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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.

Month

January 2018

At Home in the Darkness

In the past few months, years after the inception of “Stories from the Edge of Blindness”, I started to really explore the blogging world rather than just stand on the outside in abject terror, feeling like a loser.  I started to interact and exercise my voice. I started to read some amazing blogs, written by writers I have come to admire; writers who teach me things and make me think.  One of my favorite blogs is Tom Being Tom, and recently, Tom wrote something that has had me thinking about being, becoming and changing, as the years of my life traverse, meander, sink  and soar. His post is, “We Are Not But One Thing”, and when you read it, you will understand why it got me thinking.  Tom is an intelligent and thoughtful writer.  He presents new ways of thinking about and looking at life. He is a hopeful realist.
Continue reading “At Home in the Darkness”

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Stuck in the Escape

I feel sad this morning.  It isn’t new, feeling sad and writing about sadness; I do that a lot.  But, today it isn’t just sadness, or darkness or blindness, that is in my head and on my skin, it is shame. I have written about shame before, in regard to my disease and feeling different, but the shame I feel today has nothing to do with RP; it came long before blindness.
Continue reading “Stuck in the Escape”

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.

 

On the Edge

This has been a monumentally good week for me in regard to my vision.  Finding out that I will likely maintain enough sight to be able to read and write and see the faces of those I love, has altered my path and left me feeling immense gratitude.  I have allowed myself to feel genuinely happy about this, which isn’t something I always do.  However, I am also left reflecting on how RP has and will always impact my life.

I have spent so many years on the edge of blindness, agonizing about living in this confusing place between being sighted and being blind.  I have held my breath in anticipation of being pulled from the precipice by inevitable darkness.  I have been on a wire, balancing precariously, waiting.  Now, knowing that I may never go completely blind, I am elated, but still on the wire, still faced with the confusions that come with being partially sighted.  I had resigned myself to the future I thought RP had in store for me, but now I have to figure out how to settle onto this weird middle ground, how to resign myself to the fact that I may always reside on the edge of blindness.

I am, of course, grateful for the vision I maintain, but living in what I have called the “purgatory of sight”, comes with its own set of challenges.  I am not a religious person by any means, but from what I have read, purgatory seems like a pretty fucked up place to be.  It is neither here nor there.  It is nowhere and nothing.  It is a place of invisibility, which does not hold the cool factor of a cloak of invisibility, because you can’t take it off when you feel like being part of the clear and whole picture. There is a picture and I can see it, but it will never be clear and it will never be whole. I feel stuck, trapped, truncated. But, this isn’t new.  This has been my reality for years.  What’s different is that I have been given a choice I didn’t know I had a week ago.  I can choose to focus on the good fortune of the sight I still have and may have for the rest of my life.

The landscape in front of me has at once changed and stayed the same.  I still have RP.  I am still blind and the reality of my brand of blindness is incredibly confusing.  I am still on the edge, but perhaps it is where I have always been, long before RP came into my life.  It is a crazy view from where I stand, but it is the view that gives me the words that I write and see on the page.  I can see the words on the page.  I will be able to see the words on the page tomorrow and maybe for the rest of my life.  I don’t take this lightly. I am so incredibly lucky.

 

The Good News and the Bad News (which really isn’t so bad)

I am so incredibly grateful and overwhelmed for the outpouring of care and support I have received since my last post.  I am a lucky woman!

This has been a month of having my whole body poked and prodded and getting preventative procedures, but today I am finally home and done with all of it and wanted to write an update regarding my visit to the retinal specialist.

I will start by saying, it was a good visit.  That said, it was an incredibly long day in which my eyes were put through hell, but I am a pro at this point, so I take it all pretty much in stride.

I won’t give a detailed account of the 5 hours I spent at the Eye Institute, but I do want to write a little bit about the research and gene therapy thing.  Truthfully, I am still learning about the science of it all, and if it interests anyone, I will share information as I learn more, but I did learn a little yesterday, which leads me to more questions, which is I guess the nature of science, in some ways.

I have met others with RP who feel the way I do about following the research too closely and many who are very invested in following it closely.  My experience at Jules Stein yesterday is a good example of why I personally haven’t chosen to follow the research.  Part of it is emotion and part of it is trust and good fortune.  I was in the waiting room (about half of the visit is always spent waiting) and started to think about this new FDA approved gene therapy, which led me to start thinking about what it would feel like to have the independence of being fully sighted, and I started to feel excited and hopeful.  How cool would it be, after all of these years, to be able to feel the ease and convenience of a sighted life?  Then, I overheard bits of a conversation; a patient and a research assistant were sitting near me going over release forms for the patient to be enrolled in a research study.  During the conversation, the woman asked something about retinal diseases, other than her own, that were part of the study; the research assistant said something along the lines of,” there are other diseases, like Retinitis Pigmentosa, but they are much more difficult when it comes to genetic research”. I don’t know if this is true, but it was enough to  knock me down from the heights of hope, and that fall is a long fucking way down.  I started crying right there in the waiting room (yes, I am that person) and thinking of a way to describe the feeling of plummeting from hope into despair in regard to the research issue; perhaps it is like being offered something sweet over and over again and every time, you are left with a mouthful of bitterness.  It was a good reminder for me of why I don’t keep up with research.  I am not criticizing those who do; we all have our own ways of grieving and coping and we can only be true to ourselves in that respect. There is no right way to do it; there is just what works for us as individuals.

