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Posts Tagged ‘ehlers-danlos syndrome

When I received my diagnosis of hypermobility syndrome in 2008 I began reading up on the subject. Back then, the only book available was written for physiotherapists, but I devoured it and experienced many moments of revelation. Since then, three more books have come on the scene – another aimed at healthcare professionals, and two which are a little easier to read. Here I review them all. There’s also a fifth book which is purely for experts. Bear in mind that I am reading from the perspective of a patient – my last experience with science was at A level!

Before I begin, it’s worth noting what the hypermobility syndrome actually is. Some people are hypermobile, i.e. their joints move further than they should, and for many of them it’s not a difficulty. When this hypermobility leads to a problem such as pain, it’s known as the Hypermobility Syndrome (HMS). This hypermobility is generally caused by collagen disorders such as Ehlers-Danlos Syndrome (EDS) and Marfan Syndrome. EDS type 3 is considered by many professionals to be synonymous with HMS – so you can see the overlap – and potential for confusion! These books are for everyone with Hypermobility Syndrome, no matter what the cause.

Bendy books aimed at professionals

Hypermobility Syndrome: Diagnosis and Management for Physiotherapists (Keer, Grahame) (2003)

This book was a great starter for me: it begins (as do all the books) with an introduction to HMS and the Beighton test for hypermobility. It is clear and easy to understand. Although this book is aimed at physiotherapists (and patients might want to skip over recommended exercises) it is easy to read and leads you through how each area of the body is affected. Sections about HMS in children, adolescents and then adults allow you to reflect on your life – it was full of eureka moments for me as I came to realise that my slow walking as a baby, my bad handwriting in school, and my frequently turning ankles, were all part of the same condition that caused my knee to dislocate.

It is well illustrated – full of pictures that I thought were normal; this led to adventures where I asked my husband to move his joints and realised how we differed! It was the book from which I learned that what was “normal for me” was not at all “normal”. The penny dropped several times as I read on. While that could have happened with whichever book I discovered first, I feel that this one is well set out and although aimed at physiotherapists it is easy to read, and calmly factual. My only issue is that this book addresses only the movement of joints; it doesn’t consider the other issues such as gut and bladder problems which may occur, and for that reason it’s best accompanied by one of the two books aimed at patients. It was, however, very useful to lend to my Pilates tutor so she could understand me and tailor exercises to my needs.

Most importantly, while this book taught me how I was different from normal, it didn’t make me feel that this was a problem.

Hypermobility, Fibromyalgia and Chronic Pain (Hakim, Keer, Grahame) (Sep 2010)

I bought this book because it had my consultant’s name on the cover; I thought it would answer everything about my condition. In some ways I was right; it’s incredibly thorough. However I realised it was a textbook when some sections seemed to have more pages of references than they do of content! It’s a great reference book and the chapter on the physiology of pain is well worth reading, if you can keep up with the terminology. This falls within the first section, on clinical science. The second section on therapy is much more accessible to the lay reader and covers everything from drugs to exercises and from CBT to sleep hygiene.

I’d recommend this book for the more determined expert patient, someone with a thirst for knowledge on their condition, perhaps looking for passages they can quote to their own doctors if necessary. However, I found it hard going in places and certainly not bedtime reading.

Hypermobility of Joints 4th ed (Beighton, Grahame, Bird) (Sep 2011)

It’s worth a quick mention of this forthcoming book, which will be the fourth edition of an existing tome. This publication is clearly aimed at experts, with topics such as “molecular basis of hypermobility” which suggests it is unlikely to be useful to patients unless they are also medical specialists! The high price also betrays that it is a specialist textbook – £90, with earlier editions costing well over £100. For that reason I haven’t bought it, and unless you are a healthcare professional (in which case, it’s worth noting that the authors are highly respected in their field) it’s probably not a useful addition to your bookshelf.

Bendy books aimed at patients

Issues and Management of Joint Hypermobility: A Guide for the Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome (Tinkle) (Sep 2008)

Tinkle’s book differs from the others in that it’s written by an American. When I mentioned it to my consultant he dismissively told me that it had quoted some of his colleagues without their permission, and discouraged me from it. However, I’d already bought the book so of course I would read it!

It is very straightforward, first explaining the types of HMS and EDS and then covering how each part of the body is affected. This means that it does not go into much depth in any section, but there is a broad cover of all systems in the body so it gives a good overview of how people with HMS may be affected.

