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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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blind women

Genetics and RP

I am, by no means, an expert when it comes to the science of RP, but I want to try to explain the bits that I do understand.  Anyone who has RP and reads my blog, please don’t hesitate to share your knowledge in the comments section; I have a lot to learn.

RP is most often characterized as an inherited disease.  There are 3 different inheritance patterns: Autosomal Dominant; in which one parent and one or more children are affected. Autosomal Recessive; in which both parents are carriers, but unaffected, and children can either be affected or just carriers or completely unaffected.  X-Linked; in which the disease is passed only from the mother and affects sons, more frequently and severely, than daughters. More than 60 gene mutations have been linked to these inheritance patterns.  More than 20 are associated with the Autosomal Dominant pattern, with mutations in the RHO gene accounting for 20 to 30 percent of all cases.  At least 35 genes have been associated with the Autosomal Recessive patterns, with mutations in the USH-2A gene accounting for 10 to 15 percent of all cases.  At lease 6 gene mutations are thought to cause X-Linked RP, with mutations in the RPGR and RP2 genes together, counting for most cases.  My understanding is that identifying the specific gene mutations is what will enable researchers to create gene therapies for all 3 types of RP.

There is a fourth group of people affected with RP: In 10 to 40 percent of  all cases of RP, only one family member is affected; in these families, the disorder is described as Simplex. It is difficult to determine the inheritance pattern of Simplex cases because those affected can have either no affected relatives, or be unaware of other affected relatives. Some Simplex cases can also result from a new gene mutation that is only present in the affected individual.  It seems to me that these Simplex people are kind of screwed; if no inheritance pattern or specific gene mutations can be located, then gene therapies can’t be applied to these patients.  I am, most likely, part of this Simplex group.

As far as I know, I am the only person in my family with RP.  My father’s father may have exhibited some signs that he had trouble with his eyes (primarily light sensitivity), but I think I was just trying to make sense of why I ended up with this crazy disease.  I wrote a poem about it that was published last year in Foxglove Journal.  Is it possible that I have Autosomal Recessive RP?  Possible, but unlikely.  And, my mom died 30 years ago, so there is no way I could find out if she was a carrier.

I just read about the Simplex cases this morning, but now Dr. Saraff’s  explanation about me having the most common form of RP, and his not recommending genetic testing, makes more sense. I feel like I am on an RP island, but apparently, there are a lot of people on the island with me. In some ways, my situation seems grim, but I don’t feel grim.  I still have hope, but after my appointment last week, I put it back on a shelf, out of sight and reach, which is exactly where I need it to be.

 

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.

The Human Condition

I feel buried beneath the weight of a fog that permeates my thoughts. I am on a dangerous precipice between blindness and sight, my strides uncertain and guided by terror. I find myself again and again, tangled up in a desire to escape the person I have always been and the disease that murders my eyes with slow, but voracious determination.  I long to be extraordinary and graceful, but in wakefulness I am defective and covered in unsightly cracks.

As a child I struggled to catch up, racing and climbing on legs that weren’t strong enough, pushing against the barriers of a mind that wasn’t expansive enough.  In conversation, unable to keep up, I float away, tangled in an extraordinary web of words that sparkle and splash the edges of my consciousness.  I dream in language rather than concepts or theory.  I don’t get your jokes or understand the machinations of a corrupt government.  I drift because the landing is too jarring and too cruel.

I have mostly felt like something to be kept hidden in the shadows, to remain unmentioned and shackled.  Beneath the lights, I am beaten with rods of ridicule and pity.  How sad that she is the blind one, the fat one, the ugly one, the broken one. But I have learned to love the outskirts, to languish in the disappearing edges of expectation .  I have learned that I can be the quiet one, and in the silence there is safety and a stillness that muffles the chaos of loathing.

I know that I am not unique or special; just a shadow of a woman suffering, like everyone, from the malaise of the human condition.  It’s just that sometimes, in moments that glimmer and tantalize, I dream that I will wake up, no longer blind, no longer human.

Am I too Negative?

Something has been on my mind lately and  I have been hesitant to write about it in this particular forum; I worry that it may upset some people, but I feel compelled, so I will write it and face the decision to press publish (or not) when the time comes.