I was feeling pretty depleted, but I decided I would talk to Dr Sarraf anyway and get his take on it.  I asked about the gene therapy I had read about in the New York Times, and about being referred for genetic testing; he told me that there is no existing therapy for me, that I have the most common form of RP and therefore the genes related to it are more difficult to pinpoint (this is not a direct quote).  I felt thoroughly un-special. He said that he doesn’t recommend genetic testing right now, but let’s see what happens in the next few years and if there are trials that are looking hopeful, we can talk about it again.  So, this is where the trust and good fortune part come in.  Dr. Sarraf is one of the two leading researchers at UCLA,  in  the study of degenerative retinal disease.  I am incredibly lucky to be one of his patients, and I trust that if there is a treatment for me, he will make sure I know about it and get in on it.  I am lucky to be able to leave the research stuff in his hands, while I go about the life with RP stuff.

I think Dr, Sarraf was feeling bad about not being able to offer me anything in the realm of treatment, but, he gave me some really good news! He told me that given the fact that the RP hasn’t started invading my central vision, he thinks there is a good chance that I may never go completely blind.  This is incredible; it means I will be able to keep reading and writing with the use of my eyes.  It means I can look at my husband’s beautiful face and into the loving eyes of my pugs and my cats, for many years to come.  It means I will be able to travel and see things I have never seen. It is not lost on me how incredibly fortunate I am.

Dr.  Sarraf’s parting words to me were,” So, the good news is that your central vision isn’t deteriorating( with the exception of a very minor change in your right eye), and the bad new is that you have RP and very limited peripheral vision”.  I already knew the bad news, and the good news, at this point, far outweighs the bad.

I am not particularly brave or strong; no more than anyone else and much less than many.  I am finding my ways of coping with my personal journey through this crazy disease and through life.  It is my way to look at and embrace the messy things that face me; I have learned that trying to escape them doesn’t work.  Some days are great and some days seriously suck, but I choose to muddle through and write about it honestly.  I try to keep my blinkered vision at bay and be as truthful as I can, as a woman and as an artist.  I am grateful and I am so damn lucky!

Now, it is time for me to rest my eyes,  and treat them with kindness, respect and gratitude.  Thank you again for all of the amazing support.  And, please please don’t hesitate to ask questions; I know I always inevitably leave stuff out.

 

The Goodness of People

I was trying to keep to a self enforced schedule of posting on Sundays and Wednesdays, but this is a weird week for me, so here I am on a Tuesday. And, this post may be a bit all over the place.

January is typically a rough month for me. The 2nd is the anniversary of my Mom’s death and at some point every January, I see my retinal specialist; this year, it is today.  These are, and will remain, difficult things for me, but this year, I came into January feeling more positive and less full of dread.  Yesterday, I read an important piece by, “A Gypsy’s Tale“, and it helped me see that the reason I am feeling different this year is due, in large part, to the goodness of others.  I hope you will take the time to read her extraordinary post.

Every year, when I see Dr. Sarraf, I go with some anxiety.  For a number of years, I went to see him and he told me (always with great enthusiasm) that my vision was stable.  I felt incredibly lucky, but began to expect the news; last year, he told me that my vision had deteriorated.  It may sound a bit nuts that I have to be told whether or not my vision has gotten worse, but it is actually really hard to tell.  RP progresses pretty slowly (in my case anyway) and there have been years when I was convinced it had gotten worse when it hadn’t, and years I thought it was stable and it wasn’t.  Basically, you don’t know what you aren’t seeing because you can’t see it.  For years, I thought I was seeing normally and it turned out I had this crazy disease and was going blind.  Anyway, I digress.

Today, I am anxious, but ready for whatever he may tell me and feeling fortunate for all of the love, support, kindness and goodness that I receive from others.  I feel fortunate to be a part of an extraordinary community of kind, caring and intelligent people. I feel fortunate to have a doctor like Dr. Sarraf and to be able to go to one of the best eye institutes in the country.  I feel grateful for the vision I still have.

Todays visit will also be different because I am going to talk to Dr. Sarraf about genetic research.  My tendency has been to keep myself away from news about the research on retinal disease; I followed it in the beginning, but it felt as if I was being given false hope over and over again, and at the end of the day, I still had RP and had to learn to live with it.  Recently, there has been a lot in the news about a new FDA approved gene therapy that treats retinal dystrophy (basically, deterioration of the retina), but that term is a huge umbrella over a multitude of genetic mutations.  It may sound nuts, but when I heard the news, I wasn’t excited; I took it in stride, but my step mom read about it and got all excited and saved the article for me and  insisted I talk to Dr. Sarraf.  It is hard for her to understand that I don’t really want to know about the research, that all of these years, I have been living with RP and trying to accept the reality of my vision and how that impacts my life; but, this was the first time she has ever talked to me about RP (it can be easy to ignore – that is another post for another time), and I was touched by her enthusiasm.

