Wishbone Day: Because Awareness Makes a Difference!

Yellow doesn’t suit me. As a child, I had a pale yellow hand-me-down sundress which, conscious of how awful I looked in it, I wore solely to spite my mother. Vanity having surpassed mischief, I’ve rarely worn yellow since. However, tomorrow I will make an exception to the rule. On 6^th May, I wear yellow as part of Wishbone Day, an international day of awareness of brittle bones.

Brittle bones, or osteogenesis imperfecta (OI), is a genetic collagen disorder whose most commonly recognised symptom is that it makes bones particularly fragile and prone to fracture. This, however, is a bit of a simplification. Collagen, a doctor once explained to me, is the ‘scaffolding’ of the body, essential for the structure of all muscles, ligaments and tissues. This means that any part of the OI body can be affected, causing a wide range of conditions varying from chronic dislocation and scoliosis to hearing loss and respiratory problems. Living with OI is not dissimilar to travelling around London during a Tube strike: carriage is slow, unreliable and prone to stopping unexpectedly without reasonable explanation.

So, why is it important that you know this? Because awareness makes a difference.

OI is a very rare condition, affecting approximately 1 in 15,000 people in the UK. This statistic covers a wide range of types and severities, from Type I, which can be so mild it is never diagnosed, to Type II, which is lethal. Some of us can run. Some of us can’t stand at all. The majority of doctors (let alone council staff, social workers and benefits advisers), do not understand OI, and the consequences of this lack of knowledge can be devastating. It is mildly irritating when a new GP, looking through my notes, comments, ‘ooh, osteogenesis imperfecta, eh? So, what’s that?’ It is annoying when A&E doctors fail to spot fractures until weeks after the injury, because my x-rays are difficult to read and the surrounding tissue rarely swells or bruises. It can be incredibly dangerous when a person with OI is given incorrect treatment by doctors who simply do not understand how our unique bodies work.

But I cannot imagine what it must feel like to have your child taken away from you because its bones keep breaking without explanation and nobody has worked out why. This is the tragic reality for many families of OI children, whose injuries are assumed to be evidence of abuse and who are often put up for adoption. Even if OI is later diagnosed, some parents will struggle for years to regain custody of their own children, and to have their names cleared. In some cases, parents will never get their children back at all.

Living with OI is never going to be easy. Just yesterday, my mum (who also has OI but hadn’t broken anything in years,) fell over and smashed her left arm into pieces. She is currently awaiting surgery. It is unclear whether she will be able to use her left hand again. Until scientists come up with a cure for OI, incidents like this are unfortunately not unusual. In the meantime, it’s not just up to the doctors. From teachers, to shop assistants, to the kind strangers who helped my mum yesterday, a little awareness at every level and in every situation would make a huge difference to the lives of those affected by OI. That’s why Wishbone Day isn’t about raising money. It’s about wearing yellow, and celebrating the diversity of the nutty but fabulous OI community, and telling as many people as possible along the way. Please wear yellow tomorrow, and please feel free to share this post (or any other Wishbone Day paraphernalia!) as widely as you can. Because awareness makes a difference.

So Whose Fault Is This, Anyway?

Waiting outside Addenbrooke’s hospital on Tuesday, after yet another metabolic bone specialist had been unable to explain the excruciating pain in my previously ‘good’ ankle, it was hard not to want somebody to blame. The latest specialist had been kind and honest, but still I blamed her for not being able to help. If only she had known what was wrong, there would, I was sure, have been some treatment, cast or surgery which could have fixed me. So I blamed her, in the same way that I so often blame my back pain on my spinal surgeon, or my leg pain on the various doctors who, two years ago, did not spot a severely stress-fractured talus before it was too late.

