Nightlight

I found myself singing a Garth Brooks song in the shower the other day - unusual not only because I don't often sing in the shower (someone might hear me!) and because I can't remember the last time I listened to a Garth Brooks song on purpose. But as the water beat down on me, I found myself singing the chorus, "And I'm glad I didn't know the way it all would end, the way it all would go…"

The song has nothing to do with retinas. It has nothing to do with not being able to hear. It has nothing to do with my life at all, really - it's Garth and a failed relationship and some steel guitar and a whole lotta feeling, and yet I found myself singing anyway and feeling it and finding this bit of understanding.

I recently got the results of my genetic testing back. Those results confirmed that yes, I have Usher Syndrome. The results confirmed that there are two changes (mutations) to the USH2A gene. The results confirmed that the hearing loss that I've had all of my life is related to these new vision symptoms I'm only just discovering.

Finding out has meant both everything and nothing at all - which feels like it makes no sense, but perhaps might to other people in my shoes. There are no cures, there are no treatments and there are currently no clinical trials for people with my particular type of Usher Syndrome. Having this answer isn't really an answer right now - it's just a little piece of information that leads absolutely nowhere and so I have found myself so completely relieved that I'm only just now finding out.

I think of what would have happened if I had known this for all of these years - had genetic tests been done years ago to determine the cause of my hearing loss we might have discovered these mutations to my USH2A gene long ago - what would life have been like if I spent my whole life waiting for my vision to fail, looking for clues, looking for symptoms and signs? Would I have been scared? Would I have gone down the same path? Would I have been more restrained? Would I have made different choices? Would I constantly monitor every nuance and every little thing - the way I find myself doing now?

My god, I'm so glad that I had all of those years of not knowing.

Honestly? The hearing loss was bad enough. The hearing loss has always been a challenge and it's been a struggle - had I found out earlier, younger, that my vision could go too? I can't imagine.

So here I am. I am 38. I'm just a month and a few days shy of turning 39 and in this last year, instead of just fretting about hitting the big 4-0, I'm also dealing with a life changing diagnosis that I can't do anything about.

And I thought fine lines and my slowing metabolism were rough.

I can tell myself, "You know it could be worse," all day every day, until I'm blue in the face. I DO know it could be worse. And there are days when I truly believe that the worst case scenario WILL NOT apply to me because my vision is still pretty good so maybe, just maybe, I'll hang on to more of it.

Who knows? Some people will say that blindness is a certainty, some say that's not necessarily the case and I still really don't know enough about it, really, to know one way or other.

And that's because it's so new to me.

And because I hate reading about it.

Had I spent my whole life knowing, I don't know… would that be the case? I don't think I could dwell in the same level of quasi-ignorance in which I currently reside. Surely time would fill in some of these informational holes - holes that I don't mind not having filled, really.

I wish i still didn't know, you know? That I could still just leave it at, well, my hearing is awful and just keep on not knowing about the other stuff, because not knowing was nice. Not knowing let me be fooled into thinking I was finally getting ahead - that everything was fine.

I know that everything will be okay. Somehow. Some way. I do know that.

But I'm glad that I got to spend decades of my life not knowing what was coming. I'm forever grateful to not have had years of waiting for the other shoe to drop. Years of living the best life I could, playing the cards I'd been dealt. And now the game has changed a bit, I have new cards. I have to figure out how to play this hand.

(And that's where the card analogy ends because I hate playing cards and I don't even know if what I just said made any sense)

I have never liked the unexpected. I don't like surprise parties, I am not spontaneous, I hate when my plans get changed at the last minute. I am not…entirely rigid, but yeah, I like to know what's what and when and why and no, I really don't much like flying by the seat of my pants.

It means that I'm virtually unbearable right now.

I know that it's normal.

Last week, I read an e-book by a woman who has RP. In her book, Believing is Seeing, Laura Lawson describes the grief of this diagnosis - and it was almost a relief to see some of the feelings that have been bouncing around in my head expressed by someone else - a relief to have a shred of normalcy, that oh, feeling this way is probably a totally legit way to feel in this situation. (Note: Laura's blog is here. I'm so glad my friend told me about her and pointed me towards her blog - because a) what are the odds that a friend of mine knows two people with RP? and b) I'm a little less scared when I see all that she's doing - reassuring to know that I might be able to hang on to my camera, you know?)

We don't need permission to feel the way that we feel but when you're dealing with something unfamiliar, it's kind of a relief somehow to know that the set of emotions somehow does match the circumstances. The emotionally evolved side of me knows that whatever set of emotions I should happen to feel would also match my circumstances - because our feelings are our feelings and blah blah blah - you feel what you feel. But my gosh, sometimes I am sad and angry almost in the same breath and what do you even do with that?

In short: You go on.

I go on.

On the toughest days, I know that I must radiate some kind of force field of emotion that repels those I encounter. I don't know that there's a "right thing" to say to me, but I have found a thousand wrong things.

I am not easy these days. And I want to be. I want to be the strong person some people think that I am, but the reality is that the same person who hate surprise parties and hates not having a plan really hates this knowledge that my retinas are dying and are going to continue to die and what do you even do with that knowledge if you think about it too hard?

I admit, I have always been a bit of a pain.

"You're the worst kind; you're high maintenance but you think you're low maintenance."

"I don't see that."

"You don't see that? Waiter, I'll begin with a house salad, but I don't want the regular dressing. I'll have the balsamic vinegar and oil, but on the side. And then the salmon with the mustard sauce, but I want the mustard sauce on the side. 'On the side' is a very big thing for you."

"Well, I just want it the way I want it."

"I know: high maintenance."

But these days? I'm probably a lottle bit worse. And I'm sorry. But. Forgive me? I'm hoping that time will give me a bit more of the strength I need.

*Lottle - More than a little, less than a lot