I read a lot of blogs written by other blind and visually impaired people and I have noticed that most of them seem to be much more positive than mine.  Am I too negative?  Am I doing a disservice to the blind community by writing more about the struggles than the triumphs on my road to blindness?  I try to make it clear that I am not speaking for the blind community, but is it clear enough?  Am I inadvertently hurting people?  Am I casting a harmful light?  Or is it clear that what I write is simply my personal truth and nothing more?

As an artist, I have always felt that the best art comes from a place of total honesty, and whether that place is positive or negative doesn’t matter as long as it is true.  But, is this an irresponsible way of looking at things when it comes to writing about disability? I don’t want to hurt anyone or instill fear or further stereotypes. On the other hand, isn’t it my responsibility to myself as a writer, to write my story from my own perspective, no matter how uncomfortable it may be?

When I started writing about RP and going blind, I made a promise to myself that I was going to convey my story in a way that was neither overly “woe is me”, nor overly “I can overcome anything and screw this pesky blindness”.  As my story progressed, I allowed my nature to conduct my journey and my experiences, and my nature does veer toward the darker side of things.  I have never been the kind of person who races to the top of the mountain and shouts my triumph to the world; I am more the person who contemplates the mountain itself and explores its darkness.  I am in no way knocking the person who races to the top of the mountain; I actually admire that person.  I am just not wired to climb.

The way I write about my journey into blindness isn’t dictated by blindness itself, but by the nature of who I am, and that is something that was there long before I even knew I was going blind. As an artist, I am compelled to write from the center of my own truth, and that is going to make some people uncomfortable, but I am a writer, not an advocate.  I believe that art, in all it’s forms, is powerful because it inspires all kinds of feelings. I don’t set out to hurt anyone or to make anyone uncomfortable, but I do choose to write my truth and sometimes, it will be messy and hard to swallow.  My writing isn’t for everyone, but it isn’t meant to be.

Am I being irresponsible? naive?  I just don’t know.

 

 

Reluctant Side Kick

*To new readers: When I refer to Zelda (or Z), I am writing about my white cane.  Thank you for reading “Stories from the Edge of Blindness”

Reluctant Side Kick

Zelda and I have reconciled, again.  I suppose we are together for better or worse, but I am still her somewhat reluctant side kick, reluctant to give in and give over to her, to let her lead the way, but, my self guidance system is seriously faulty.

Joe and I were grocery shopping the other day – before I rescued Z from beneath a mound of coats and hats and bags, on the hat rack and I collided with a shopping cart that someone had left in the middle of an exit isle.  It wasn’t a bad collision; I was just left with some bruises on my legs, but when it happened, I looked up at Joe and said,”I guess I could have used Zelda today”.  He just smiled and nodded and we left the store.

As a result of this particular collision, I had to admit that I have been treating Joe unfairly.  Whenever we go out together, I depend on him to keep me from collisions and falls and the always possible rage of strangers, but what about Zelda?  If I had Z with me, Joe could shop and walk and roam to his heart’s content without having to worry about me the whole time.  I have been so selfish.  I have been telling myself that using or not using Zelda only affects me, and that simply isn’t true.

So, I have put Zelda back on a prominent hook, next to my purse and sweater, so I will be reminded to always bring her with me. I don’t want to need her, but sometimes I know I will, and when I no longer have the amount of useable vision I have now, I will be grateful that she and I have become well acquainted.

Being a Writer

 

While reading a friend’s blog this morning (The Incurable Dreamer), I found myself thinking about what it means to be a writer.  She is a brilliant writer and has chosen to dedicate herself to it, which makes me happy for her, and for me, and everyone who gets to read her stuff. She gets what it means to be a writer.

I come from a family that is overflowing with achievement; there are doctors, lawyers, city planners and politicians, and then there is me.  Whenever I meet a friend of my parents (invariably another doctor), they always get excited and ask me if I am the “doctor daughter”, and then look uncomfortable and slightly disappointed when I tell them that I am a writer; when I tell them I write poetry, they can’t get away fast enough.  I don’t fit into the perfect package that my family represents and that makes people uncomfortable; and, ok,  having an abundance of tattoos may also factor into the discomfort thing a tiny bit, but I kind of love that.

