It’s 7 a.m., and I’m sitting cross-legged at my desk, eating cereal and listening to NPR, when a smooth-voiced reporter tells me the first patient has been treated for an eye disease using CRISPR. The procedure involves injecting material directly into the patient’s eye, which the researchers hope will alter the errors in the genetic code that cause the disease and allow the patient to regain vision.
I jump up, run upstairs to the bathroom where my husband is showering. Through the curtain, I tell him about what I’ve just heard.
He doesn’t catch the words, asks me to repeat myself.
I get closer, shout over the water, probably louder than necessary this time, that there finally might be a chance they can fix me.
In the dim and small examining room, the doctor asked me a long string of questions about what I’d been experiencing. I was seventeen, had been spending most of my time hunched over textbooks in the final manic push before AP exams. I’d been getting headaches, noticed the words occasionally seeming to slant, distort. When I closed my eyes after staring at a page for too long, sparks chased themselves around the darkness.
“You probably have an astigmatism,” the doctor said. “Very common. Easily fixed.”
There were tests, then, an hour with his face inches from mine, I was presented with an array of things to look into or look away from or put my head into. Hold still. Blink. Don’t blink. I could smell his wintergreen breath, hear each inhale. I matched my breathing to his, tried to be obedient and empty.
“Is the light uncomfortable?” he asked, his voice soft.
It was, extremely, though I hadn’t let myself acknowledge it. I nodded.
He apologized, and, after a moment, switched it off. He was murmuring to himself, about accumulation in the retina, visual anomalies, photophobia. I didn’t know what any of this meant, but it didn’t sound like good news. “What’s wrong with me?” I asked. I wasn’t quite able to control the shake in my voice.
He scooted his examining stool a little away from me. “Maybe you want your mom in here for this.”
I have Stargardt disease. It’s a rare condition that results from mutations in the genes responsible for the clean-up work inside the eye. Normal eyes remove cells from the retina once they’ve died; my eyes, however, leave the dead cells, which causes them to pile up and progressively destroy my ability to see from the center of my vision outward.
At ten, despite absolutely no signs that anything would someday be wrong with my vision, I became terrified of blindness.
An anecdote like this, if I were reading it in one of my student’s fiction stories, would prompt me to write marginal notes like “implausible” and “too on the nose.” I understand this, the deep weirdness of my prescient fears. Bear with me.
It began when my mother read the Little House series by Laura Ingalls Wilder to me. We’d settle into the couch together, me leaning against her and watching the progress of her hands down the page, her fingers marking our place. My then-perfect eyes had permitted me to become a very good reader. I had a book in my hands whenever possible. I ruined copies of Great Expectations and Jane Eyre by accidentally dunking them in the sink and my cereal bowl, respectively. But I still loved being read to, the soothing way my mom’s voice gave shape to the characters and places, allowed us to experience the unfolding of Laura’s life together.
But then we arrived at the part of the series when scarlet fever strikes Laura’s sister, Mary, and takes her eyesight. Bit by bit, colors and light fade, until only the brightness of the sun penetrates, and then nothing at all, plunging her into a world of eternal dark. This terrified me, imagining what it would be like to strain and strain but see nothing. The vulnerability of it, the utter finality of the loss.
I reached over, snapped the book shut. “Please don’t read any more,” I said.
My mother tried to reassure me, to convince me to let her find the page again, to continue, but I couldn’t pull my mind away from the tragedy, I felt betrayed by it. I told her no, that nothing could ever be all right for Mary again. I didn’t understand how she could be so deprived so suddenly. The bitter unfairness of it, and that it could happen to anyone without warning, at any time. Could happen to me.
That night, my dad tried his hand at comforting me, told me stories about tragedy were useful because they prepared us for the world’s difficulties, but at the same time, living in fear of things out of our control was useless. This was his way: practical, matter-of-fact. “No matter what happens,” he said, “you’ll be fine.” Then he tucked my feet in, his special dad move that meant conversation was over and that I needed to at least pretend to sleep, because, as he was always telling me, pretending to sleep usually led to actual sleep.