I found this a very good book to highlight the areas where I was affected and lent it to my mother, so she would get a better understanding of my condition. It would be an ideal starting point for someone with HMS, or a good overview for the parent of a child with HMS, but for detail you will need to tackle one of the books aimed at professionals.

N.B. This book has now been superseded by Joint Hypermobility Handbook.

A Guide to Living with Hypermobility Syndrome: Bending without Breaking (Knight) (July 2011)

The narrative is a combination of Knight’s own experiences, and attributed explanations. This means the reader has to check whether a statement is a fact established by research, or Knight’s own opinion – for example, I’m still not clear on the origin of Knight’s belief that “people with HMS are more likely to experience fatigue … because there is at least twice the amount of effort required constantly just ‘to be’”. Each section is frequently illustrated by quotes from hypermobile people – most of which sound as if they came from messageboards, but are well sourced and relevant to the text.

This book openly tackles some of the issues of living with HMS which others don’t address (or only in a clinical manner) such as anxiety and intimate relationships. I was so pleased to see a book discuss how people with HMS fare in the bedroom – but disappointed that the section was coloured by Knight’s description of a failed relationship. The final sentence begins “I saw no point in continuing to live…” when I was hoping for encouragement!

Because of the mixture of Knight’s experience and that of others, this would make a reasonable first book for people who have just been diagnosed with HMS or a related condition. I think that in particular, teens and young adults would benefit from reading other people’s experiences and realising “it’s not just me”. The difficulty I personally have with this book is that many of the experiences differ from mine so they are not necessarily familiar, or helpful. Also, as I’ve been aware of my condition for three years now, I didn’t learn anything new.

Summary
If you are newly diagnosed then both Tinkle and Knight’s books complement each other and make a good starting point. Tinkle comes across as more factual while Knight shares experience. If you are a teen or young adult, and you want to feel you are not alone – try A Guide to Living with Hypermobility Syndrome. If you are moving past that point of initial diagnosis and would like to learn a little more about how every part of you could be affected – or you’re the parent of a child with HMS, looking for an overview of the condition – read Issues and Management of Joint Hypermobility; that’s the volume I would recommend if you can only buy one. However, neither book will break the bank if you want to give them a try.

If you are already familiar with your diagnosis and your body, and need more in depth information, then pick up Hypermobility, Fibromyalgia and Chronic Pain; it’s useful and very thorough, but more of a textbook and not easy to sit down and read cover to cover. If you consider yourself the “expert patient” then this could be what you need in order to quote to your medical professionals as you find your way through life!

It’s been an odd three months, since my dad died. I’ve been wearing painkiller patches which have turned me into something of a zombie, making me sleep 14 hours a day. Last week I ripped off the patches and for the last few days I’ve woken naturally at 11am – a great improvement! I’m getting my life back on track. That includes resolving to blog here more often, too! So…

Now I’m almost back to my usual everyday state, I thought that I’d demonstrate what the “everyday state” for someone with Ehlers-Danlos Syndrome can be like. It’s a condition that people know I have, but unless you’re also affected it’s hard to fully understand. Let me explain by making muffins.

It’s 4pm. I think it would be nice to make strawberry and chocolate muffins, a treat for both me and my husband. I find a simple “foolproof” recipe and pop to the corner shop to pick up some fresh flour.

Of course, I need a sit down after walking to the shop (a distance of about 30 metres), a little rest for my knees and hips. At about 430pm I gather myself and go to the kitchen. I put out the ingredients and begin chopping the chocolate and strawberries. The chopping takes me 15 minutes, and I get backache after standing for more than 5, so I’m hurting and sit down for a rest against a specialist supportive chair.

This is a good point to introduce the concept of “being a spoonie”. Christine Miserandino’s magnificent Spoon Theory describes the concept of spoons representing energy. You might keep a spoon in your back pocket, or borrow from tomorrow’s allocation, but you always have a limited capacity and whatever you do will have a physical payoff. Or as I always used to say before I had a diagnosis, “the more I do, the worse I am”.

After some painkillers and half an hour’s rest I felt able to go and blend the ingredients. It sounded simple, until I had to add melted butter. Hang on, where did the recipe mention melting? Now I had to find a pan! However, all went well until it came to stirring the final mixture. The recipe suggested it should be done by hand, to leave small lumps. My wrist didn’t feel strong enough to stir such a stiff mix and I had to wear a wrist brace to finish the job.

At last I could drop the mixture into cases and pop it in the oven (using two hands in case a wrist wobbled). I had a lovely 25 minute sit down which I needed to recover from 12 minutes mixing! And then, as 6pm approached, a wonderful hot chocolate and strawberry muffin to enjoy!

