5 minutes he'd disappeared off to grab him. He asked me questions, we took a tour of my scars (I'd just had a teeny tiny minor surgery under a local anesthetic on my arm so I could show him what my fresh scars looked like - keloid i.e. all raised up and above the skin surface), we took a tour of my dislocations and talked about all the information my mum had written down for me about her and my sisters problems.

And then he said the magic words. 'I'm referring you to my physiotherapist. She's a specialist in rheumatology. She'll understand exactly what's going on and help you sort this out. We don't believe in surgery here, we think it causes more problems than it solves'.

And so my work started which is still carrying on. I saw the physio for 3 months between September and December before she got promoted and moved on. Through her I was referred to a Woman's Physio and then onto Urology for help with my bladder problems. I was referred to Occupational Health who sorted me out with a home visit so I could have aids and help in my home to allow me to do fundamental things like shower without collapsing in bed for 2 hours afterwards, and bannisters on our stairs so I could walk up and down stairs properly for the first time since originally dislocating my knee. But more importantly than that, she diagnosed me with wrist nerve compression and the OT (Occupational Therapist) has sorted splints and supports to help with that. My little fingers are at a ridiculously funny angle permanently thanks to all the dislocations so I have a latex buddy sleeve to sleep in to help them come back together. I have thumbs which drop down way below where they should be so I have thumb splints to put them back where they should be.

But perhaps the most important thing to come out of all of this is the last referral.

The Rheumatology Physio, whose name was M and who works at Blackburn Hospital is perhaps the greatest star in all this, even more so than my Rheumy, though he is a star, because in his team, and almost everyone I have mentioned is part of his direct team, you can see the sense of pride and passion that they come to work with, and that he has instilled in them. A team is nothing without its leader and you can kind of tell he's a bit awesome at leading them. Anyway, I doubt any of them will ever read this but just in case, cos it is the Internet I'll stop there cos I don't want to embarrass people,

Except M. I'll risk embarrassing her. Because she did something no one else has ever done before. She got me to talk about my weight. Not by offering me gastric band surgery when I'd come to talk about something else entirely. Not by trying to persuade me into the kitchen where frankly I'm such a liability it isn't even funny - I have burned myself on a fork in a Pot Noodle. No.

She talked to me like a human being, with compassion and gentleness. She earned my trust, listened to me in other things (I found bed based exercises quite tricky because I kept getting dizzy every time I stood up so we reduced the amount of them I did) and eventually raised the question gently and quietly. As a result of that talking, I self referred to the Healthy Minds service because the Rheumatology Psychiatrist was just about to go off on maternity leave and they weren't getting a replacement. As a result of finally managing to be honest with the second person I spoke to who phoned me back, I was referred to the Eating Disorder Service locally.

Last week I started a 6 week workshop set with the Eating Disorder Service.

Not an easy one to type that.

I don't have a diagnosis. The Clinical Psych I saw said she didn't think anything would be benefitted from it. What she did say was that the reason I was having so many problems was exactly the same as the reason those with anorexia or bulimia have them and so the treatment would be the same - to learn about myself and why I do the things I do and hope to manage those thing and eventually stop doing them in such a harmful way. I left the hour initial assessment feeling like someone finally finally FINALLY understood me without me even needing to actually say anything. She knew things about me and my childhood and my life without me needing to tell her. And that more than anything else made me realise I was in the right place. Labels don't actually matter. Fixing this shit matters. I have EDS, my weight is affecting it and exacerbating it even if it is most definitely not causing my little finger to dislocate or my toe nail beds to turn purple (yeah I just noticed them, must do something about that in a second). I have to sort this out. On my own. With my brain and my effort and my thinking and my trying.

And oh gosh it's hard. The first workshop, last week, was by turns brilliant and awful. So many realisations about how I am and why I am. So much stuff to process that I think it took me three days just to work through 10 minutes of it. But I'm so grateful and so glad.

So when I say the NHS saved my life...

I walked into the Rheumatologist's office on a walking stick, on the wrong anti-depressant medication which was turning me into an unfeeling zombie who was still depressed, with no hope and sick to the stomach of seeing specialist after specialist who would do tests, the tests would be negative, and they'd drop me. Back to the GP I'd go to see yet another GP and have to start explaining the whole damn rigamarole all over again.

I now don't need a stick to walk at least half the time. I sometimes need it because I'm tired. Sometimes I need it because a bone in my foot is out and it helps. Sometimes I need it and can't use it because my wrist is too painful or I've dislocated my finger. It's a tool. I use it when I really really need it. Increasingly I don't.

I've dropped 3-4 dress sizes. OT have had to cut an inch of my thumb splints because they were so big for me the velcro went over the edge of the other bit of velcro it was supposed to be meeting. My anxiety is under much better control thanks to the right anti-depressant. I'm getting out of the house a couple of times a week and managing to go for small walks. I'm still very tired a lot of the time and that's something that will need addressing if it continues but I've just changed anti-depressants and tapered of 30mg of Mirtazipine in a week so you'll excuse me if I'm a bit shot to pieces at the moment. I'm managing to attend a group workshop. I'm managing to do a little bit of housework.

Slowly but surely, I am getting better. Except this isn't of course, something you get better from. There is no cure. There is only management. But the team effort of all those lovely Rheumatology people at Blackburn Hospital has changed one really really fundamental thing.

I want to live.

I can never repay that. I can never adequately express what that means to me. So instead I have written an essay, because it is an essay of thank you. It is my story of EDS and me and it is only just beginning.

[To the Rheumy team: I know I've said my heartfelt thank you's to some of you and I know this post will embarrass you horrid should you read it but I felt that explaining what EDS care should look like might help those who seem to be struggling with it somewhat ;o)]