Retinitis Pigmentosa Awareness Month
If there’s one thing I’ve wanted more than anything else in my entire life, it’s been to be normal. That may be hard to believe for most people in my life, given that I’m prone to living in a manner some might describe as dramatic and “unnecessary”. Maybe a part of me realized a long ways back that on some level I was never going to blend in, that I was never going to be quote unquote normal. Growing up, I was never normal. I was the kid with the big hearing aids, the kid with the lisp. When I wasn’t the deaf kid, I was the twin who ohmygod shared a womb with his sister “and isn’t that awesome do you guys share a psychic connection what’s she thinking right now?” (As we’ve grown up, I have developed a keen sense of her moods - right now, she wants wine and carbs). Neither of those things that made me different were inherently bad - they were just things that I didn’t have control over. I’m big on being able to control things in my life (more on that later).
Then of course, when I had maybe just become as normal as I’ve probably ever been in high school, where my identity wasn’t wrapped around my hearing aids or my womb-mate since my teachers weren’t all aware of either, I tuned into my emerging sexuality. So then I became the gay kid. I couldn’t control that, either. But if I wasn’t going to be able to be plain jane normal, I was going to control the narrative the best I could. So I was the school faggot who wore six inch stilettos to class because fuck the patriarchy, amiright? I mean, if I wasn’t going to be able to be normal, I might as well be balls-to-the-wall obnoxious. I sure wasn’t going to blend in with the jocks or the gamers or the preppy girls, so there I was making questionable life choices in zebra print jeans and a leopard print purse. Still, somewhere underneath three inches of caked-on foundation and bleached, blue, green, red, black, damaged hair, all I wanted was to be normal. I clearly wasn’t motivated to put in an effort to make myself normal, because I have all the self-motivation of a sunbathing iguana, but if I’d have woken up one morning with some innate ability to fly under the radar, my dreams would have come true. Nobody said teenage me was good with logic, alright?
Because none of the above were labels enough and because we live in a society where we worship excess, I further became the kid with the eating disorder, the kid whose life basically just frayed at the seams until I was spending my eighteenth birthday in the hospital, where surprisingly, I kind of felt…normal? I mean, not one of us there was normal - we were all twisted by some unknown force, hiding mashed potatoes in uggs, pouring shots of ensure in the carpet, and splashing urine samples on the poor nurses (poor woman coincidentally retired that same year). We had fixations that bonded us, a common experience in the ridiculousness of being told to lick out jam packets and eat the cores of apples (Laura, I’ve been recovered for a while now and you’re still just wrong on that front). I fit in without trying, and it was oddly comforting. When I left treatment, my life became as normal as I imagine it will ever be. I worked two jobs, found myself in a relationship that didn’t careen off the tracks (bless Joey’s heart), and then went away for school. It was the Canadian dream, and it was wonderful. I adored it. I mean, my roommates in Ottawa shat on the floors and made hash with my hair straightener, but I was normal! It was everyone else who was crazy - a true revelation.
Fast forward a few years and we get to the point of all this rambling about my life story. I was studying (lol) at Ryerson and I’d met fellow normals who I did normal university student stuff with like the wonderful normal me wanted. Except my one friend kept telling me I needed to get my eyes checked because apparently I couldn’t see well enough. Like, excuse me? I could see just damn fine thank you it wasn’t my fault other people got in my way or things moved into my path or that cars didn’t pay attention to pedestrians or that sometimes it was just too damn dark outside, okay?. “But no seriously”, Mathew said, “you should be able to see more. It’s probably like glaucoma or cataracts or something, just get your eyes tested”. The man works for Lasik MD, so I immediately heeded his words of advice…like nine months later.
It was now October of 2015, and I was normal, god damn it. I sat with patron saint Joey in an eye doctor’s office with a Louis Vuitton print trash can (I couldn’t make this up, I swear) and a poster of Patrick Dempsey on the wall. “MCDREAMY LIVES” I believe was what Joey said to me. This was a real eye doctor, not just some man my mother, bless her heart, was harboring a not-so-secret crush for. I sat through what seemed like a ridiculous number of tests that featured some eye drops that truly made me unable to see - I haven’t sent so many misspelled texts since the drunken lead up to my hospitalization.
My first sign that “whoops, maybe Mathew was right” was when I was doing a field of vision test where I was supposed to be pressing buttons whenever I saw a flash of light - the visual version of a hearing test I’d been doing since I was like four and screaming into a microphone at some poor audiologist who deserved better in life because he wasn’t MY audiologist. I digress. Anyways, the lady running the test kept telling me “so you need to press the button when you see the light. Do you see the light? You have to press the button! So you know to press the button? Press the button!” In my mind I was cussing her out - “GURL I AM DOING THE BEST I CAN I HAVE PRESSED THE BUTTON A MULTITUDE OF TIMES I HAVE SEEN LIGHTS” - but in reality, “yes. I understand. Gotcha. *click*. *click*. Want to feel like an imbecile? Sit in front of a machine with your face in a cage and listen to someone dispassionately remind you that you need to press the button blah blah blah.
