Stories From the Edge of Blindness

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



By Your Side

I have been in a bleak place lately.  It is a familiar place, a place of introspection where I can try to figure out what is bringing on the sadness.  I thought it was because of the shit storm of rejections I have been getting, but they were just the cap on feelings that were already dragging me under.  I have been feeling overwhelmed for so long.

After coming to the conclusion that it isn’t the rejections that are pulling me into the clutches of sorrow, I had to stop and breathe and look behind my eyes to see what has been troubling me.  This can, at times, be a herculean task, as I seem to be troubled far too often, and it is never just one thing. But, I have become good at peeling away the layers, seeing what lurks beneath.
Continue reading “By Your Side”

Tiny and Immense

When I was first diagnosed with RP and thinking constantly about what it would be like to be blind, I was gripped by the spikes of claustrophobia.  I imagined that the world would become tiny, the darkness choking me and snatching my breath; the term tunnel vision, which is used liberally in describing what it is like to have RP, absolutely terrified me.  The thought of it was constricting; even writing this, I am holding my breath, as if doing so will stave off the inevitable. The day of my diagnosis was the day I started waiting for the world to close in on me.

I have always been somewhat addicted to darkness, prone to seeking out its path and wrapping myself up in the comfort of its fingers, but in the 15 years since my diagnosis, darkness has taken on new meaning and form; it has left the confines of my mind and heart, and settled into my eyes. I have come to realize that the reality of darkness, in relation to blindness, is the opposite of what I originally feared.

Now, the darkness feels immense. It makes me vulnerable and exposed rather than protecting me, like it did before RP showed up.  The darkness swallows the edges of everything and comes into a twisting life of its own.  In the dark, I feel as if I am always about to fall and whatever space I am in loses its definition and its structure.  It is unsettling.

The most recent experience I had of total darkness, was during my ERG, which you can read about here. During the ERG, the darkness came to life; I could see its tendrils twisting around me, escaping boundaries that only exist in the light.  I realized that the darkness is limitless; it has no parameters and nothing about it is predictable.  It takes something compact and at once expands and erases it.

Although I am still afraid of  what continued vision loss will do to my life and to my relationships,  I arrived at a place of acceptance a long time ago.  I suppose I have grown to both love and fear the darkness.  RP has been a part of my life for a long time; fighting it is futile and although I never welcomed it, blindness is a part of my fabric and I accept its texture.

I want to help expand peoples understanding of what it means to be blind and how a disease like RP works, so I am going to write a series of posts about the mechanics of RP and how it has manifested itself in my particular case. The first in the series, called, “What do you See?“, I posted a few days ago, and I will continue the series next week.  I hope that anyone who reads “Stories from the Edge of Blindness” feels free to ask questions; if there is something you are particularly curious about, please let me know and I will write a post about it. Thank you for reading and for being a part of my crazy road to blindness.

You can also find me on Facebook and Twitter

And, if you are in the mood for some pictures of some seriously cute pugs, you can find my babies, Blossom and Jade, on Instagram.

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.

ERG – Take 2

The day of my RP diagnosis was filled with different tests, including an ERG (Electro Retina Gram), which tests the retinas response to light.  It was by far the most grueling of the tests that day ( I wrote about it in another blog post when my blog was brand new), but I was lucky because I didn’t have to have another one…..until yesterday.

I have to say that this time, the ERG was much less traumatizing, both because I knew what to expect and because the woman administering the test was compassionate and friendly (the opposite of my first experience).  Don’t get me wrong, I was still freaked and the test still seriously sucks, but a friendly and competent tech makes a huge difference.

For those of you not familiar with the procedure of the ERG/Dark Adaptation test, let me give you a run down. After your eyes have been numbed and dilated,  you have to sit in a completely dark room for 30 minutes; and I mean completely dark, like the woods in the middle of a cloudy night with no moon dark.  The idea is that 30 minutes will be enough time for your eyes to adjust as much as they can, which with RP is pretty much not at all, as most of us have night blindness.  I sat there in the room trying to meditate and, every few minutes, I put my hand in front of my face to see if it was becoming any clearer.  It never did.  The first time I went through the 30 minutes in the dark room, I was terrified and crying; this time, I was more contemplative.  I kept my awareness on the darkness itself; it was as if I could see inside of it.  It felt like a web around me and was at once suffocating and endless.  I was more curious about it than afraid of it.

After the 30 minutes went by, the tech came back into the room, through a special door, between the outer room and the dark room, that prevented any light from entering.  It is very important that the first part of the ERG be in darkness; hence the dark adaptation portion. And, then the real fun began.  First, she gave me some more numbing drops and while they were doing their thing, she attached an electrode to my forehead and started prepping the discs.  Plastic discs (they call them lenses) get inserted between your upper and lower eyelids to prevent you from blinking and ensure your eyes stay as open as possible during the test.  The first pair of lenses she tried to insert (the one’s for grown ups) were too big.  She left the room and came back with pediatric lenses; also too big.  What the hell, I thought.  I know I have tiny eyes, but they are bigger than a babies eyes.  She came back with the smallest lenses and those, finally, went easily into place.  She told me that I required the smallest lenses because I have high cheekbones, like Cindy Crawford.  I think she was just being nice, but I was grateful that she didn’t try to force the grown up lenses between my lids.  It became clear to me that the first ERG tech had forced the larger lenses and that must have added to the horror of the whole thing.

To insert the lenses, she asks me to look down while she pulls my lashes and upper lid open, placing the disc first into the top part of my eye and then into the lower.  She then attaches electrodes to each of my cheeks and tapes the wires into place.  The room is still dark and I still can’t see a thing, so I turn my chair to where I think the testing device is (turns out I got it right) and she guides me toward the chin rest.

The testing device is a big dome with the chin rest at the opening.  At the back of the dome is a red light; she asks me to try and stay focused on the red light; it is the first light I have seen in an hour.  Then the test begins.  For the first part, the dome remains dark.  Lights shoot toward my eyes about five or six times, then stop and then start again with varying intensities.  There are about 6 rounds of flashes in the dark and then she turns on the light in the dome.

It is an insanely bright light.  And remember, my pupils are dilated, my eyes are being kept open so I can’t blink and I have increased light sensitivity even when my pupils haven’t been dilated (another perk of the RP).  This is pretty much my worst nightmare. The tech tells me I have to wait five minutes to adjust to the light. My eyes are in agony.

The first sets of flashes are, like the ones in the dark, about five or six seconds a piece, but this time, I have to wait 2 minutes between each set.  My eyes are burning and stinging and the lights in each set increase in brightness and speed and each 2 minute waiting period feels like two hours.  Thankfully, the tech talked to me and kept me aware of the time; she was really sensitive to how I was feeling.  She knew what was coming.

They save the best for last.  The final two sets of lights are successive and last about ten seconds each.  The lights are like frantic strobe lights that shoot at your with a force of brightness that takes your breath away.  The very last sequence was so intense and painful that my lower lip and chin started to spasm.  Ten seconds of pure hell.

The technician was pretty quick, thankfully, to get my face out of the dome and the lenses out of my eyes.  Then, she rinsed each eye a few times with a really cold saline solution and told me my pupils would be dilated for at least another 2 hours (I knew it would be more like 4) and that my eyes may feel a bit dry and scratchy for a few hours after the numbing drops wore off.  She led me to the door (she was a good guide, by the way) and I stepped out of the nightmare and into a bright blurred hallway.

I couldn’t see a thing.  Luckily, I had Zelda.

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