So, today I will talk to Dr. Sarraf about genetics and ask him to refer me for genetic testing. I am stepping onto a new path in my RP journey, but I am curious and open to learning more and it is probably about time.

 

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.

Sidlak Poetry

Sidlak poetry (sid/lak) is a structured poetry consisting of 5 lines with 3-5-7-9syllables AND A COLOR. The last line must be a COLOR that describes the whole poem or the feelings of the writer.

I just learned about Sidlak poetry from the love blogger, https://mukhamani.wordpress.com and I thought I would give it a go.  I am not sure if I am totally following the form, but this is my attempt:

She sinks down

Under the deluge

Safe in the branches of earth

The black threads of noise falling silent

I love exercises like this! Anyone who wants to, please give it a try, and post it in the comments section!!!

 

Bad Eye Day

Almost every RPer I have met has bad eye days; my bad eye days are most often due to light exposure or over use of my eyes.  I have been thinking about this lately because I have gotten some questions about my reading and writing capabilities, in regard to my vision loss, and because I have been having more bad eye days.

I have always loved to read.  When I was a child and my family took plane rides, we all got to choose a book to take with us on the plane; it was my favorite thing about traveling.  I loved going to the book store and spending hours carefully choosing which book I would take on the plane.  I was also terrified of flying, so having a book to read transported me to another world where I didn’t have to think about being thousands of feet in the air.

From the time I learned to read, I read voraciously.  I remember when the final Harry Potter book came out and it was delivered to my house; I read the whole thing in a few hours because I wanted to finish it before I heard anything about it on social media or the news,  and then immediately started reading it a second time to catch all the things I may have missed on the first read.  That book was probably the highlight of my year that year; is that sad?  Oh well.  Potter Rules!!!!!!

You get the point, I love to read.  As I have written in other posts, I am incredibly lucky that I  have pretty good central vision and can still read.  Most people know about 20/20 vision and how that is the ideal, and most people I know who wear glasses get their vision corrected up to 20/20.  For me, without my glasses, my vision is about 20/300, but with my glasses, my vision can be corrected up to 20/35; this is really fortunate for someone with RP, but even for me, reading has its challenges.

If I spend more than 20 minutes (sometimes much less) looking at a back-lit computer or tablet screen, my eyes start to sting and burn, and I will often have to close my eyes to rest them for an hour or so.  Of course, there is a wonderful remedy to this, which is inverting the colors on the screen; right now I am typing on a black screen with sort of peachy beige text.  However, even with the screen colors inverted, if I am at the computer for too long, they will hurt simply from over-use; I have to make sure that I take my eyes away from the computer every 15 minutes, and I am not great about doing that.

As far as reading books, I have a lot of trouble focusing, due to both the RP and cataracts, so I can’t read real books, newspapers or magazines anymore without ending up in serious pain.  Trying to focus on small print is way too much work for my eyes, and reading glasses just make it worse.  So, thankfully, I have my  kindle, which has no back light and the capability to increase font size, but even on the Kindle, my eyes will start to ache after about an hour of reading.  The thing is, I can get so engrossed in a book that I don’t pay attention to the time or the fact that my eyes are hurting, and I pay for it the next day.  I have had days when I can’t look at anything, even in dim lighting, for more than a minute without experiencing pain.  Unfortunately, RP hasn’t taught me how to make better choices when it comes to over indulgence.

Another big culprit in a bad eye day, is spending too much time outside.  Whenever I go outside, I wear a big hat and sunglasses, but even with my eyes protected from the sun, if I spend a day outside, I inevitably spend a day in bed with my eyes closed.  I used to love to take long hikes, but I can’t do that anymore because of the sun; and let’s be honest, I probably shouldn’t have been hiking on my own anyway, given that I can’t see what’s going on around me.  So, basically, any all day outdoor events are something I dread, and don’t even get me started about the beach.

I can’t deny that when it comes to my vision and my life choices, I can be reckless; it is in my nature.  I like to do what I want and I have often paid the price for my stubborn ways, but as my vision gets worse and my RP symptoms increase, I am having to quell my rebellious side and pay attention to what my body is telling me.

Over the past year, I have truly dedicated myself to a writing life, which has been amazing, but it has also resulted in more bad eye days.  I am spending more and more time, not only writing blog posts, but also working on my poetry, writing stories and reading the work of countless amazing poets and writers.  I feel inspired and uplifted and incredibly motivated, but I realize that a bit of scheduling is in order.  Scheduling is such a pesky word (pesky is a fucking awesome word), but for the sake of my eyes, I have to find some reading/writing  and life balance. I have to give my eyes some TLC and give myself days away from the computer.  I need to take breaks and pay attention to time.  I need to stop fighting my RP, but it is going to be hard.

 

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