Sometimes this worked. But this wasn’t one of those nights. After he’d shut my bedroom door, I counted to fifty, long enough to be sure he’d be back downstairs, then disentangled myself and slid out of bed. I shut myself into my closet, stuffed t-shirts into the crack beneath the door to blot out the glowing sliver from the streetlight outside my window. Full and complete darkness. Knees tucked in tight, I sat, eyes opened wide, looked deep into the nothingness. I imagined only this as my reality, forever.
The next day, I told my mother we could continue with the book. I needed to know what things would be like for Mary.
My mother, clearly relieved, resumed the story with me that afternoon. In a subsequent chapter, Mary and Laura ride a train for the first time. Laura is looking around, taking it all in, describing it to Mary. The seats are beautiful velvet, Laura tells her, and Mary reaches out in front of her, runs her fingers over the material. “I see!” Mary says.
When my mother read this line, a thrill ran through me.
“She’s cured!” I said. “It’s over, and she’s fine now!”
My mother shook her head. “No, honey,” she said. “Mary meant that there’s a new way she can experience things now. She still can’t see.”
My fears about going blind persisted past our conclusion of the Little House books. In one of my scientific magazines my dad subscribed me to, intending to encourage my curiosity about the natural world, I came across a passage about how staring into the sun would cause permanent damage to the eyes. This took obsessive hold in me, and I began averting my eyes from the sky whenever possible, fearful that I would inadvertently sear my retinas. This persisted into my first year of middle school, causing me to choose the desks farthest away from windows in my classrooms and to shade my eyes during the walk to the bus. I was already not a terribly popular kid, and my new weird behaviors certainly didn’t do me any favors. Perching at the edge of bus seats, I’d bury my face in books, shielding myself from the kids who eyed me sideways.
The last straw for my parents came during one of my summer soccer games, when the coach pulled me off the field. “You can’t watch the ball with your hand over your eyes,” he told me, exasperated. “If you can’t look where you’re going, you’re not going to play.”
The solution, my parents decided, was to take me to an eye doctor who would explain to me that I was being irrational, that a quick glimpse at the sun was not going to harm me, and that I was in no real danger of losing my ability to see. The eye doctor, obliging, explained all of this to me, patiently and in multiple different ways, since I kept asking questions.
“You have nothing to worry about,” he said, smiling with what I imagine now was a serious and concerted effort.
After several years and some quality therapy for anxiety, my fears of blindness had dissipated, been done away with through logic, forgotten. And then, at seventeen, this impossible diagnosis.
The immediate plan, my parents decided, was to consult the specialists the eye doctor referred us to, to confirm that I did indeed have Stargardt disease. I didn’t want to see specialists. I wanted to ignore it all, pretend there was nothing different or diseased about me. If I didn’t pursue any of this, it wouldn’t feel real. Focusing on my eyesight had once opened a chasm of fear in me, and I wasn’t ready to lose control of the calm and balance I’d worked for years to cultivate. I’d finally achieved that very elusive state for teenagers: being perceived as normal, and having friends who liked me. I couldn’t bear the idea of going back to being the weird kid everyone avoided, the one with something wrong with her. All I wanted to worry about was when I’d hear back from the colleges I’d applied to, my inscrutable calculus homework, walking the dog, which potions best concealed my stress breakouts so that boy who sat next to me in Latin class might notice me.
I was, therefore, not in the best mood when I left for the Cleveland Clinic with my parents. I sat sulkily in the backseat and listened to them talk like they always did on road trips. Arguing lightly about directions, pointing out vanity plates, discussing me. I pretended to sleep, then actually slept.
At the clinic, we waited for nearly three hours before we were seen. First they took my blood so they could use it to further research the disease. This I was excited about—I imagined the tube passed from hand to hand, scientists murmuring excitedly before placing it under microscopes, scribbling notes, and then leaping up as they made great discoveries.
Finally, a doctor came in to see me, a nervous man with a nasal voice. He had a crew of medical students with him, and he asked if I minded if they participated in the exam. I didn’t feel like I could say no. He went first, took a hold of my head firmly, and shone lights in my eyes. These lights were painful, white, and sharp. He had to hold my eyelids open because the light felt physical, like hot metal entering my skull and pounding deep inside it. It went on and on, him searching around, so all I could see were my blood vessels transposed weirdly on to my vision, red and raw-looking. I imagined each one opening a tiny mouth to protest.