My husband arrives home and asks “What’s for dinner?” At this point I realise I’m exhausted. I’ve used all my spoons making the muffins, and don’t even have the strength to cook pasta. My back hurts from standing. My wrists ache from stirring. So, guess what we both ate that night?

That’s what happens when you’ve got Ehlers-Danlos Syndrome! It’s a genetic collagen disorder, meaning the collagen in my skin, ligaments, tendons, every tissue in my body, is too floppy. My joints bend in ways they shouldn’t and sometimes dislocate. My gut misbehaves. Everything aches, and some bits of me stab in pain. But hey, there’s no part of my back I can’t reach (to scratch) so that’s alright, eh?

A few days later I made these double chocolate muffins “to use up the ingredients”, and they were perfect. To anyone who has the spoons to cook, I recommend them. After all, when it’s a choice between muffins or dinner, you want the tastiest recipe!

Every week a lovely lady comes to my house, relieves me of two purple notes, and spends an hour talking me through physical exercises which must look bizarre to passers by.

For months I have wanted to improve my fitness, but I needed someone who would be able to understand my joint hypermobility and co-ordination issues. I have Ehlers-Danlos Syndrome which causes all kinds of problems, but mainly means that my joints are too bendy (some people think of it as being “double jointed”), my knees dislocate easily, and I have a lot of pain. I am naturally limited in many ways (for example, how far I can walk) but also have to be careful, even within my limitations, not to do myself damage or aggrevate the pain. Naturally, this makes it very difficult for me to get moving or tone up.

I wasn’t sure about Pilates to begin with. I’d read that it was the only form of exercise that “people like me” can do safely, so I asked my physiotherapist about it and she dismissively said that there was no point; basic Pilates would be similar to the kind of homework she was setting for me anyway. But when the physio department discharged me, I was left without guidance, and no way to exercise. Being overweight, and knowing I was quite unfit, this was something I wanted to tackle. Yet in my mind I associated Pilates with Yoga and other hippy type, faddish things. I had visions of people pretending to be a tree. Google didn’t reassure, informing me that the exercises had names such as “The Clam”, and that I’d have to do some kind of special breathing so that everything came from my “core”. Still, it seemed the only option available to me, so I spotted an advert in a nearby Vegan cafe, and made the call – to a friendly lady called Alison Bray.

The first thing my new tutor did was talk to me, learn about my impairment, and borrow my hypermobility for physios book. So she really wanted to help, rather than just take my money. She listened and learned. That was a good start.

Alison is slowly guiding me through exercises, and I’m enjoying the work. It’s much more cerebral than aerobics or going jogging – I’m having to remember how to breathe, and consider exactly which muscles to move, in a series of precise actions. I focus on relaxing my body and only engaging the few parts that are necessary for each movement. At first I found this hard – so many parts of my body wanted to join in! But now I’m getting familiar with muscles I hadn’t even realised existed.

Each week I mention any joints that are playing up, and we carefully work around them, but there are physical benefits for my condition too. For example, I usually wake up with pain across my lower back. Yet if I lie on the floor and run through a few pelvic lifts and some pelvic curls, it’s not long before I feel scrunching in that area – and then the pain is relieved as my bones move into the correct alignment. I know that the problem will return the next night – but at least I now have a way of shaking it out of myself.

The special Pilates way of breathing wasn’t so strange after all – given that I used to play trumpet I am aware of different breathing techniques, and I am reasonably strong around the middle since I have always enjoyed sit-ups. Once I understood what was necessary – how to harness the muscles in my centre so that they can stabilise me for other movements – everything made sense.

Unexpectedly, the most important part for me has been in training not my muscles but my brain. For example, I was asked to undertake an exercise where I moved my arms back, as if pushing on an invisible wall. The object of the exercise was not to work my arms, but my lats. Initially I thought “oh, that’s odd, I can feel something moving in my back!” Yet today when I was given a similar but harder exercise, I was able to relax my arms and to consciously tell my lat muscles that they were the part that had to move. And it worked. Two months ago, I’d never heard of a “lat”!

One of the symptoms of my condition is poor proprioception; if I was to try and run, I’d lollop around like a drunken elephant, and do myself damage in the process. But through Pilates, I am learning to instruct my muscle groups more specifically, and consciously, and to have better physical self-awareness. I think in the long run this is going to be very valuable. My brain is laying down pathways every time I repeat a movement. Slowly, I am taking control of my body.

