This is reblogged from Book Frivolity.
This is not really book related, this is me related. It’s a cause incredibly important, and personal to me, so I thought I’d share it here. Kind of reluctantly actually, the whole idea of being so personal scares the bejeezus out of me, and I’ve mulled over whether to do this for days.. But here goes nothing..
So, today is Rare Disease Day.
And, I’ve just lost 95% of people’s attention.
Fair enough. I can’t dress a rare disease in funtastic cosplay for a selfie, I can’t turn it into a ripping fantasy novel plot line, I can’t make it into a hilarious Deadpool meme.
And, what does a rare disease matter? It’s rare, right?! You’ll probably never need to address it, it will probably never affect you directly, nor will you know somebody that has one. But you do. You know me. And I have a rare disease.
I have Parenchymal Neurological Behcet’s Disease, which is a subset of Behcet’s Disease proper. According to statistics, of the 24 million people in Australia, I am approximately only one of 116 in this country to be graced with it. So, I’m rare, bitches.
Let me tell you, I’m not fond of it. We aren’t having a hell of a good time together. It’s meaner than the popular clique in high school; except I don’t get to leave this disease, and recreate myself in post-raredisease college.. Because it’s incurable.
What I can do, is make people aware it exists. I can put some lights around it, spark em up, and let the world know “We Are Here” with big flashing strobes. We might be rare, but we will not be invisible.
If you’ve made it this far, you might be wondering what Parenchymal Neuro-Behcet’s disease actually is! I appreciate your curiosity, thank you!
Here’s the run down in technical terms from the all wise Wikipedia (or, you may check out the useful meme provided in the post for the tl;dr version) :
Behçet’s disease is recognized as a disease that cause inflammatory perivasculitis, inflammation of the tissue around a blood or lymph vessel, in practically any tissue in the body.
P-NBD main symptom is meningoencephalitis which happens in ~75 % of NBD patients. Other general symptoms of Behcet’s disease are also present among parenchymal NBD patients such as fever, headache, genital ulcers, genital scars, and skin lesions. When the brainstem is affected, ophthalmoparesis, cranial neuropathy, and cerebellar or pyramidal dysfunction may be observed. Cerebral hemispheric involvement may result in encephalopathy, hemiparesis, hemisensory loss, seizures, dysphasia, and mental changes including cognitive dysfunction and psychosis. As for the spinal cord involvement, pyramidal signs in the limbs, sensory level dysfunction, and, commonly, sphincter dysfunction may be observed.
Some of the symptoms are less common such as stroke (1.5%), epilepsy (2.2-5 %),brain tumor, movement disorder, acute meningeal syndrome, and optic neuropathy
So, yup. It’s all about the funz! Everyone loves a bit of Sphincter Dysfunction! Woot (or poot)! The basic gist, is that my blood vessels can become inflamed at any time, anywhere in my body. My body, actually hates itself enough to try and internally combust, without asking my permission first. Bad Rover! For me, Behcet’s likes to stick around in my cranial region, cartilage in my joints (chondritis) and GI system; but occasionally it likes to travel to new and exciting locations! It’s full of wanderlust! Others with Behcet’s, can have different problematic areas, but it can attack anywhere blood travels in the body. So, my toenails are safe-ish.
I’m lucky enough to be on a medication that controls the mouth and genital ulcers. You have no idea how good it feels to be able to sit without gingerly mincing about on a seat to find a position, that doesn’t make your undercarriage feel like razor blades are jostling for position in your underwear. No really, it’s a state of bliss!
The rest of it, is still very bloody messy. My brain looks like Swiss cheese (a zombie probably wouldn’t even attack me). My motor skills are toddler-esque, my muscles don’t work respond to commands, and sometimes I just randomly fall on my arse for the heck of it. It’s taken days to write this, because my fingers have a problem with authority. The headaches, are pretty much constant, and can’t be controlled without medication that makes you as dopey as a kitten rolling in a field of catnip. If you look closely at my blog, you’ll see evidence of the times I’ve tried to write when on it. So mostly, I just put up with them. It’s a decision I’d rather not have to make, neither option is a good one. If I can walk, I use a stick. If I can’t, I use a wheelchair. It’s a kind of ‘Wheel of Misfortune’. What symptom will you spin up today?’. And, there is no proven treatment.