Don’t get me wrong, my family is supportive of my writing (not so much with the tattoos), but for so long, I felt like being a writer wasn’t a viable thing because I hadn’t gone to school to study how to be a writer and because I wasn’t getting published in all the top magazines; I had no clear signs of accomplishment and that meant I was a big pile of nothing sitting off in the corner while my accomplished siblings were being praised for all of their hard work, as if I could never understand what it means to work hard. My parents and my brothers and sisters are all super amazing people who do work really hard and I am proud of them, but I am not nothing. I am a writer.  I am a writer; but what does that mean? It took me a long time to figure it out and to be able to talk about it with confidence.

For years, I struggled with the question of whether or not I was actually a writer and doubted myself whenever I told people that I was. I have written everything from poetry to one-act plays, but until recently my writing was sporadic.  I was a writer in moniker and desire, but not in practice. I wasn’t writing.

I didn’t believe I had the credentials to be a writer, so I went about trying to find a real job.  I found a thousand real jobs; some I liked, some I hated, but none made me feel fulfilled, or like I was doing the thing I was meant to do.  I knew, in my heart, that I was a writer.  I had been a writer since I was 6 years old when I wrote a Thanksgiving story about a turkey who commits suicide; it was called “The Sad Turkey”, and it was about a turkey who decided to commit suicide because he knew he would be killed for some fancy Thanksgiving dinner.  Pretty imaginative for a 6-year-old, right?

When I stopped working a regular job in 2009, I had big plans. I was going to write a memoir and get published; instead, I made a lot of popcorn and watched a lot of T.V.  When I started this blog, I was certain that it would be the answer to my writing woes; it would help me get my memoir done and get me published.  But, I barely posted; months went by and I didn’t go near the computer because I felt guilty for not writing blog posts.

I know now that I needed the time to adjust to the reason that I had stopped working a regular job; I had to give myself time to come to terms with the fact that RP had started to affect my life in more impactful ways and I wasn’t ready to write about it on a regular basis.  I had been given a gift of sorts, but I wasn’t ready to open it up.

Years went by and I still wasn’t writing regularly, even though I know that when I am writing I feel full and satisfied.  I had stopped writing poetry because I felt that it would never be lucrative and I really had no business calling myself a poet given that I have no formal education in writing.  The problem was that poetry is my first love, as a writer.  Poetry is where my pulse lives and it is what inspires me.

A few years ago, I decided to stop stressing about the blog and start writing poetry again. I felt inspired and satiated.  I wrote tons of poems and edited a bunch of old pieces; I got motivated and started to enter contests and submit my work.  I returned to the blog with a new energy and I dedicated myself to writing.

Degrees and  publication credits don’t make me a writer.  I am a writer simply because I write.  I have discovered that, for me, being a writer means doing the writing, fulfilling that part of myself that only writing can fulfill ; it is reaching into your darkness and your light, being brave enough to face whatever you may find there, and sharing those discoveries. Being a writer is hard work and often lonely work, but it is the work that speaks to me.

I am not nothing.  I am a writer.

 

Under the Lacquer – Part 2

I haven’t had the luxury of traveling or living in other countries, but what I learn from the people I meet in my regular, run of the mill life, enriches who I am and gives me images of a world I wouldn’t have without having met them.  I always feel like a person can give me a much better feeling of a place than a relic or even nature.

The entire time I have been frequenting my local nail shop, it has been managed by 2 sisters, Carol and Jean. The shop is a bit of a family affair; at least 2 other sisters and a cousin have worked there at one time or another.  They are a very close-knit family.

On one of the occasions when my regular pedicurist  was in Vietnam for a month, I had the pleasure of spending an hour and a bit with the older of the two sisters.  I didn’t know Carol very well at the time; I had gotten pedicures from her sister, Jean, and knew about Jean’s children and how they spent holidays together with all the family that lives in California, but I only knew that Carol was Jean’s older sister and that she often hosted these family holiday get togethers at her house.

That day, I ended up staying a while after my pedicure was over, and Carol talked to me about her life; her life before California. Two of the things I often ask people who I know are from different countries, are how long they have been in the states and have they only lived in California.  I find that asking these questions can often lead to someone sharing stories about where they are from; talking about life before nail shops and Hollywood.