It doesn’t matter how many aches and pains I feel before my tutor arrives, I always feel better after she’s been. Pilates is a calm, precise form of exercise and it seems to stabilise my joints without pushing things too far, and to work my muscles without overtiring them. Even better, time with Alison is an hour spent away from my computer, using my brain in another way so I can forget the world outside.

Pilates turns out not to be a hippy thing at all, but a wonderful way of harnessing your mind and body. If you’re looking for a safe, cerebral way to exercise, just find a tutor and give it a try. Pilates is changing me for the better, and I’d recommend it to anyone.

When I look in the mirror I don’t believe the figure smiling back at me. It’s only when caught unawares – such as at a service station toilet with full length mirror opposite the loo – that I am confronted with the fact that I have a spare tyre around my middle. Otherwise I would be blissfully unaware, and still expect to see a thin version of myself in mirrors. Although I’ve been fat for years now, it just doesn’t seem like me.

I know what size I am. I’ve been buying clothes of that size at M&S for years. But still when I unpack them, they seem too big. They fit me, but it always feels wrong.

I haven’t always been overweight. In my sixth form I was depressed and ate so little that my uniform had to be ordered in specially, a size 6. (For the men reading who have no idea what that translates to, think Victoria Beckham.) Through university I was a slightly more healthy 10 to 12. When I discovered eBay I was pleased to find that I was a normal, healthy size and I could fit into a range of gorgeous goth clothing!

Slowly, the weight has crept on. Mostly this came about once I started a full time job and could no longer attend gym classes several times a week. Exercise was on the backburner, but I thought having a full time job would keep me on my toes.

Once I recognised that I was overweight, I tried healthy eating and then even Alli, the weight loss pills that were promoted in every pharmacy when they became available over the counter earlier this year. The name is a misnomer (“friend” or “helper”) as any meal with more than 10-15 grams of fat resulted in what the manufacturers so nicely call “treatment conditions”. Basically if you don’t stick to a very low fat diet, you will end up farting oil – and nobody wants that. I stopped taking Alli, recognising that fat content wasn’t all of the story and that as a vegetarian, fat content in my diet was likely to conflict with Alli’s strict limits when I ate meals which were based around cheese – although it would still fall within the government’s “Eat well” recommendations. It seems that Alli helps you lose weight by terrifying you into not eating any food containing fat at all.


Flash in the mirror at a hotel, during 2010
Flash in the mirror at a hotel, during 2010.

There is an elephant in the room – my disability. I have a condition called Ehlers Danlos Syndrome, and when I was diagnosed, the first thing my consultant ever told me was not to go running or swimming. Straight away I wondered how to exercise, but I’d known for years that my knee dislocates easily and other joints behave strangely, so I am very limited unless I have a low-impact programme. Now that my weight has crept up (taking me to a size 18) it is even harder to exercise safely and within my limits, as aerobics teachers are unwilling to support me, not understanding my impairment or unable to suggest safe alternatives to the routines in their class.

I still look in the mirror in disbelief, not knowing how best to dress to disguise my curves and jowl. Size 18 may be just two inches larger than the average British woman, but that doesn’t make it acceptable – it just means that there are lots of fat people out there! However, even though I do want to exercise, I am restricted by my impairment to doing just sit ups and “chairobics” – a form of exercise I have created which lets me stay sitting down. Hopefully it burns calories as much as aerobic exercise, but I have no way of knowing, and I can only manage 20 minutes at a time which is barely enough to get the blood pumping. Still, it’s fun to dance in my wheelchair at parties, and other people will boogie with me, so I must be doing something right! But at home it’s embarrassing – I would hate anyone to see me doing my chairobic “dancing” to Blur and Ian Dury as I try to work out. I set an alarm on my phone each day to remind me to exercise, yet four days out of five I am unable to physically manage it, having to rest my knees when I would rather work off some fat.

Although I am working on my weight, it seems I really need to tackle my self perception – after all I know which dress size I buy, so I should be used to seeing a fat person in the mirror – but I’m not sure how to address this. It seems to be harder than dieting. There may be many recommendations on how to diet and eat well, but I’ve yet to see any suggestions on how to retrain your brain! I still don’t recognise the body staring back at me.