The objective truth is that I have a rare bone condition which, due in part to its coincidence with other medical conditions, is currently presenting itself in a very unusual way. Osteopenia (low bone density) means my x-rays are difficult to read at the best of times, let alone when I have no clear break, only two unidentifiable white patches around the ankle which match the area of my pain. As much as I wish there was a simple answer, a definite solution, to each of my weird and wonderful complaints, the fact is that there was no clause in Tuesday’s specialist’s medical training which taught her to deal with whatever I’ve got going on – and nor could there have been. My body has spent much of its short life surprising experts by playing unexpected tricks at unexpected times. It is not the specialist’s fault that I will now spend weeks waiting for a CT scan, followed by another few weeks’ wait to get the results. Realistically, even when I do know what the problem is, there is unlikely to be a clear, easy solution.

None of this is my specialist’s fault and, until recently, I would have claimed that none of it was my fault, either. However, Tuesday’s appointment also revealed another home truth which I had been attempting to deny.

Since the age of nine, I have lived with anorexia, in a variety of forms and severities. I’ve had phases of eating almost normally, pushing guilty thoughts to the back of my mind, and I’ve had phases of living on two or three meals a week, spending days in total starvation. Twelve years on, my Eating Disorders Unit have teamed up with my bone specialist. Noting my weight gains and losses in correspondence with my Dexa (bone) scan, Tuesday’s specialist declared that there was no doubt in her mind that my current fracture cycle is a direct result not only of osteogenesis imperfecta, but also of prolonged malnutrition. My legs are no longer strong enough to walk on, and I’ve almost certainly done this to myself.

The news was hardly a surprise. For years, doctors have told me that my eating patterns would ultimately have a serious impact on my already brittle bones. From an anorexic perspective, convinced food was for the weak and that anyone who thought it a necessity was a fool, this was an easy claim to dismiss. Now, the only long-term medical prediction I’ve ever really been given has come painfully true.

It’s difficult when there’s nobody to blame. It’s even more difficult when the only person who deserves blame is yourself. Ironically, my anorexia is rooted in the desire to control my uncontrollable body; when my body has been particularly painful, the anorexia has worsened. I know this ought to be a wake-up call to put weight on, but I am torn between the desire to get better, and the illogical yet total conviction that everything would be better, if only I was thin.

I have been in charge of my own healthcare for a long time, and much of the past four years has been spent researching and organising referrals to the best specialists I can find. It is only now that I am beginning to accept that, with the best will in the world, even the greatest experts with the most cutting-edge treatments cannot ‘fix’ me. It’s not enough to turn up to every appointment and take every drug. Health starts from the inside out. I was born with a body which doesn’t work like it should, and it’s up to me what I do with it.

Let me tell you, it’s a lot easier said than done.

Let’s Talk About Pain

I know that, pain aside, I am incredibly fortunate in almost every other respect, and that I could be a lot worse off. I also believe that chronic pain is widely misunderstood. In the name of increasing understanding, this got a bit whiny; please excuse me.

What’s the first thing you think of when you wake up?

A close friend asked me this a few weeks ago. I chuckled a bit, and began to mumble something inane, when I stopped. This was a close friend. Why couldn’t I be honest?

‘It sounds a bit depressing,’ I said, ‘but pain. I think of pain.’

I live with chronic pain and, time spent under the influence of drugs or alcohol aside, have done so for as long as I can remember. Call me naïve, but until the age of eleven or twelve I genuinely believed that this was the case for everyone, and that my own pain was just a bit worse than other people’s.  My pain is located in my legs, feet and back, and fluctuates in severity from a nagging ache to crippling agony. On a good day, I barely think about it. On a bad day, I can think of nothing else. Sometimes, a bad episode is self-inflicted; I might have walked too far, or slept in an odd position, or done too much exercise. More usually, it is spontaneous. On any given day, the severity of my pain dictates almost every decision I make, from the clothes and shoes I wear to the places I go. Despite this, chronic pain is something I rarely discuss except when amongst close friends and family. I consider myself a pretty open person, happily broadcasting to the world my latest fracture or diagnosis. But chronic pain is different.