“Just another moment,” the doctor kept saying. “Hang in there. Just another moment.”
And then it was his students’ turn. Each of them did the same, pointed the same light-pain deep into me, and by the third student I had to close up the back of my throat so I wouldn’t sob aloud.
One of the things we’d learned from the informational booklets in the clinic was that exposure to light caused my vision to degrade faster. Yet here was this parade of people, training their lights on me, continuous and unrelenting. It felt like they were peeling days of good eyesight away from me. I thought of the movie The Princess Bride, when Westley is strapped to a machine in the Pit of Despair. The machine turns on, and with a horrible grinding and rumbling, he begins to scream. The man running the machine then snaps it off, and calmly says, I’ve just sucked one year of your life away.”
I realize now I should have told the students to stop, but I was afraid I’d entered some agreement I couldn’t back out of, that my treatment was conditional upon letting myself be examined and examined. By the time the fourth and final student was done with me, my head was throbbing and I had nearly bitten a hole through my lower lip.
“Bathroom,” I said, and stumbled up out of the chair. My vision was a jumbled mess of black orbs where the lights had been, and I could only see tiny pockets of the world around me. I lunged for the door and my mother caught my arm, helped me into the hallway. We managed to make it into the bathroom before I began to cry. Within those tiled walls, I let go completely. I gave myself over to the deep heaving and gasping, let myself be loud, be miserable. This was happening to me, all of it, and there was no escaping this body that was flawed, breaking down, betraying me.
My mother held me as I cried, letting go only to bring me more paper towels to soak up the dampness on my face. I don’t know how long we were in there. A long time, I think, before the torrent subsided into quiet hiccups. My mother held my hair back as I washed my face. “We can go home if you’d like,” she said. “We don’t have to stay a minute longer.”
Knowing I had an out gave me some strength. “No.” I sniffed, tried to sound confident. “It’s important that we stay, find out everything we can.”
Back in the examining room, the doctor confirmed the Stargardt diagnosis.
“How long until—I mean, until it all goes dark for me?” I asked.
The doctor tilted his head at me. “The good news,” he said, “is that you’ll never be fully—dark, as you put it. You’ll always still have some vision, albeit indistinct. As to the progression of the disease, there’s no way to say for certain. The degradation occurs at a different rate in every patient, so only time will tell.”
My father wanted to know about treatments, to know how long until they could cure me. “I’m afraid I can’t give you anything definitive,” the doctor said. “There are a lot of promising studies, but these things take time. You and your daughter will just need to be patient.”
For nearly half of my life now, I’ve been living with limited vision. There has been no treatment; just vague references to research, slow and without promise for me. The effects of the disease have interwoven themselves with my identity, become my daily reality. I’ve had no other choice but to adapt, rearrange my expectations for my future. These effects have, unlike Mary’s, come on slowly, which has allowed me to adjust, bit by bit, to what I can no longer do.
The first thing to go was reading. The center of the eye is responsible for fine detail, and there came a point when the words on the page took too long for me to work out. This was—it’s a laughable understatement to say—difficult for me, because books have always been my companions. Opening a book and shoving my face in it, falling into a world that isn’t mine, allowing the words to fully transport me, has always been a necessary part of my life.
I’ve been fortunate, though, that text-to-speech software had been developed and fine-tuned by the time I needed it. During my junior year of college, I retrained myself to hear books instead, listening to a computer-created voice deliver the contents of my textbooks to me. Now, I don’t even hear the voice, as if the contents are being directly injected into my brain.
The next thing to go was driving. This was during my first year of graduate school. I could still drive at this point, my peripheral vision able to compensate for the holes in the center, but I didn’t want to wait for the moment that determined it was over, when I injured myself or someone else. Goodbye, car.
I was living in Lewisburg, Pennsylvania, a small college town that was reasonably walkable, and I had good friends who were happy to take me on errands if I asked, would check in to see if I needed anything if I didn’t.
The next was recognizing faces. This was only a few years ago, just after I graduated from my MFA program at Purdue. I’d decided to stay on for another year as an adjunct professor, and had committed to teaching three sections of English Composition. When I entered class the first day, I discovered that my students’ features were unreadable to me, indistinct shapes I couldn’t distinguish.
