06 May 2011

♫...So scared of breaking it that you won't let it bend...♫

There's an awareness day for everything these day. And so today is that day for my mobility impairment. Today is Wishbone Day, raising awareness of osteogenesis imperfecta.

There are lots of mythconceptions about OI around. Most notably the lies peddled in the dreadful M. Night Shyamalan film Unbreakable. Despite it being 11 years old, and you'd think most people would expel a film that dire from their memory, I still get people saying "oh, like in Unbreakable?" When they find out I have OI.

No. Not like in Unbreakable. For starters when Samuel L Jackson "explains" OI in the film he claims that type I is the mildest and it's a spectrum through to type IV which is the most severe and usually fatal at birth if not before (no mention of types V to VIII but I think at least some of those hadn't been discovered then). I am a 31 year old type IV. My mum who was also a type IV made it to 70 before dying from her familial predisposition to drop dead from heart failure at a reasonably young age. OI was not a contributing factor in her death.

It's actually type II that's fatal. Type III is usually pretty severe. Type IV varies from very mild to almost as severe as type III. Type I is different to the other 3 well established types. Bone breakage in types II, III and IV is the result of poor quality collagen. Collagen being the protein that provides reinforcement for bones. Type I is the result of a lack of collagen rather than the right amount of crappy quality collagen so it's quite different to types II - IV. There are type ones around who've had more fractures than me so to claim it's the "mildest" type is untrue.

There are many other inaccuracies in the film. By casting a non-disabled actor to play a person with OI he was, unsurprisingly, lacking all the physical characteristics of the condition. Elijah says he's had about 50 fractures; roughly the same number as me. Medical care of people with OI is constantly improving and Elijah was much older than me so he would've had worse medical care than I did when I did the bulk of my breaking as a child. Between not being very sturdy to begin with and then being constantly broken; bones with OI tend to bend. The bones in my right forearm have a bend in the middle of about 80°. The bones in my left leg are bent to about 30°. Had a I received the medical treatment a child with OI would receive today my bones would be less bent. They probably wouldn't be as straight as a person without OI's, but they'd be straighter than they are.

Elijah, being roughly the same as me in severity, should have bent bones. Bones bent more than mine because even less effort would've gone into straightening them. Samuel L Jackson not having OI has perfectly straight bones. Total credibility fail.

The other glaringly obvious characteristic fail is the eyes. Because collagen is the protein that makes the whites of your eyes white, most people with OI have blue scleras. Most, but not all. However, the people that do have white scleras are not type ones. Types ones always have blue scleras (like I said, it's different to the other types). SLJ does not have blue scleras. They could've made his scleras appear blue of course, in many films and TV shows you have demons and other monsters with completely black eyes. Tinting SLJ's scleras wouldn't have been too hard, whether through contacts or SFX in post-production.

So, yes. My impairment is nothing like Jackson in Unbreakable. Also, spending a lot of time in hospital as a child doesn't turn you into a terrorist.

Unbreakable isn't the only film to peddle poor facts about OI. Amélie is considered by many to be a piece of loveliness. It made me want to punch my TV screen. Amélie's neighbour with OI lives in a padded flat. Though perhaps I could do with his padded tellybox so that if I did end up punching it in frustration I'd be less likely to break my fist.

People with OI do not live in padded houses. There's no point. Breaking your bones is such a random thing. I've fractured my spine while sleeping on something soft and padded whilst conversely I've gotten drunk, fallen down a flight of steps in a club, and not broken a thing. Over-cautiousness can actually result in a reduction in bone density so living in a padded house can make you more, not less, likely to break.

I'm a big fan of forensic TV shows so obviously I watch Bones. OI has cropped up a couple of times, once just as a mention with regard to some ancient bones that Brennan had been looking at. In the episode "The Truth in the Lye" in season 2 they found some bones in a bathtub on a building site. Brennan looked at the bones under a microscope and noticed the lack of collagen.

The big fact fail here was with regard to reproducing. Bones said that in all the other types OI is the result of a spontaneous mutation but sometimes it's inherited in type ones. Hello! Type IV child of a type IV right here! And I'm not unique, I know tonnes of types IIIs and IVs who've had kids. When a person with OI has a child there's a 50% chance of the child inheriting it. Yes, OI is often caused by a spontaneous mutation. In the case of my family my mum was the mutant and I inherited it from her. But claiming that it's only type ones that breed is ludicrous.

The most common storyline when OI crops up in film and TV is that of parents being falsely accused of abuse. It's been done in Home & Away, ER, Casualty and many more shows.

It's an incredibly serious issue and unlike the other OI storylines I've mentioned it's one that's usually fairly realistically represented.

The Home & Away storyline was in the days when Pippa had a house full of foster kids. She fostered a child who'd been taken away from his parents because his recurrent broken bones had led to his parents being accused of abuse. Whilst in Pippa's care he broke his arm. Pippa took him to the local hospital where he was treated by a doctor who didn't sleep through the lecture on OI in her medical training. She diagnosed him and he was returned to his parents.