In my experience, discussion of chronic pain will generally provoke three forms of response. The first is a frustrating attempt at empathy; with all due respect, claiming you know how I feel because you once broke a toe is a bit like me claiming to know what it’s like to be blind because I once wore a blindfold. Even more frustrating is the second form of response: ‘there must be a solution, you just haven’t looked hard enough.’ A taxi driver once told me he was convinced I would be cured of all pain if only I tried googling my complaints. A friend of mine, (worryingly, a medical student,) was incredulous as to why I didn’t ‘just take painkillers.’ The amount of time, effort and money my mother and I have spent on attempts to cure my pain does not bear thinking about. I’ve tried yoga, meditation, Alexander Technique, cold baths, hot baths and circulation therapy. I’ve tried every kind of medication, pain management technique, diet plan and weird herbal concoction I have ever been recommended. I’ve tried steroid injections and surgery, healing crystals and prayer. Few were in any way helpful, and some made things significantly worse.

Sunshine and a strict physiotherapy regime make my pain marginally more manageable. Alcohol and/or drugs will sometimes ease the pain altogether, but the strength of painkiller needed for that is simply not compatible with leading a functioning, fulfilling life. Yes, I’m sure morphine was great fun that time you had your appendix out, but try writing an essay or going to work while you’re on it and you’ll see what I mean. In fact, I’m lucky that, when I’m desperate, morphine still does the job. There are many people whose chronic pain is so severe that no painkillers exist to help them. The speed with which medical science is advancing is incredible, but human understanding of pain is still lacking, and there is no such thing as the miracle cure of which my taxi driver was so convinced.

The third response is awkwardness, because chronic conditions make people uncomfortable. Tell a friend you’ve caught a cold, broken your arm or need your tonsils taken out, and they will (hopefully!) tell you to get well soon. It’s easy enough to say, and both parties will feel better for the sentiment. But what do you say when the prognosis is chronic and likely to get worse?

The truth is, a little understanding goes a long way. Chronic pain is usually invisible and always subjective, which makes it easy for others to forget about. A person in chronic pain might take longer than usual over certain tasks, or might need a bit more sleep than most, or might not always be able to go out when they want to. Knowing that your own body can’t keep up with its owner is immensely frustrating, but knowing that others understand why you are the way you are is a huge relief. My pain is constant, but with a little understanding and a helping hand when needed, it does not have to define me.

Please click here to read the Spoon Theory, a really great explanation of life with a chronic condition. I don’t have lupus, but I do have spoons.

The ‘Social Model’: Why I Am Not the Problem

It has been just over three weeks since I learned that I will almost certainly never walk again, and I have been overwhelmed by the gestures of kindness and messages of support I have received. Friends both at home and at university have been fantastic, and I am so grateful for that. One thing that has struck me throughout this time, however, is the language within which some of these messages have been phrased: it is a language of loss, of bereavement. This, it seems, is symptomatic of societal attitudes not just to disability, but to wheelchairs in particular. As Daily Mail accounts of freak accidents and injuries go, losing the use of your legs is often right up there with brain damage. I will admit that, as someone whose legs have always been a bit dodgy anyway, using a wheelchair is easier for me to handle than, say, a former able-bodied athlete or soldier. Still, this is an attitude which seems to attach unnecessary melodrama to an event which, with the right adaptations, need not be a hindrance.

Accepting the permanence of my wheelchair has been a massive blow, but it is not a death sentence.

That said, the availability of ‘the right adaptations’ can rarely be assumed. The bane of the life of a wheelchair user is often, quite simply, stairs. This brings me to the social model of disability, a theory which claims that a disabled person is not disabled in themselves, but by environments which are not effectively suited to their needs. As a young deaf teenager, this was a theory I found frustrating. Sound is not a man-made construction, but a sense which the majority of humans are naturally adapted to use. My audiologists, hearing support workers and sign language teacher were all fantastic but, with the best will in the world, they could not replicate for me a world in which sound was not something on which I felt I was missing out. Likewise, even in the most well-adapted, wheelchair-accessible of situations, chronic pain remains the greatest obstacle in my efforts to lead a successful, independent life.

