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Stories From the Edge of Blindness

In 2002, Retinitis Pigmentosa changed my life. This is my story of a slow approach to darkness.

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Heckenlively

Writing Sample Part 2 – Dx Day

In re-reading what I sent in as a writing sample to the Emerging Voices Fellowship, I can understand why they didn’t choose me to be one of their 5 fellows (but they are still fuckers).  I think the pieces have good bones, but I could weave around them more creatively to make the stories more intricate.  Editing and re-editing and re-editing again is just part of being a writer, and it is actually a part I don’t mind too much.  That said, I am still going to post what I sent to them.

Dx Day

Visual Field

On the day of my formal introduction to Retinitis Pigmentosa (from here on out affectionately referred to as RP), I woke up to a chilly five a.m. alarm, took a quick shower, threw on lounge around the Eye Institute clothes, and waited for my dad to pick me up. The day began with a series of scary sounding tests; well, let’s face it, tests by any name sound pretty scary and I had a history of doing lousy on tests.

The first test of the day was a Visual Field, but with a twist. Rather than being scored by a computer, I got a live proctor. Damn, there would definitely be no cheating. During a Visual Field, you are asked to place a sterile patch over one eye, stick your face into a large white dome and press a button each time you see a flashing red light. Sounds simple, straight forward, totally passable. It sucks.

The proctor instructs me to stick my face into the dome and asks if I can see the big red light in the middle, then tells me to focus on it for the next fifteen minutes, with one eye, while pressing a button as other red lights flash in the distance. Instantly, I feel an impossible urge to blink and my eye starts to dry up. I am really trying to focus on the big red light in the middle, but it keeps disappearing; and then I remember the flashing red lights that are supposed to be appearing with some regularity, but they seem to be on hiatus. Then, right there in front of me, like a beacon of hope, I see a big red flash……or I think I do…….so I press the button, hoping that I answered correctly. 40 minutes later and both eyes tested, I am excused; sore neck, dry eyes, and fuzzy red lights flashing all over the room.  One down………

ERG – Electroretinogram

After the visual field, I was led further into the basement maze of Jules Stein Eye Institute, to the second waiting room of the day.  The room was empty, except for a weary mustard yellow couch.  The air smelled like doom.  I don’t remember how long I waited; I was only aware of the quick rhythm of my heart.  After a time, a woman with bushy hair and a staccato voice came into the bleak room and called my name, looking at me with a hurried and impatient glance.  She ushered me through a creaky beige door that led to a dimly lit room with whirring, then unfamiliar, machines.  She asked me to sit in a chair so she could put drops in my eyes, and told me I was lucky to be there; patients came all the way from Egypt to see Dr. Heckenlively.  I was feeling anything but lucky and had yet to actually meet the infamous man.

After two sets of eye drops were administered, the bushy haired woman led me to a small room with a black leather couch and told me I had to wait there until the drops took effect.  She closed the door and turned out the lights.  The room was pitch black.  I had no idea why I had been locked in this dark room with the lilting voice of Sade telling me she would never leave me sad; all I knew was that I couldn’t see my hand in front of my face and I was terrified.  Pure darkness removes the security of boundaries, the familiarity of edges; it swallows everything. Was this what my future held?  This total darkness that made the world impossible, overwhelming and immense.  I waited for my eyes to adjust to the dark.  That day, in that room of pitch and fear, I learned about night blindness and that RP eyes don’t ever adjust to the dark.

The tech returned after an interminable 30 minutes and opened the door; the room was bathed in red light and I couldn’t see her face, but felt her take my arm as she proceeded to guide me into the torture chamber.  She sat me down in a chair akin to the kind you find in a dentist’s office; this should have been a warning sign to run, but how can you run when you can’t see?  I was at her mercy.  She put two more sets of drops in my eyes, slathered them in a cold gel and then inserted plastic disks under my eyelids.  “So you can’t blink”, she said.

“So I can’t blink?” I thought, “What the fuck?”   I felt like I was in a Stanley Kubrik film.

She led me, and my unblinking eyes, to another chair and helped me find the chin rest at yet another entrance to one of those strange domes.  “Lean forward and focus on the red light”.  Five seconds, ten, thirty…….wham.  Bright white lights shot out of the walls of the dome, assaulting my eyes and stabbing at the limited fields of my vision. This happened multiple times, each burst brighter and more intense than the next.  It was so painful, my bottom lip and chin started to spasm.  And then, the machine stopped and I was once again, in darkness.

“You’re all done”, she said, sounding chipper.

I wanted to shove plastic disks under her eyelids and see how chipper she’d be then.

The Man Himself

After the Clockwork Orange experience, I was dismissed for lunch.  I walked out of the test room, with help from the tech, and met my dad out in front of the Jules Stein building.  I felt pretty traumatized at this point and my eyes were sore and watering, vulnerable to the assault of sunlight. We went into Westwood Village and ate Greek food and I tried to keep a smile on my face; I knew my Dad was almost as scared as I was.  We waited quietly for the final appointment of the day; I was finally going to meet Dr.  Heckenlively.

I couldn’t help but wonder, what kind of man comes with the name Heckenlively?  When I first heard the name, five years earlier, I knew I would never forget it; it is hardly an unforgettable name. I imagined Heckenlively to have a jovial disposition, to be kind and of course a total genius who was going to give me hope of a cure for my rare and unique disease. I thought he would be a bit portly in stature with gentle hands and a slow easy voice. He would exude warmth and compassion. He would spend time with me, explaining the intricacies of RP and comforting me in my time of distress; or maybe he would tell me I didn’t have it, maybe he would tell me it had all been a big mistake.