So I’m trying to find a way to shift the kilos, but as I already eat fairly healthily it will be a long process – there are few things I can cut out for instant wins. (I’ve already swapped from sugary to diet drinks, from regular to reduced-fat cheeses and from mayonnaise to natural yoghurt.) To keep my spirits high I simply remember how little I recognise my body when it is undressed – the curves which should not be there – and then I am ready to aim for the figure with which I actually identify, similar to my early university days, while recognising I can never recapture my youth or reverse the progression of my impairment. For starters, perhaps I should be proud that I have stopped the gradual increase in my weight and seem to be slowly reversing the trend.

It may take a long time until I am able to squeeze into smaller dress sizes, perhaps many years, but hopefully over the long term I will be able to lose weight, respect myself for the effort, and move forward. For now, my old dresses are still in storage – but at least I haven’t given up and thrown them away altogether.

Where can you find out about disability benefits? Where is it advertised, who tells you, and how are you pointed in the right direction when you start to find it harder to manage?

I am wondering where I first heard of Disability Living Allowance, the main benefit for disabled people. DLA is not means tested as it is intended to compensate for the extra living costs which are incurred by everyone who has additional needs, no matter what their background. It can be a lifeline, but nobody ever tells you that you might qualify, especially if, like me, disability has crept up on you slowly throughout your adult life.

I think I must have heard of it through my family – my brother gets DLA to help with / compensate for his learning difficulties, and I remember my mother urging me to stop being stubborn and apply for everything to which I was entitled.

At this point, I need to give you a little background. Some people are born with conditions which mean they are disabled for their whole life. Others might suddenly become disabled, for example in a car crash. I had a normal childhood, but at the age of 12 dislocated my knee. Since then things were not right, and grew worse – it’s hard to nail down, but I remember asking for a ground floor room in halls aged 18, as I was beginning to find stairs hard. I also recall boyfriends from my university years nagging me to carry a stick, as it was unfashionable but made my life easier.

By the time I received a diagnosis of Ehlers-Danlos Syndrome, a connective tissue disorder which makes my joints too loose, the horse had well and truly bolted. The stable door was irrelevant. I was already a wheelchair user and now disability was a part of my everyday life, although a diagnosis was useful in order to better understand myself.

My consultant kindly said he would write me a letter for DLA if it helped – but that was in 2008. By that time I’d been dislocating for 20 years, needed “reasonable adjustments” for 14 or so years, and had already been receiving DLA for 6 years.

I think Disability Living Allowance is a great benefit, actually. Of course it’s not enough to compensate for all my extra needs (it just about covers my cleaner dashing about with a hoover for a few hours, and a few taxi rides here and there) but it IS a recognition, and it is nice to get something which is not means-tested, acknowledging that our additional needs will have extra costs no matter how much or little we earn. I am lucky enough to have an indefinite award and the reliability of almost £400 arriving every 4 weeks is a lifeline, even though my husband and I are not borrasic. It means that whether I have been able to work or not, I still have some money to contribute to the family funds, and thus I still have my pride.

If only more people knew about DLA! Also, I am aware that I was extremely lucky to get an award that accurately reflected my needs, for an indefinite period, at first attempt. According to my disabled friends, this is very rare. Some apply but are turned down and don’t appeal, but many who do appeal will win. However, the award may be for a limited time, so the process is barely over before you have to re-apply and go through it all over again. It is shocking that disabled people, who have the least energy of anyone, have to jump through hoops and fight for their rights in this way.

Once I received DLA I still didn’t know about half the doors that had opened, even though many were automatic. After attending a focus group with other disabled people, I was inspired to create the website www.enabledpeople.co.uk, to let everyone know about other concessions and entitlements.  Sure, I saw leaflets about disabled railcards when I was in a station, but I didn’t know that if I travel in my wheelchair I can get 50% off the standard fare – no railcard needed. Funnily enough this isn’t advertised. Nor is the fact that you can travel for free on London Buses in a wheelchair. Plus of course cinemas, theatres, gig venues, festivals, and museums will not go out of their way to let you know that you can get a carer in for free. Why would they? All that lost revenue! But why should I have to find the energy to ask for a discount or ring around to find out whether they can accommodate my assistant?  It is incredibly frustrating!

However, I am very grateful for my DLA. It covers the cost of a cleaner and means that I feel some sense of pride in the house, because my cleaner is only doing what I would do myself, if only I was more able. I feel that I have contributed.

Disability Living Allowance is, as far as I know, the only disability benefit that is not means-tested. And although my husband earns a good salary, it is essential – it means I can justify using a taxi or hiring a cleaner, and so genuinely making my life easier.

And that, surely, is the point.

Spread the word!


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