I am not sure where in Vietnam Carol lived, but I do know that her husband was a soldier during the war.  I always felt as if the war must have profoundly impacted her  entire family, given the kind of bond that they all share. Carol didn’t talk about the specifics of the war itself, as Kim has, but she did tell me that her husband was part of a program that brought Vietnamese soldiers to the United States for asylum.  Carol’s husband was imprisoned by the VC for years and upon his release, he and Carol and their family were brought to safety.

It might be that Carol was able to bring most of the rest of her family here to California because she had been granted citizenship, but I may never know.  All I know is that most of the siblings are here, but her parents are still in Vietnam; at least one of the sisters goes to Vietnam every year to visit the parents, and this year they are all going because their father is 93 and very ill.

Carol is a remarkable woman.  The minute you meet her, you can feel her strength.  She is in charge and everyone around her knows it.  I felt really honored that she chose to tell me even a small piece of her story and I hope that I get a chance to spend more time with her and learn more about who she is and what has shaped her life.

I may never have the good fortune to actually travel to places like Vietnam, but I do have the good fortune of learning about and getting to know people who have come to California from vastly different countries and cultures.  I have learned that I don’t have to actually be in a place to feel it; I only have to take the time to get to know someone who has worn the fabric of that culture.

I guess I also, on some level, have to feel grateful for RP.  Although I think I have always had a curiosity and interest in people and their cultures, if I didn’t have RP, I may just be another Angeleno who spends their life in a car and in a hurry.  RP forces me to slow down and that suits my nature. I am grateful for all of the opportunities I have had and will have to truly know people who come onto my path, even if I can’t see them until they also take the time to slow down and come out of the shadows of the periphery.

Under the Lacquer – Part 1

Because I have been stripped of my wheels in an automobilecentric city, and because I am losing my vision at an unpredictable rate, I am happily forced to pay more attention to the world directly around me.  I have always had an interest in people and all the ways that others are different from me, and I haven’t ever been the person who is in a rush, so I stop and take the time to at least try to truly see. It is an ironically positive side of RP and it brings things into my life that may not have been there if I didn’t have RP.

Every two (or three if I am lazy and/or forgetful) weeks, I go to the nail shop around the corner for a pedicure. It isn’t because I am a princess, but because I can’t see my toes clearly enough to cut the nails in a way that even approaches presentable. I have been a customer at my local nail shop for over 10 years, and, as most nail shops in Los Angeles, it is staffed completely by Vietnamese women.

Every woman in the shop has an American name, and although I ask them to tell me their Vietnamese names, they are often reluctant to do so. When they do tell me, the names are often hard for me to pronounce, but I keep working at it because I want to be able to call them by the names they were originally given.

My tendency is to stick with one person for my pedicures; I like to establish a relationship with the woman who is touching my feet every two weeks. In my 10 years at All Star Nails, I have had just two different regular pedicurists (I have decided that is a word); first, it was Michelle (An Mei) who left because of a back problem, and for the past year, I have been seeing Kim.

Kim’s Vietnamese name is Nguyet; it means moon. It is a perfect name for Kim, as her mother must have known.  She is like the moon; she has a beautiful and vivacious light that makes it a joy to be around her, but she isn’t all flowers and sunshine.  Nguyet has an edginess to her that I equate to the darkness around the moon; it is where her secrets are kept and it is where her sarcasm lies in wait for those she truly knows and likes.  She has a quick, brilliant, and sometimes strange sense of humor and she loves being alive. The more I learn about her, the more I want to know, and I love her name, but Nguyet does not; she says it is too hard in the mouth, so I continue to call her Kim.

Kim is tiny in stature; there just isn’t another word for it.  She is 4’8 with a 7’8 personality.  The first time she did my pedicure was by chance; I hadn’t called for an appointment and she was the person who was available when I walked in.  I had wanted to meet her for a long time.  She is definitely the coolest looking pedicurist in the shop.  She has a short bob and an air about her that feels just slightly punk rock.  She wears a lot of printed leggings and a jade bracelet that has been on her wrist for over fifteen years; it is her version of a piercing.  During our first hour together, she told me that having the bracelet put on was very painful; there is no clasp, so it had to be forced on.  Kim told me that they use oils to help get it on, but her hand still had to be contorted and squished and after it was on, there was no way she was ever taking it off.  See, pretty damn punk rock; but, now I know that it isn’t Kim’s bracelet that makes her cool, it is who she is and the life that has shaped her.