In ER Carter treated a kid who'd been brought in with a persistent cough. A chest x-ray revealed numerous broken ribs. Carter ordered full body x-rays and found that the kid had old and healed, currently healing, and fresh fractures all over. Carter called the police and social services claiming the mother had been beating the child. Carter performed a lumbar puncture on the baby and then took some more x-rays. Looking at the second set of x-rays Carter noticed that there was a new rib fracture not on the first set of x-rays and realised he'd broken one of the kid's ribs whilst doing the lumbar puncture. This resulted in him diagnosing OI and calling off the hounds.

In real life however, these stories don't always have a happy ending with the kids being returned to their parents. In some cases the children end up being adopted before diagnosis and adoption orders cannot be undone. Even when the child is returned to the parents it's often after the psychological scars of the child being fostered have been inflicted on both parents and child.

These days there are many diagnostic tests available for OI, there's no need for families to be destroyed. But it still happens because doctors don't think to check for it and social workers - in this target-driven post-Baby P era - don't mention it because they're desperate to whisk kids away from parents to meet their targets for kids fostered.

Wishbone Day could be a wonderful way to target doctors and social workers, to make them aware of OI, the clinical presentation, how to differentiate it from abuse, and how to test for it. But from what I've seen that level of awareness-raising aimed at the people who need it isn't happening. It's seems the thrust of Wishbone Day is "wear yellow and do the Facebook status meme," which isn't going to raise awareness amongst the people that need to be made aware. I'm aware that even this post picking apart media myths of OI is slightly redundant because it'll mostly only be read by people that know me so aren't gonna be taken in by Shyamalan's bullshit. So I'm being no more helpful to the cause than those who are simply changing their Facebook status and I throw my hands up and admit that. We need awareness targeted where it matters: A&E staff and social workers. Until that happens I think Wishbone Day is a bit wishy washy.

6 comments:

  1. I keep seeing these things, like "awareness" is some universal good that has become terribly fashionable and often without a central theme or point.

    "Breast Cancer Awareness" - well what is that? Who lives in a country with universal free primary and secondary education is unaware that breast cancer exists? I don't think it's a big audience. What you want is some central thing you want people do DO about that - do you want them to be more aware by examining their breasts properly? Do you want them to be more aware by reading your literature? Do you want them to be more aware that it can happen to men? That it can happen to very young people? What *action* should stem from that new awareness?

    Rare conditions are the most likely to be the target of awareness campaigns as people find out about a new thing and feel that as they didn't know it existed they were tragically unaware and must raise some awareness. To what end? Is this your target market? Once people have become aware that, say, EDS, ME, etc. exists, what should this change about their behaviour, attitude, charitable donation habits?

    I am not against awareness, but every campaign needs a central theme and a call to action.

    This is my one-woman awareness awareness campaign. Please be aware of awareness and ensure that if you are part of any awareness campaign that you have properly teased out what you wish people to become aware OF and what they should DO.

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  2. Oh Unbreakable! You found me because of that movie!

    As someone with Type I, I often believe that it should be classified as another disease. Yes, I break bones (because of shitty collagen) but, for a different reason than you. IDK, that's just me. I guess because some of the characteristics are the same but, I feel like a fraud.

    I once watched an episode of Extreme Makeover Home Edition where they re-did the home of a kid with OI (I don't remember what type). They padded the hallway. That just insulted me, "Do you think we bounce around down the hall?? DO you think padding will make us not break??". They put a bunch of soft bean bags and pillows in his room for him to lay in. MY joints screamed at the thought of him trying to get himself out of that without something slipping out of joint.

    How about they give him tons of hay fever medication so he doesn't sneeze, and a maid so he doesn't have to touch anything. Oh I know, put him in a padded bubble.

    And on the bent bone thing. I have a dent in my shin from being kicked in the leg (with cleats, don't ask). I don't know if I broke but, it sure is dented in and, it hurts if I push on it (THEN DON'T PUSH ON IT!)

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  3. I actually didn't know much about the different types, so you've educated me if no one else. I've managed to miss seeing 'Unbreakable', so there wasn't so much in the way of misinformation to undo, but having whinged about the extremely weird set of symptoms 'House' tried to pass off as EDS a couple of weeks ago I can see how you might be more than a tad peeved!

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  4. I knew that Unbreakable, like many other films that involve disability had at least some facts wrong but I did learn some things from your post!

    It's horrible that kids are still being taken away from their parents without being tested and I had no idea that it could take so long to find out (after adoption) or that adoption orders can't be reversed!

    You are absolutely correct when you say that A&E (Emerge., ED, ER etc.) staff and social workers need to know about OI. Unfortunately, this could take a massive amount of effort on many people's part to happen.

    ReplyDelete
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    ReplyDelete
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