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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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RP

Eating Barbequed Iguana

I fell a few weeks ago, on the sidewalk, while gawking at another new group of hideous town houses that are being built in our neighborhood.  When I fell, I cried, not because it hurt, but because I felt humiliated, broken, slapped in the face yet again by RP.  My depression and self loathing voices took center stage and told me I was useless and really shouldn’t even be outside if I can’t manage to walk a block without falling down and scraping my knees.  I wanted to hide, from the RP and the day and the world.  I wanted to hide from myself, pretend I was graceful, dream I could float.  My sadness turned to anger and I stumbled home, terrified that every step may be the one to send me back into the unwelcome embrace of the pavement. Until very recently, this had been my usual response to falling.
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Hiking Blind

A friend recently asked me how I manage hiking with limited vision.  I have actually been thinking about writing a post about this since I started hiking again. I thought it might be something other people wondered about as well.  How the hell can a blind woman go hiking?  You have to remember, I am blind but I can see.  It is probably less confusing if I refer to myself as VI (visually impaired), but I am legally blind.
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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.
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That Kind of Day

I had leftover sweet potato fries for lunch.  Yeah, it’s that kind of day.  I can’t focus.  The city is bumpy and screeching outside my windows and I can smell summer approaching. I know that spring has only recently officially begun, but there is no Spring here, not really.  I fell on the sidewalk, while walking the dogs and gawking at the monstrous buildings that are popping up like weeds in Hollywood. I got a bit scraped up and bruised. Why does falling hurt more when you’re almost 49? And almost blind? I cry when I fall, not because it hurts, but because it reminds me.  I am resenting Zelda (my white cane, for those who haven’t been introduced) and RP and middle age. I should be working on poetry.  I am writing this blog post instead. Continue reading “That Kind of Day”

A Guest in Someone Else’s Home

When I discovered Steve’s blog, MSich Chronicles, I was an instant fan, of Steve and of his writing.  So, you can imagine how thrilled I was when Steve asked if I would write a guest post for his blog.  This is my first foray into guest posting and it is a real honor to be asked to be a guest writer in another’s virtual writing home. Thank you Steve for putting your trust in me.

I Don’t Do Sick Well

I am sick.  Nothing serious, just a cold that won’t seem to go away, but I don’t do sick well.  No one likes being sick, I get that, but when I get a cold, I act like the world is crumbling.  I wasn’t always like this.  I used to get sick and ride it out and never talk about it.  Now, I am not only talking about it, but writing a blog post about it.  I must sound pretty crazy, or like a total princess.  Either way, it’s not cool.  I need to figure out where my tenacity and grit escaped to.  Thanks to my Dad, I think I have an idea.

I was visiting my Dad 4  days ago, the day the beast cold started attacking my throat, and I mentioned that I felt like I have become super sensitive to just a simple cold, making a huge deal about a sniffle or two.  He told me that he thinks I may feel like any physical ailment is just one more fucking thing on top of the big thing, the blind thing.  And, yes, he did say the F word; I come from a family who use expletives freely.
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A Hundred Secret Senses

Toward the end of last year, I started writing a bit more about the ways in which RP affects my physical life and tried to answer some questions.  One question that I haven’t answered, posed by two of my fellow bloggers and friends, Mandi and Tom, is about how my other senses are affected as my sense of sight is swallowed up by RP.

There are differing opinions about this.  Some people say that the idea of your other senses getting stronger is a myth, others think it is pure fantasy – who doesn’t want to be DareDevil after all? I think it is an inevitability, but also something that can be cultivated over time.
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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.

 

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