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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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Vision Loss

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”

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Contemplating the Water

My Dad lives across town and when I visit him, usually a couple of times a week, I often take the bus to UCLA and he picks me up by the medical buildings.  I was walking to meet him the other day, at the usual pick up place, not really paying attention because I am pretty familiar with the route, and bang; I had a head on collision with a bright blue light pole.  The thing is, I had Zelda with me…tucked safely inside my bag.

Zelda is my white cane and we hang out together, a lot.  I take her pretty much everywhere I go, but I have to be honest, I haven’t actually been using her.  I figured I could just use her when I need her, but this is some pretty skewed logic.  The nature of my blindness is that I don’t see what’s around me, so things like poles and curbs and cars, jump out at me from, seemingly, nowhere.   With the exception of walking at night and in dark spaces, I can’t really anticipate when I will need Z.  I understand that this means I probably need her all of the time, but that means accepting her and the truth of my vision loss.  How is it that I can live so deeply inside the reality of my blindness, and yet turn my back on it with such alacrity? Perhaps I am not really living inside the reality of my disease, but more tangled up in the confusion of it; one of the most difficult parts of my blindness is that I can still see.

I know that one day I will come to  accept and appreciate Zelda, but it has always been my way to come to things slowly.  I am more of a stare at the water for a really long time and contemplate the idea of putting a toe in, rather than a jump in with both feet, kind of person; honestly, I often get up and walk away from the water altogether, taking a road no-one else seems to see. I guess I am trying to walk away from my affliction, but the reality is that now, all roads lead back to RP.

Isn’t that Something Only Old People Get?

RP is anything but straightforward; it comes with lots of extras that you wouldn’t expect. A year after my diagnosis, I thought I had become pretty versed in the language of RP and what I might possibly be faced with as the disease progressed.  Night blindness had become a part of my fabric, light sensitivity was something I was constantly dealing with and I was learning how to become a vigilant scanner while walking down the street.  But, I had only skimmed the water.  And, at that time, I still had about 50 degrees of vision.  This diagram may help with the whole degrees of vision thing.

IMG_0445

As I understand it, normally sighted people have about 85 to 110 degrees of vision, well into the far peripheral.  At the time of my diagnosis in 2002, I had about 50 degrees.  Now, I have between 15 and 20 degrees, and in the RP world, that is actually pretty good. I think this diagram also shows how much of what we see is considered peripheral vision. This was a bit tangential, but I hope it gives some clarity.

So, back to 2003.  I was due for my annual visit to the retinal specialist; Dr. Heckenlively had left UCLA to work in Michigan and his patients had been split between 2 other Retina guys.  It would be my first time meeting Dr. Sarraf, and despite the fact that he would inform me of an RP extra I hadn’t expected, I loved him.  Dr. Sarraf is an incredibly kind and gentle doctor, who gets genuinely excited to give good news and is insanely smart; he is one of those serious research guys and I am lucky to be in his care.

The day I met him, he walked into the darkened room, shook my hand, chatted to me for a while and proceeded to examine my eyes.  I can’t lie, I don’t look forward to this eye exam; it hurts, a lot.  My light sensitivity is extreme on a regular day, even with sunglasses, so imagine how much a bright light aimed directly at my dilated pupils feels; it’s like someone is poking my eyes with hot needles.  Not fun.  Anyway, I digress.

Dr. Sarraf does the exam, sits back down in his chair and, in his soft and comforting way, tells me that I have cataracts in both eyes.  What the Fuck?  Cataracts?  Isn’t that something only old people get?  Apparently not.

Here is a simple explanation of cataracts: A clouding or loss of transparency of the lens in the eye as a result of tissue breakdown and protein clumping. Cataracts affect most people who live into an old age. Symptoms include double or blurred vision and sensitivity to light and glare.