When we returned to Jules Stein, we checked in at a huge reception desk in the Retina Clinic and I was taken into another room for more drops; this was the fourth set of dilation drops I received and apparently this last set was just for good measure.  My pupils were not happy. Back in the waiting room, I had to keep on my sunglasses and couldn’t read a magazine, so I just looked around at the other patients waiting for their fate to be served up by the illustrious Heckenlively.   There was no pattern to the population of patients in the waiting room; no predominant age, race or gender.   RP doesn’t discriminate.  There aren’t many of us who have it, but we come in an array of packages.

One person from that waiting room stands out most clearly in my memory.  He was a young African-American guy, maybe in his twenties, tall and athletic looking.  He was sitting between two older people who looked like parents; his head was bowed and his hands pressed against his eyes.  I knew he was a first timer, like me; I knew he was waiting to be told that he was going blind and I knew that he had realized the months of hoping for a different outcome had been fruitless.  It was the first moment that day that I didn’t feel so alone because I knew he understood exactly how I was feeling.

I think we must have waited at least an hour, watching men in white coats disappear behind mysterious doors, patients with anxious and terrified faces startling at every entrance.  Finally, they called my name and my dad and I followed a nurse into one of the secret rooms; I almost felt guilty leaving everyone else behind.  The room was dim with another one of those pseudo dentist’s chairs filling the middle of the ominous space.  The nurse told me to sit there, and I was tempted to tell her to go fuck herself, but I looked to my dad for some courage and took my allotted place.  The nurse left and about ten minutes later, the door opened again.

Dr. Heckenlively wasn’t what I had imagined or hoped he would be.  He was tall, lean and middle-aged, with a Hitler mustache and a cold demeanor. He looked like a sadist.  I knew instantly that this man was not going to be my savior or my champion or my friend; I was just another blind number. I remember he shook my hand curtly, barely looking at me, and then sat down to take a history.  We didn’t have much to tell him; no one in my family had any hint of vision impairment. With no obvious genetic ties to RP, I was obviously a boring case for him.

After the history, he turned to me and flipped the dentist’s chair back, coming at me with one of those super power head lamps that eye doctors tote around.  He examined my traumatized retinas for maybe five minutes, flipped the chair back into place and told me I had RP.  On his way out the door, he casually mentioned that someone would be in to take a blood sample, just to rule out syphilis.  That was the one and only time I would meet Dr. Heckenlively.

That afternoon, I was given a day of torture, 10 minutes with the expert, a heartless delivery of my diagnosis, and a lifetime sentence of imminent blindness.

Writing Sample Part 1 – Retinitis Pigmentosa

Along with my application for the Emerging Voices Fellowship – that I didn’t get (fuckers) – I had to send in a writing sample.  I felt compelled to share the pieces I sent them, so this will be the first of 5.  If you know me well or have been following my blog since it’s inception, you may recognize some of the stories, but they have been edited again and again since their original appearance.

*Retinitis Pigmentosa (RP): A group of inherited disorders in which abnormalities of the photoreceptors (the rods and cones) of the retina lead to progressive visual loss. Abbreviated RP. People with RP first experience defective dark adaptation (night blindness), then constriction of the visual field (tunnel vision), and eventually, late in the course of the disease, loss of central vision. 

 The first time I heard about Retinitis Pigmentosa was from Barbara Walters. She reported a story on 20/20 about a family whose father and three children all suffered from a rare eye disease that caused something they called “tunnel vision.” I remember all the kids were very blonde, with extraordinarily bright blue eyes and pale skin; it was as if they were rarely exposed to the sun.  The program attempted simulations to show what an RP sufferer’s deteriorating vision is like, and it was terrifying; can you imagine your entire view of the world being the size of what you see through the hole in a straw? Knowing that the hole would get smaller and smaller until everything was darkness?  I remember feeling frightened and sad for the family, and thinking how lucky I was that everyone in my family had such good eyes.

The second time I heard the words Retinitis Pigmentosa, I was in my late 20’s and visiting my optometrist for a routine refraction. I was there to get some new glasses because my night vision seemed to be worsening; not unusual in nearsighted patients.  He asked if he could dilate my pupils and take a closer look inside my eyes. The exam only lasted about ten minutes, but it seemed like an hour.  It was only the second time my pupils had been dilated and it was excruciating. The bright light from his instruments felt like fire and my eyes burned.  When he finally finished the exam, he said something about bone spicules in my retinas and a rare genetic disease that caused blindness. He referred me to a specialist named John Heckenlively.  I nodded my head, pretending to understand what the hell he was talking about, took Heckenlively’s number and proceeded to blow the whole thing off. There was no way I could have some random, rare eye disease.  No one in my family even wore glasses; except me of course.

Fast forward about five years; I am walking out of a Borders bookstore heading toward my car, and as I turn a darkened corner, the sidewalk in front of me disappears. It was like the darkness had consumed the ground beneath me. I stood there in abject terror for a few minutes, until I looked up and found a street light shining dimly a block away. I rushed toward the light, telling myself to call the eye doctor in the morning.  I obviously needed some new glasses.

Two days later I am in the Ophthalmologist’s waiting room, filling out-patient forms, and I come to the question about previous problems with eyes. I hadn’t thought about my visit to Dr. Vogel, the aforementioned optometrist, or Retinitis Pigmentosa in five years, but sitting in that waiting room I remembered, and I think at that moment I knew something was wrong, something that couldn’t be fixed with new glasses. That afternoon I had my first visual field test and was once again referred to Dr. Heckenlively. This time I couldn’t ignore it.

Five months later, I was formally introduced to Dr. Heckenlively and Retinitis Pigmentosa.

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