I maintain that the social model does not apply to every aspect of every form of disability. However, with regards to mobility impairment, it is a theory which can have hugely positive ramifications both for the mobility impaired individual and for wider society. Dealing with inaccessibility on a daily basis is wearing; I am fortunate in being both sufficiently petite and willing to be carried up and down stairs, but waiting to be carried, pushed or otherwise assisted, I often feel like a burden on my family and friends. On a bad day, I wonder whether I will ever prove myself deserving of this assistance, or whether I will spend the rest of my life reliant upon the tireless and entirely unwarranted kindness of friends.

The truth is, I’m not the burden: stairs are. When the lift up to the University Library packs in because there are leaves trapped in the gate at the top, I am not the problem. When staff at Vodka Revs take away my wheelchair on the grounds of health and safety (?!) I am not the problem. When I find myself stuck in my parents’ home, unable to leave the house unassisted, I am not the problem. Living in Cambridge, where I enjoy the comparative luxury of two sets of electric doors and a ramp into my flat, where streets (aside from the cobbled ones!) are largely flat and where public transport and its staff have never once failed me, I am utterly independent.

I am not inadequate. I am just unusually appreciative of a well-maintained lift.

Please follow this link for a lovely little animation explaining the social model.

For the past three years, my life has been geared around one main goal: to walk to Cambridge University’s Senate House on graduation day 2014, and graduate. Three years ago, my biggest challenge was the work required to pass my BA, which then seemed almost fantastically insurmountable. Over the past year and a half, however, another aspect of the day has become the greater challenge: to walk the 150 yards from St John’s College to the Senate House. Eight months ago, flicking through photographs of the sunlit, beaming faces of friends in the year above on that infamous walk, I promised myself that, however much physiotherapy it might require, I would follow in their footsteps.

Yesterday, I sat in my orthopaedic surgeon’s consulting room and accepted that, whatever happens on graduation day this year, I will not be walking. Scrolling through a recent MRI scan of my right leg, he traced with his finger the white patches which criss-crossed the healthy black. ‘Your bones,’ he said in amazement, ‘are crumbling.’

The Royal National Orthopaedic Hospital in Stanmore is an incredible place, and they are very kindly funding – at great expense, with a generosity many hospitals would not permit – an ‘Exogen’ ultrasound machine, which will hopefully heal the worst of my existing leg and foot fractures. All my life, the healing of an injury has precipitated frantic encouragement to start weight-bearing as soon as possible, however much it hurts, so as to increase bone density and muscle mass. It is a catch-22 situation: bone density and muscle mass are essential to prevent further fractures, but the stress of weight-bearing might cause further fractures itself. The fragility of my bones has now tipped the balance; walking is no longer an option.

I cannot count the number of times I have learned to walk. I also cannot count the number of nights I have spent in sleepless agony, because walking is something my body is simply not designed to do. ‘Giving up’ walking feels like a heart-wrenching waste of the years my doctors, surgeons, physiotherapists and other specialists – as well as my family and I – have invested in getting me walking. Yet it is also a great relief. I have been using a wheelchair more-or-less full-time for a few months now, and the pain which I have felt in my knees, legs and feet for as long as I can remember has almost completely disappeared. I am sleeping better, and my mood and concentration span have improved dramatically. Once my current fractures heal, there is a real possibility my legs might never break again. My life is no longer in a state of flux, prefixing every long-term plan with ‘If I can walk by then…’, working constantly towards increasingly unreachable goals. More importantly, my day-to-day plans will no longer be dictated by the short distances I can walk. I can wheel faster and further than I could ever walk. The complicated logistics of life in a wheelchair aside, my horizons have only broadened.

I am fortunate to count amongst my friends not only the black-robed graduands who walked to the Senate House last summer, but a number of fabulous wheelchair-users who are living proof that a wheelchair is in no way a barrier to success. I will not be walking to the Senate House this June. I am learning to accept that such an ambition, the product of a society structured around able-bodied norms and values, was never that important, anyway.