Cataracts are commonly seen in RP patients, but doctors and researchers don’t seem to know why.  When Dr. Sarraf told me I had cataracts, I felt devastated, which was difficult for some people in my life to understand.  I remember my step mom telling me that it wasn’t a big deal because cataracts can eventually be treated, but for me, it was a huge deal.  Of course I knew that cataracts could be removed, but I was 34 years old and I sure as hell hadn’t met any other 34 year olds who had fucking cataracts. It was one more thing piled on top of everything else that RP had brought into my life; it was one more thing that made me feel defective, broken and different.

Almost 16 years later, the cataracts are still there, slowly ripening, but I don’t give them much thought. They are just another thing that comes with having RP, an added challenge to an already challenging disease. I don’t love that I have them, but I have accepted the cataracts as part of my RP journey.

 

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.

What do you See?

Most of the time, I find that people seem reticent to ask about my vision; I don’t know if it makes them uncomfortable or if the concept of someone being partially sighted is just too hard to grasp, but the presence of my blindness often takes up a strange space in the room. When people do ask, I actually appreciate it because it gives me an opportunity to explain how RP behaves, and also because it offers a window into what I am missing visually, by seeing what others see.

Sometimes, I’ll be walking down the street and it will occur to me that I am seeing something in a way that may be different from other people. The other day, my husband and I were out walking the dogs; he was walking about 5 feet in front of me and I was looking at the back of his head, when I realized I could only see the back of his head and a bit of the tops of his shoulders.  I asked him to stop and look at me from behind at the same distance; he could see all of me and cars parked across the street and buildings to our left and a whole lot more. When you have RP, but still have some usable vision, it can be easy to forget how much you are missing visually.  I find myself lulled into a false sense of security, so I think it is good for me to get a good kick in the pants from reality when I slip off into space.

When asked what I actually see, there are a couple of tricks that I use most often:

  1. Look straight ahead.  Stretch your arms out to the side as wide as they will go, thumbs pointed up, then slowly bring them in and stop when you can see your thumbs.  You may not have to move them at all, or maybe just a little bit, before your thumbs come into view.  When I do this exercise, I see my thumbs when they are about 7 inches apart.
  2. When you are sitting in a chair, put your hands in your lap. Keeping one hand flat, palm parallel to your lap, start raising it and stop when you can see it.  You may have to raise your hand a little bit to see it, or you may see your hands resting in your lap. When I do this exercise, I don’t see my hands until they are in line with the bottom of my nose.

These exercises are by no means definitive, but they do give an idea of what it’s like to live with diminishing peripheral vision; and, in the world of RP, I actually have a good amount of vision….I think. Every case of RP is different.

I thought I might write a series of posts that give a better idea about the mechanics of RP.  If you found this post interesting or helpful in gaining a better understanding of RP, and you would like me to write more, please let me know.  If you have any specific questions, please ask.

You can also find me on Facebook and Twitter.

 

Waiting Games

For new readers:  When I refer to Zelda, I am not talking about a pet or a child or a childhood toy I just can’t seem to part with; Zelda is my white cane.

It seems I am always waiting; waiting for the next decline in my vision, waiting to hear about that poem I submitted 6 months ago, waiting for the package to be delivered from Amazon, waiting for the scale to give me good news, waiting for the next time I get to eat, waiting for the end of the day and that bottle of wine, waiting for it all to be over. And then, suddenly, something I have been waiting for, arrives.

On Monday of this week, I spent the day seeing doctors and getting tests; nothing serious, just inconvenient, exhausting, and honestly, pretty gross, so I am not going into further detail.   That said, I spent a lovely day maneuvering through the seriously fucked up American healthcare system, without Zelda.  I have been leaving her at home a lot lately; we are having a heat wave in Hollywood and I have been feeling lazy and not wanting to carry yet another thing when I go out, so Zelda gets left behind.  I may have also still been in a tiny little bit of denial, but it really is fucking hot here.