Kim came to the U.S about 12 years ago, with her husband and two small sons; she later had a daughter who was born in the U.S.  She loves to show me pictures of her family and tell me about her kids: I have seen videos of her middle son giving his 8th grade commencement speech, photos of her oldest son with his new guitar and at his high school graduation, and videos of her 9-year-old daughter who is a total fire cracker, just like her mom.  Kim is always joking about her husband being so old – he is 13 years older than her – but I know that she adores him and that they are a super close family.

Just after Valentines Day, I was getting a pedicure and Kim brought out her phone to show me some new photos that her middle son had taken.  They were of Kim in her garden, sitting beside what looked like a giant topiary heart.  Her husband had apparently let the grass grow long so he could mow it and leave the heart shape in the center of the garden.  Kim said she doesn’t care about Valentines Day (and I am with her, no V day for me either), but I could tell she was thrilled at the romantic gesture from her husband.  He is clearly the softy in the family and she loves it.

Another afternoon, Kim showed me pictures of her family in Vietnam.  She was born and grew up in a beautiful city near the water; she met her husband there and her first two children were born there; her parents and most of her siblings still live there. In the photos, I recognized her kids and her husband and 2 older people I assumed were her parents, but there were also some people her age; one of them was in a wheel chair.  I asked about him and, at the time, she just said it was her oldest brother and he had been hurt.  I didn’t press her.

More recently, Kim has been taking more about her life as a child in Vietnam.  She told me that her brother is in the wheel chair because of a bomb.  Their city was bombed by the VC, and at the time of the bombing, Kim, her brother and father and 2 of her sisters were in the blast radius.  They were all injured.  Her older brother was hit in the back with shrapnel and a piece lodged in his spine; he was 5 years old and he would never walk again.  One of her sisters almost lost her arm, but was taken to a nearby hospital by helicopter, where they managed to save it.  Kim was only 3 years old at the time of the bombing and was left with shrapnel scars all over her legs.  She recalled to me how much blood there was and how afraid they all were.  She told me that her father still suffers from the memory of that day and that is plays like a film in his head over and over again.  One moment, they were a family in town for the afternoon and the next their lives were altered in every conceivable way, forever.

It is remarkable to me that I live in Hollywood and every two weeks I get to spend an hour or two with a woman who has seen things I can’t even begin to imagine; and yet we laugh and joke and have truly become friends.  Kim is one of the strongest and funniest people I have ever known and I feel so lucky to have met her and to be learning from her; I feel grateful that she has chosen to share the stories of her life with me.

 

 

The Iron Fist Came Crashing Down

Last night, Joe and I were finishing season 1 of Iron Fist; we love all that Marvel Super Hero stuff, and although Dare Devil (obviously) holds a special place in my heart, I really like the Immortal Iron Fist (a.k.a.Danny Rand, a.k.a. Loras Tyrell).

Anyway, there we were, awaiting the fate of Madam Gao and all of a sudden I found myself staring into the, quite lovely, eyes of Danny Rand and felt the weight of the Iron Fist smashing me back into reality.  I asked Joe to pause the show.

I stared at the paused screen and did what I have been doing a hundred times a day for weeks.  I looked straight ahead and tried to determine what I could see in the peripheral field.  Joe was sitting next to me on the couch, about 2 feet to my right, and the dogs were on either side of me snuggled in close.  I asked Joe to look into the eyes of The Immortal Iron Fist and tell me if he could see me and if he could see the dogs.  He said he could, and I burst into tears, sobbing and understanding that all of the self-administered testing of my vision, and attempts at trying to prove to myself that I don’t need Zelda, were in vain.

When I looked at the screen, dead ahead of me, I couldn’t see Joe or the dogs or the picture above the T.V., or my fingers resting on my own shoulder, or even the entire T.V. screen. It was like I was being told for the first time that I am legally blind; the mask of the normally sighted woman, that I have donned for years, was torn away and smashed to pieces.

I think it is time for me to stop asking people what they can see; time to stop asking myself and just accept that no matter what I can still see, it isn’t enough to keep me safe.  I need to give Zelda another chance.

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