Punctate Inner Choroidopathy, the fun retinal disease that's killing my vision
I'm 28, and I've been going blind since my freshman year of college.
I don't talk about it often, and I hesitated for a long time before putting it online (medical diagnoses + Internet = privacy risks), but there's such a lack of information about it that someone had to do it. It's a rare eye disease called Punctate Inner Choroidopathy (PIC for short) that causes patches of blindness in the retina. It affects near-sighted women starting in their early twenties.
I first noticed it over spring break in 2004, when I was home from college working on a paper in a Word document. I didn't realize it then, but a white background and black text is the perfect canvas for spotting anything odd with your vision. I noticed a black spot in my left eye that moved wherever I looked.
At first I thought it was a problem with my contact lenses, which I wore for way too many hours in high school and college. Like, days in a row. And this was before high oxygen contacts were on the market. My bad. But the spot kept getting bigger, and it moved with my eye, not with my contact. While at first I couldn't see one or two letters on the screen, it progressed to blocking out entire words.
I saw an optometrist, who referred me to a retinal specialist. He had me tested for Toxoplasmosis, a disorder you can get from parasites in cat litter. And while I waited for the results of that test, which take a few days, I went to Boston to hang out with my boyfriend at the time. I was playing Star Wars: Knights of the Old Republic, and the dark spot had grown to a blob that completely blocked out all the text on the screen (and those of you familiar with KOTR know there's a lot of text, so that put a serious damper on my Jedi powers).
I was literally playing with one eye closed by the time the doctor called back, sounding worried, and saying I had to get to New York City immediately for emergency treatment because my retina was bleeding. Meanwhile, my cat was vindicated that he didn't cause it.
My dad dropped everything and drove me to Vitreous Macular Consultants in New York, a group of specialists who really knew their stuff. I went through a bunch of weird imaging tests, which have become routine to me now, that let the doctors see the tiny details of my retina. It was bizarre to be the sole patient in the waiting rooms under age sixty, yet experiencing the same symptoms as everyone else.
The doctors delivered an ultimatum: either undergo experimental laser surgery to cauterize the bleeding spot, which would burn out some vision permanently; or take my chances, do nothing, and hope it stops on its own, but risk losing all the vision in that eye. I went for option number 1.
The treatment, called Photodynamic Therapy (PDT), was a strange thing to go though. The doctors put Clockwork Orange-esque metal things under my eyelids to hold them open while a technician stood behind me, dropping saline in my eyes because I couldn't blink. And I just watched the laser as it burned out my eye. I mean, I have a morbid curiosity about these kinds of things anyway, but I was physically incapable of looking away even if I wanted to.
The surgery left me with a big blind spot in the center of my left field of vision, but it stopped any further bleeding. I still have that spot today. If I look at you out of only my left eye, it looks something like this:
It's especially pronounced on black and white backgrounds and on straight lines, which is why the Amsler Grid test is used to identify new problem spots. You're supposed to look at it every day. I have it as the background of my phone.
You can probably imagine the hilariously fun times I have trying to align objects in Powerpoint and other programs when straight lines look wavy.
Thankfully, I still have my right eye, the "good" eye, which lets me read and see details. The peripheral vision in the left eye is fine, too.
However, the worst part about PIC is the uncertainty of having a flare-up, a period when new holes, or spots, appear in the retina. Active spots show signs of inflammation and fluid buildup, which can permanently destroy vision. As soon as I see any dark spot in my eye, I have to nail it with treatment or risk more blindness.
Spots are usually treated with The World's Most Cringe-Inducing Procedure: needles to the eyeball. That's right: the doctors stick a needle into my eye and give an injection of steroids or substances like Avastin, which inhibit the growth of new blood vessels that can lead to bleeding. It actually sounds a lot worse than it is. The eye doesn't have a lot of nerves, so that combined with some numbing drops results in nothing more than a feeling of weird pressure and a grossly bloodshot eye for a week or so. But bonus: if you have it on Halloween, you can be an awesome zombie.
Flare-ups have an autoimmune component, but nobody really knows what triggers them. For me, it seems to happen in the winter and/or when I'm under more stress than usual. Celiac Disease also runs in my family, and my general practitioner suggested I try eating gluten free for a while to see if it helps.
I'm in the middle of a flare-up now, and it's in my right eye. The "good" eye. The one I'm relying on to read, look at people's faces, play video games, see stupid things on reddit, have a job, have a life. The spots I'm seeing are like a triangle grouped right around my center of vision, just outside of it. I can see them creeping in as I write this post. If any one of them starts leaking, it could kill my vision forever. All I can really do is notice them when they appear and get to a retinal doctor as soon as possible. Other than "don't hang upside down or lift really heavy things," no one has any advice for me.
The spots have gotten dramatically worse over the past few years. This picture compares my eye in 2001 (on the left) to 2013, a few days ago. Note all the white spots: they aren't supposed to be there. Most of them appear far from the fovea, the center of vision, which means I can't even detect the loss of vision. It's the ones close to the center that pose a threat.
I'm a visual person; I learn by reading. Almost all my work is reading and writing. I don't know what I'd do if I couldn't see words. I'd still have peripheral vision, though, which is far better than total blindness, and I'm grateful for dodging that. It runs in my family: my grandfather, my dad's father, went completely blind in his thirties and no one ever knew the cause.
So I'm headed to the doctor in just over an hour for a series of needles and injections and uncertainty. All I can do is hope for the best, appreciate the vision I still have, and share my story in case it helps anyone else out there who's as scared as I am.
That, or just wait for stem cell replacement to take off. Or cybernetic eyes. I want those.