Anyway, I made it through most of my healthcare nightmare day unscathed, until I was being escorted out of the maze of the hospital by a kind, lovely and very fast walking ultra sound tech.  I was matching her pace, feeling confident striding down the corridor, and then she said, ” take a right here”, and she turned and I didn’t and the collision ensued.  When she said to turn right “here”, I thought she meant a right turn that I saw coming up about 10 feet ahead of us; the right turn she was actually talking about, I didn’t see. I had no idea what was next to me, or how close I had been walking to the wall, or how many adjoining corridors we had passed.  When she and I collided, my confidence plummeted to the ground, but I quickly scooped it up, apologized to her and told her I have severely limited vision and I really should have been using my cane.  I felt bad about almost knocking the poor woman down, but I didn’t feel embarrassed about admitting that I had a cane and that the collision was my fault because I should have been using it; it was just the truth.  If I had taken Z with me, the tech would have walked slower and I wouldn’t have been trying to groove right alone with her, feeling dangerously confident about my non-existent visual capabilities.  I took my time for the remainder of my walk through the hospital, and found a comfy chair to settle into while I waited for my husband to pick me up.

Yesterday, I was taking one of my frequent walks to the grocery store, sans Zelda, for the same reasons listed above.  As I approached the first street crossing, I thought I heard someone walking near me, but I had no idea how near. I slowed my pace a little, tuning my ears to the sounds of footfalls and rustling clothing, but when I got to the corner and reached for the cross button, I bumped into a woman who must have been just inches away from me. I still get fooled by RP a lot of the time and think people and objects can’t be as close to me as they actually are; my ears are not that well trained, yet.

The woman was super nice and friendly and didn’t seem to think twice about our collision, but it gave me pause. I realized, or perhaps I have known for a while, that my vision has gotten worse.  Decline in vision is something that someone with RP is always waiting for, but in my case there has been a lot of uncertainty about whether or not it is actually happening.  I am fortunate that my vision loss has had a very slow progression, and there have been so many times when I feel pretty convinced that I am not seeing as well,  and it turns out that my vision is stable.  This time is different. I feel the world pressing in against me; the shrinking circles of my vision have become more prominent.  But, somehow, being in the center of the decline, looking at the world with the heightened sensation of tunnel vision, I don’t feel afraid.  I have been waiting for this.

During the rest of my sojourn to the grocery store, I must have had at least 10 near collisions and people coming at me from, seemingly, out of nowhere.  I kept thinking over and over again, “I wish I had Zelda with me”.  I think I’ll take her out today, no matter how hot it is.

 

Writing Sample Part 4 – Surrender

It’s impossible to think about going blind without thinking about loss.  When you have a degenerative disease like RP, the loss comes in unpredictable bursts that steal pleasures and pluck freedom from disappearing fingers.  In the weeks just after my diagnosis, I thought a lot about how my life would change and what blindness would take from me; I was most afraid of losing the ability to read the printed word.

 I have always loved words; the way they can simultaneously smooth and sharpen the page. A life that leaves me unable to see the printed word is unimaginable.  I feel paralyzed by the thought of no longer seeing how the shapes of the words meld and flow.  I wonder what kind of writer I will become when the pages turn dark. Perhaps I will learn to rely solely on the musicality of language; the taste of the words in my mouth and the feel of their vibration on my lips. I am sorrowful when I think of no longer taking the words in with my eyes, but I will read voraciously until the darkness has swallowed them up.

Although the loss of the ability to read was my greatest fear after RP became part of my life, one of the most significant rites of passage in an RPer’s life is giving up driving.  I was a terrible driver, for now obvious reasons, and always felt anxious behind the wheel, but I have definitely felt the loss of freedom since I gave up my car for less convenient forms of transportation.

Because one of the first things that alerted me, and the doctors, to my RP was decreased night vision, the consequential difficulty led me to stop driving after dark immediately following my diagnosis. The combination of the darkness and the glaring lights of oncoming cars made it so I simply couldn’t see well enough to drive safely. In truth, it hadn’t been safe for years:  I had just been lucky.

I had also, in daylight hours, had several run-ins with walls, poles and curbs that seemed to jump out at me from nowhere.  This started serious contemplation of whether I should continue driving at all.  Walls and curbs only hurt my car, but what if it was a child or an animal that I didn’t see?  I couldn’t bear the responsibility of hurting someone just because I didn’t want to give up my car; so, about seven months after my diagnosis, I gave up driving altogether.