This last problem didn’t have an immediate solution, and it’s one I continue to work at. I’m still a teacher, though now in Connecticut. There are a few things I can do—group students so that they always sit in the same section of the classroom, move around during discussions so I can get closer to their faces, learn their voices. None of it’s perfect yet, but I’ll do whatever is necessary to make teaching possible.
There are a lot of things sighted people don’t think about that require serious contemplation or strategic planning for me. If I’m meeting a friend for lunch, I look up the menu online ahead of time and use my text-to-speech to determine what I’ll order, since I won’t be able to read any of the options at the restaurant. I arrive ten minutes early, so my friend has to find me, instead of me bumping around the tables trying to figure out which blob is the person I’m trying to eat with. I beg off of game nights because most games involve tiny print. Or, just sort of seeing in general. If I get too many text messages at once, I just call the person and ask them to explain themselves.
Most people know why I do these things, but not everyone. I don’t love telling people there’s something different about me, and certainly not right after I’ve met them. It’s a weird thing to work into conversation. “Yeah, I’m a big fan of folk music, too. And did I mention I have a form of early onset macular degeneration that is progressively stealing away my ability to see?” It’s uncomfortable and awkward, and I still haven’t figured out when the best time is to tell people. I’ve picked the wrong moment before, and seen the way it shifts the conversation, gives it a heaviness that our relationship wasn’t ready for. I can feel the emotional edging away, and it isn’t their fault—there’s no guide on how to respond to an admission like that, and they don’t know if they should express sympathy, or ask questions, or just treat me as if I’d never told them. For this reason, I still have some people I consider to be good friends with whom I’ve never really talked about it. I’m afraid to break the fragile balance, to make them uncomfortable, to burden them with the knowledge. And part of me just doesn’t want to go back to being that kid everyone stares at, doesn’t want to talk to on the bus.
So, sometimes I lie.
And I can get away with it, because what I have is an invisible disability. There’s a lot of scholarship around this, but basically, if you look at me, it’s difficult to tell I’m anything but typical. My eyes don’t look cloudy—they’re clear and blue—and I don’t even wear glasses. For people like me, you can either “pass” or “come out.” To pass, certain untruths are necessary, certain explanations for things I do. Why I don’t walk with you to the parking lot but instead call an Uber to get home from work. Why, when you waved at me from across the street yesterday, I didn’t wave back. If you show me something on your phone, I might just glance and laugh, hoping it’s supposed to be funny and not shocking or tragic. I struggle with this, the string of little lies or excuses or pretenses I use because I’m not ready to open up completely. I hope people will forgive me for the lies if I work up the courage to come clean. It’s not unlike how I felt in middle school when I first discovered I was attracted to not just boys but girls, too, that a part of me may not always be acceptable to everyone, could create distance or even disgust if not hidden away.
But here I am, in this essay, coming out to you. Hello, reader. Now you know.
Sometimes I feel sorry for myself. I draw myself a big steaming bath of self-pity and soak in it for hours, think about how much my life sucks, how much harder things are for me than “normal” people. I get a little angry, get a little sad. Get a good cathartic cry going.
It usually doesn’t last long, though. Because I am, on the whole, fortunate. I have kind and thoughtful friends and family. I have an understanding husband who sees me as a whole person, who doesn’t pity me but knows when I need help and gives it freely and ungrudgingly. I have freedom of mobility through a combination of public transportation and ride-share apps.
I have access to software that allows me to read my students’ papers, become anxious about current news articles, and stay abreast of my friend’s tweets about the ways she’s going to burn down the patriarchy. I’m able to write. As my father told me, when he couldn’t have possibly known how important it was, I am and will be fine.
But the idea that there’s a cure out there, that CRISPR could grant me my sight back, could grant me the ability to do all of the things I won’t even allow myself to list—it’s a hope so burning, so powerful, that I’m afraid to touch it. The story about Mary taught me not to trust this hope, this belief that suddenly, miraculously, I will be fine and see perfectly again.
And yet. Maybe it is possible, after all.
My husband knows all of this in the incredible intuitive way he has, sees it on my face as he sticks his head out from the shower curtain.
He reaches a soapy hand out to me, and I take it. For now, this has to be enough.
***
Rumpus original art by Madeline Kreider Carlson.