Finally at long last, Gentle Male Eye Doctor (GMED) sat down with me to discuss the results of the test I had clearly failed like I had grade 11 chemistry. “So, you see this picture here? That’s your retina. These little lines here are *eye doctor jumble I could not reproduce for the life of me*. Your visual field test reinforces my first thoughts when I looked at these images”. Sure, GMED, tell me all about my cataracts so I can get Mathew to get me a sweet discount and I can get back to my normal life. “It looks like you have retinitis pigmentosa”. Screech. Hands up, y’all, if you’ve ever heard of retinitis pigmentosa before. Zero? Good, we’re all on the same page here.
“It’s a genetic degenerative condition”
“I’m guessing from the look on your face, that’s not a good thing?”
“So how do we like, deal with it?”
“There’s no cure”.
“Okay, but like how do we treat it to keep it from getting worse?”
“Well…there’s not really anything we can do. I’m going to refer you to a retina specialist. Don’t google it, okay?”
So I went to pay, kind of just shell shocked because how exactly are you supposed to fucking process something you don’t know about? Turns out I now get free eye exams because of my condition (it’s all coming up sunshine for #sashagay!).
What’s the first thing I did when I got home? Did I
A) Have a healthy snack
B) Read Great Expectations for class
C) Go for a light jog
D) Google what the fuck retinitis pigmentosa is
The correct answer, of course, is D. (When is D not the answer to everything? - I couldn’t resist) I remained #chill the best I could, because I had not yet seen a retina specialist, which I still wasn’t sure was a legitimate thing, nor had my diagnosis confirmed.
That’s a lie. I was #unchill. Before I’d had my formal diagnosis I knew all about RP and all sorts of fun (read: scary) facts about it. And then they confirmed my diagnosis. I am going blind. Retinitis Pigmentosa is, as GMED stated, a genetic degenerative eye condition. In laymans terms, you start with a loss of night vision coupled with a loss of peripheral vision.
If nothing else, I know now I’m not the only one who hates those damn signs
I wasn’t walking into “caution when wet” signs and TTC station employees and small children and off curbs and into oncoming traffic because of a lack of grace. I had been losing peripheral vision that would have allowed me to see all of the above. Other people didn’t just have superior night vision like some freakshow - they just had normal eyes that saw trees, stairs, and again, small children. (If you take nothing else away from all this, take away the knowledge that small children are the worst). Again as GMED had said, there is currently no treatment. There is no cure. I will continue to lose my vision at an undetermined rate until I am first legally blind, and eventually completely blind.
In many ways, I am incredibly lucky. My visual acuity - the field of vision in which I can see - is pretty damn good. I can see the world around me and as of yet am largely unaffected by my condition. I have no problem reading, walking around my neighbourhood, or doing my job. I’m pretty much as normal as I was before - for now. I will never be able to drive (GMED looked like he might faint when I told him I’d driven to my appointment until I clarified that Joey was behind the wheel). I’ll never be able to see the stars at night, or go to the movies without feeling like I’m in some sort of Survivor challenge. And I’ll become, eventually, more and more reliant on using assistive devices to get around and do the things I can do uninhibited today. At my last eye exam, a different doctor told me that A) I have a field of vision of approximately 50 degrees, having lost about two-four degrees of vision in the last year, and that B) given a continual degree of degeneration, I will be lucky if I make it to 35 before I become legally blind.
So if you look at the above diagram I have so helpfully provided, you’ll see what my vision loss at this point looks like. I have approximately 25 degrees of vision in each eye - so slightly less than the full thirty degrees of ‘near peripheral’ vision a person with no visual impairment would have. Legally blind is 20 degrees of peripheral vision, so about the triangle you see labelled here as macular vision.
Retinitis Pigmentosa isn’t a super common condition - something like one in 4,000 people have the condition, but even that isn’t confirmed. There’s not really a ton of research out there, so the information available isn’t totally reliable. General consensus seems to be that those of us with the condition lose the majority of their vision in their twenties (that’s me!) and 90% of us are legally blind by the age of 40, both of which align with what my doctor told me. So that’s what I’m essentially basing my outlook on: having approximately 12 years until I’m considered legally blind, with a steady (or rapid???) rate of decline in my field of vision until then. It’s a weird situation.