Hopefully, one day, people will take enough notice of the disease, that the people with money to burn, will research it thoroughly enough, so that we at least find a real treatment. Or, a cure. *sings hallelujah chorus*
Cause we don’t have one. We’re medical Guinea pigs. We are poked, prodded, and tested by doctors that are as clueless about the disease as the general public are. I don’t blame them, they can’t know everything about every disease, and when there’s no awareness, we can’t really expect much more. I am lucky enough that one of my doctors (a neurologist that realised, gee, that’s actually not MS you have there) took the time to do some real behind the scenes research (a year of it!), or I’d still be in the dark about what’s wrong with me. Probably going a little (or a lot) nuts!
So many aren’t so lucky, and so many with rare diseases are dismissed out of hand, because they don’t fit the medical mold. Many suffer needlessly for decades without a diagnoses. And when they get one, there’s usually no proven treatment.
We are given drugs that may, or may not work, but fuck me, they have some wicked side effects that need to be endured to try to find that skerrik of relief; whether they are successful at quelling the symptoms, or not.
I have 6 different – ologists. 6 specialists, and I swear they communicate in smoke signals from building to building, so much information is lost between them. But, again, it’s not completely their fault; our community isn’t set up for people that have no proven treatment for their illness. We just get pushed from specialist to specialist, looking for the best way to mask the symptoms. (I have heard there are Behcet’s centres in other countries! I hope they are serving you well!)
And this, is why Rare Disease Day exists. Because only around 400-ish of the 7000-ish (yeah 7000!) rare diseases that exist, have PROVEN TREATMENTS (are you feeling me yet?) . That is not enough. It’s just not good enough. And it’s because, the 95% of the people that tuned out at the start of this post, tuned out… If nobody listens, nobody is listening. Hello! *waves* We Are Here! If the general public doesn’t care, neither do the people who can get this shit done. Why would they, if it’s low priority to the general populace. I wouldn’t mind a cricket stadium full of people wearing Behcet’s blue, but it’s not a reality that will ever come to pass.
We don’t really want your sympathy; we don’t need inspo-porn, we don’t need clickbait AMENS! Although your empathy is very much appreciated, what we really need is your help to spread knowledge, and awareness. A rare disease, does not suddenly make us want to lead unproductive lives, or be cut off from experiences that are taken for granted by others. Or be given quack suggestions on cures (A Quinoa and China seed diet ain’t going to cure encephalitis.. Sorry!). But that’s exactly what happens to a good proportion of us, because the real awareness just isn’t there.
What we need real, medically proven, empirically tested treatments. We want to contribute our worth, our talents, and our dreams to society. We can’t do that lying in bed with Sphincter Dysfunction (Woot Poot!) and a head full of inflamed brain tissue (a lot of this blog is actually written/spoken in that condition. It probably shows! Ha!)
Listen. Please. We Are Here. Help us get the word out that we aren’t going to lie down, and suffer. We are warriors, and a lot of us are going to cark it of old age before we see anything done to help our fellow sisters and brothers in arms. What we can do, hopefully, is help the next generation of Behcet’s warriors, and other rare disease superheroes, not have to fight so bloody hard through the jungle of medical misfortune. If we stand together, we can be noticed. By you, by the world, and by those that will be our ultimate saviors: doctors, nurses, researchers, clinicians, hospitals.
Here are some links. Please click on them, read something about it, become informed. Not just about my disease, but rare diseases in general. Donate something if you’re feeling extra generous, but the real key is knowledge. Do it, or I’ll haunt you when I die of meningoencephalitis (or being hit by a bus, whichever comes first). And I will be a roaring bitch of a poltergeist, trust me! Hope you weren’t sentimentally attached to Granny’s vase, cause it’s probably going to hit a wall when you least expect it…
American Behcet’s Disease Association
Behcet’s Syndrome Society (UK)
National Organisation for Rare Disorders
I jest a lot. I try to be as light-hearted about this as possible. But, In all honesty, no jesting, THANK YOU for reading. I’m pretty sure not many got this far, so you have no idea how appreciative I am of your time.
I thank you, the Behcet’s community thanks you, and all of those with rare diseases, thanks you! You rock!
I’d kiss you, but I get tongue cramps (true, it’s a really disconcerting feeling too.. ).
Love K.
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