Welcome to the Bendy Zebra Club!

Many of the people who follow me on twitter and on here will know that I’m not in the best of health. What many don’t know is in what way, what’s causing my problems and quite how much they have impacted my life. You see I have both syringomyelia and EDS, and best guess is that the latter may be responsible for the former though “who knows?!” as my neurologist once said. In truth I’ve been wanting to write more about them for some time, syringomyelia in particular is incredibly rare, affecting (according to 1966 study) 8.4 out of 100,000 people. The form I have affects less than one percent of cases. EDS is nowhere near as rare, but it is still very uncommon, or at least not commonly diagnosed.

Mild extension of wrist and elbow

A lot of people have great difficulty when it comes to getting a diagnosis, and I am no different in that regard. Many GPs don’t recognise the symptoms, and even a lot of rheumatologists are not well versed in it. This makes it being discovered as a cause for the problems a person may be having very difficult indeed, and many people that I know with the condition have had to do their own research and presenting it to a GP in order to get anywhere. This of course brings up it’s own issues, both surrounding self diagnosis and the potential for frustration if a GP still refuses to refer.

I’ve heard countless tales, and have my own, surrounding all of these problems. I also know a good number of people going through it right now.

Hyperextension of thumb and wrist

It’s not been a great day today; from the moment I woke up this morning I’ve been in considerable pain and oramorph doesn’t seem to be touching it; such is typical of EDS. With hyperflexible bodies and predisposition for frequent, if not constant dislocations comes a pain that few painkillers can do anything to dull. As neat a party trick as it is, a ‘normal’ body is certainly something I wish I had.
EDS is a syndrome caused by having collagen which didn’t form quite right, and I like many other people with this form of Ehlers, also have a natural and bizarre high tolerance to opiates, a difficult time with NSAIDs, and no other real choices.
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Fingers hyperextending at the knuckle joint

The photographs in this post are of me taken a little over a year ago. They’re some examples of hyperflexibility can come with EDS type 3, or joint hypermobility syndrome (I should be clear here, so as to not leave people worrying; you can have joint hypermobility without issues such as pain, dislocations, skin issues or other stuff. So if you recognise these examples in yourself it’s not necessarily indicative of EDS). Other things included in type 3 which are or have been present in my case are:

  • spontaneous or heavy bruising,
  • odd ‘scars’ on the skin which look like – but aren’t – stretch marks,
  • patches of skin which is velvety soft,
  • folds in the upper eyelids and at times under the eye
  • Scars leaving behind a silvery sheen and can take a long time to form,
  • cuts, while healing, can be very discoloured
  • Raynaud’s syndrome (incl. Livedo reticularis)
  • high palate of the mouth, causing small jaws and overcrowding of the teeth and migration
  • advanced early onset periodontitis, jaw infections and loose teeth
  • splaying of the toes
  • ankle supination or pronation
  • fragile/thin/translucent skin
  • A whole bunch of other shit

I have type 3, hypermobile type, and also thrown in for extra fun is some type 4 crossovers. I’ve had 8 teeth removed, with the remainder wired in place, orthotics to prevent ankles supinating, wrist supports, a bite riser to alleviate night-time jaw dislocations, I’m an expert at putting my own joints into place after dislocations, I’m covered in strange scars and marks that don’t necessarily have a cause, much of the skin on my body is not only bizarrely soft but virtually see-through.

Type 4, which I have very, very mild symptoms of, is commonly called ‘bleeder’ type. I bleed, oh do I bleed. I don’t have to apply pressure or even move to find myself covered in bruises, if I’m cut it takes a very long time to clot, and while I was fortunate enough to miss out on cardiac issues, I unfortunately got the menstrual ones.

I had to fight very hard to get a diagnosis at all. I’ve known for a few years I had it. One of my parents does, 2 of my siblings do, other relatives do.. It’s not rocket science to put the pieces together and finding they fit.

When the pain started to get beyond what I could handle I was around 9-10 years old. My knees had been ‘popping’ [subluxing] and I’d been seeing specialists for it at a local hospital, told that it was down to having ‘flat feet’ and ‘inverted hips’. By the age of 12 I was investigated for rheumatoid arthritis, only to have the specialist laugh when he saw me move and say ‘nope’ and suggest fibromyalgia. After a lot of fighting to get an answer, in the end I was misdiagnosed with fibromyalgia and M.E. Misdiagnoses that still get brought up with doctors today and it has impacted my care at times. There’s a lot of controversy surrounding them as diagnoses within the medical community still, and despite not being present they are still dredged up for reasons I cannot fathom.
I’m now 27 years old, my diagnosis was confirmed late last year by a rheumatologist trained by the incredible Prof G, the UK’s only EDS specialist. To get that appointment with her has taken me almost 3 years, and it took me listing my symptoms, looking at my scars, EDS lines, skin, eyelids and a few examples of extreme hyperflexibility for her to sign off on it. It’s been 16 years of fights and wrong turns, poor decisions, and most of all self-doubt. I’d asked several GPs, over and over again, to allow me to see a specialist only to be turned down. “Well they’d only offer physio and pain relief..” completely missing the fact that illnesses which are an enormous part of your life, which impact every choice you make out of self-preservation and manageability, are a part of who you are. The uncertainty in itself was a struggle. I’m a bendy, it’s a part of who I am and it will forever impact my decisions. There were also the cardiac investigations which may not have happened were I not to push, access to hydrotherapy and pain clinic, ongoing access to physiotherapy instead of a few terms of it.

It’s been a long road and a stressful one, and like so many others I am so relieved to discover that, despite all my self doubt, I wasn’t wrong. The pain, the dislocations, it’s all there and all related. It also ties in greatly with my neuro issues and could potentially give answers for Dave in future, but without the diagnosis it’s unlikely to have a correlation noted or taken seriously.

Splayed toes and translucent skin

Regardless of the state of my body and all it puts me through, I will admit that I’m proud of myself. No longer distracted by so much, and a fight in regards to health is so, so much, I’m able to look back and see all I’ve accomplished with it being there and affecting so much

I also feel incredibly lucky that, while going through a struggle to diagnosis and found out exactly what is wrong with my joints and body, as well as through the challenges of daily life, I have met such an incredible group of people. Other bendies, other activists, other people just like me. People who can joke about dislocations, laugh about reactions people give or pain or wheelchairs. Who I can moan about ableism to and they can share the same with me. It’s my journey with EDS, in part, which has caused me to meet some truly inspiring individuals who I gratefully call friends. And I will forever be thankful that I’ve had that chance to meet and share with them.

Edit: A note from a most wonderful sloth on why it’s bendy zebras

Because in the medical world, they are taught that if they hear clacking hoofs, they should expect it to be a horse. But occasionally, it’s not what they expected and it’s a zebra – Basically a rarity. So anyone with a rare condition is considered to be a zebra. The EDS community have taken on the zebra as a mascot as a result.

Revised 13/01/2016

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