I finished out my last year of university as a “normal”, basically acting as though nothing has changed. Today, I am qualified to tell you I have a bachelor’s degree in English. That’s it - there’s really not much else that degree qualifies you for, in all honesty. Just after that, I finally set up my referral with the Canadian National Institute for the Blind. I’ve taken Orientation and Mobility training where I’ve learned how to use my white cane. When I’m out in the dark, in crowded places, or just unfamiliar places, I use my cane to let other people know I’m a public menace (that’s not meant to be a jab at other visually impaired people; my condition truly manifests itself in me being a danger to children and those little wire baskets at Shoppers Drug Mart) and to help me navigate more effectively.
I should clarify a few things. I’m not ‘normal’ any more, and I won’t be any time in the future. You win some, you lose some, and I’m pretty much okay with that right now. I can still see. I’m not legally blind. If I’m out with my cane, it doesn’t mean I’m 100% blind. I will, sir, be able to see you pulling out your fucking polaroid at the subway station to take a picture of me from three feet away. (True story. I looked damn cute that day, though, so can we blame him?). I’m visually impaired, so I do walk into a lot of things and people. I will miss you waving at me from five feet away, since I can’t see to either side of me and I’m usually focused on trying to see what’s in front of me. I can still beat my face like a RuPaul’s Drag Race reject, but I can’t ride a bike by myself. I can still go to work and do my thing, but I can’t see you handing me something. I can still text, use my computer, put together a cute lewk, and be an internet sensation, but I can’t find anything to save my life. Where’s my hearing aid? Where’d I leave my phone? What did I do with my lighter?
Trying to help me find something? Keep this in mind so I don’t hulk out on you
Game over. It takes me forever to find anything, because my eyes don’t scan the way someone who isn’t visually impaired would be able to. My boss likes to play a game on her iPad that has her looking for objects in a dark room with a flashlight that only lights up a small bit of said room- it would be a fun game to try out, except that’s my life. Poor embattled Joey and I went to Mexico last summer and he wanted to try out a ‘eat in the dark experience’ - except that was every dining experience I had, because “ambient lighting” really means “#sashagay comes mere millimetres away from impaling himself and/or giving himself a second-degree burn”.
So, I’ve never been normal. I’m relatively used to being different - it’s just on a bigger scale now. My friends are all graduating university or working their way up in their respective fields; they’re building careers and establishing themselves. For me, that’s a weird thing to think about, because how do I go about building a career when I A) don’t know what exactly I want to do with my life, and B) don’t really know how long I’ll have enough usable vision to work in that career? 80% of people who are legally blind are unemployed - and to me, that’s not unbelievable, because it’s hard to imagine what I’m going to do when I’m blind.
None of this is to say poor pitiful me, give me a pep talk about how I can do anything I can put my mind to. I’m not giving up on my life or any hope for the future. I consider myself a realist. If 80% of people who are legally blind are unemployed, there is a very good chance I will be in that percentage. If my vision is continuing to degrade, the likelihood of my life changing in a dramatic fashion is pretty damn high. People who say “you can do anything! I know a blind person who’s curing cancer, who’s an astronaut, who’s a grammy-award-winning singer” (hey Stevie Wonder!) are trying to be positive and uplifting. But those people are the exception to the rule. Telling me, unprompted, that I am going to be a force to be reckoned with and that nothing will stand in my way, is not helpful. I’ve never had astronomical aspirations for myself - all I wanted was to be normal. If I wasn’t going to win a nobel prize before, I really don’t see it happening anytime soon. It’s not defeatist, because I know I’m going to be okay. I’m going to work on my goals as they come. I’m going to do volunteer work when I can’t hold down a job. I’m going to be a fucking fabulous uncle to my nieces and nephews when they come around. I’m going to raise awareness and fight for the causes I believe in. I’ll just be adding a splash of Helen Keller realness to anything I do.
It’s just a weird situation to be in, to be in your early(ish) twenties and know that you’re going blind. How do you plan for that? How do you build a life around that? I said before: I like to be in control. I’m not. It’s scary. But I’ll be okay. I have an amazing family who would move hell and earth for me. I have Joey, who isn’t going anywhere so long as I can dangle a handicap parking spot in front of him. I have incredible friends and colleagues who would never leave me dangling on my own (except when they forget I can’t see them and turn and walk in a different direction than me - oops). I have new friends I’m making in the community - hey Mara! - who show me that I’m going to be okay, that retinitis pigmentosa isn’t game over.
I don’t have a super cute way to end all this, so I’ll just end off by saying I hope I’ve brought some awareness to y’all with all these words since it is retinitis pigmentosa awareness month. Here’s to not being normal!