When I decided to give up driving, I chose to surrender my license and make the rite of passage official; I had no reason to hold onto it when I knew I would never drive again.  I needed to make it real.  I suppose it was my way of dealing with the loss head on, not allowing myself to live in denial about the gravity of my disease and how it was going to continue to change my life.  I was empowered because I took charge of the decision and I knew I was doing the right and responsible thing.  I felt the loss, but it was intermingled with a sense of relief.

It the first couple of months without my car, I joked about how lucky I was that I never had to be the designated driver and how I had always been a lousy driver anyway, so no big deal that I would never drive again.  True statements, but I also started to see how my disease was a burden to the those around me, and how I was going to become less and less practically useful as the years and the RP progressed.

I hated listening to my family agonize over who would drive me home after a family dinner, knowing how tired they all were.  I hated knowing that once I arrived somewhere, I was stuck until someone else was ready to leave; I had lost the freedom to come and go as I chose and because of this I became more reclusive.  I didn’t want anyone to have to take care of me.  I was determined to take care of myself.

My family never complained and I knew that they were just trying to make things easier for me, but I needed to find a way to assert my independence.  I had always had a fiercely independent nature and I wasn’t going to let RP rob me of that.  So, I learned the ins and outs of the LA transport system, which is no small feat given that it is the worst in the country.  I remember the first months of riding the bus, looking out the window at the enraged faces of the drivers in their cars, and feeling lucky that I got to spend my ride home listening to music, far away from the stresses of road rage and blaring horns.

Surrendering my license ended up saving an important part of who I am, and showed me that my determination means something.  And let’s be honest, L.A. busses are full of stories dying to be told.

Writing Sample Part 3 – Signs

We all look back at our lives, pick them apart and look for clues that would have helped us along the way, told us what was up ahead.  We discover things about ourselves that may have been present or growing since childhood, and inevitably think about the signs we might have missed.

A few months ago, I was going through some old family photos, and I came across a picture that was taken on a beach in Mexico when I was about three years old.  My eyes were squinted against the glare of the sun and I was reaching for my mother’s sunglasses, perched on a rock nearby.  I looked desperate to escape the bright sunlight and it is a look I recognize, a feeling I have experienced for years.  Growing up in California, my family was always going to the beach.  My parents and my siblings loved spending hours in the sun, but I preferred cloudy days.  I was called strange, moody and different, but even then, the sun hurt my eyes.

As I got older, I developed a reputation for being clumsy; I was always tripping and stubbing my toes and knocking things over. I couldn’t hit a softball in P.E. class or catch the ball when I was forced into the outfield.  I appeared careless, un-athletic, lost in day dreams; I didn’t know it then, but my retinas had begun to die a slow death.

I remember an afternoon when I was learning to drive; I was in the car with my mom, and she began shrieking that I was driving too close to the edge of the road and we were going to go off the cliff.  My mom was prone to dramatic expression; there wasn’t really a cliff, just a five-inch drop off the road into the dirt.  She thought I wasn’t paying attention, but actually, I had no idea how close I was to the edge.  I couldn’t see the side of the road.

Into my 20’s I continued to trip and fall and live up to my reputation as either the clumsy day dreamer, or the newer moniker of drunk girl.  I had a friend tell me I was the only 24-year-old she knew who actually fell down and skinned her knees.  I missed curbs, crashed into street lamps and collided with pedestrians racing down the Boston sidewalks to escape the cold.  I thought perhaps my friends were right and  I was drinking too much; I had no idea that the edges of my vision were disappearing.

For years, I nursed the bruises that peppered my skin and laughed along with my friends about my clumsiness.  I chastised myself for being careless and inattentive.  After my RP diagnosis, I became diligent in searching for current markers of my deteriorating vision. I notice how the glare of the sun gets meaner and how once effortless tasks are becoming more difficult.  I feel the light slipping away every time I call out my husband’s name, unable to find him right in front of me.  I feel helpless and terrified as the darkness slowly swallows up the contours of the world.

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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