The Equinox Was Owned - Usher Syndrome Awareness Day 2017

I meant to write earlier. 2017 has been a supreme blur and I meant to tell you all about the things - how I trained for my first half marathon, how I went to Iowa for the second time to see Dr. Stone, how I ran my first half marathon and how I cried when I crossed the finish line. I was going to write about seeing Hamilton in Chicago and how my daughter clasped my hand for the whole first act, just in complete awe that we were in that room where it happens, how tears rolled down my cheek during "Yorktown." How I stopped taking the stairs after I've been out for a walk during my lunch break because my eyes could never adjust to the dark of the stairwell and I kept tripping over stairs. How we went to California this summer and in the dark strange room, smashed my hip into a dresser and it ached for days. How I went to my first Usher Syndrome Conference in Chicago and I actually got the nerve to leave my hotel room and sit down in the audience and absorb it all, even though I was scared.

Some days, I'm still pretty scared.

Saturday, September 16 is Usher Syndrome Awareness Day.

I get scared all the time when I think of what I don't know or trying to anticipate what a future might look like.

But as Dr. Stone said this July in Chicago: We're close enough to walk.

He spoke a lot about hope, and I felt relieved the whole while to know that I have the advantage of having Dr. Stone and the amazing crew at Iowa on my team.

“I mean you can see it. You can walk three miles an hour. So if, if actually they would just  not let the car go anymore, okay, if the FDA showed up and would not let the car go anymore, you could simply get out of the car and walk downtown, and that is actually where we are on the development of cures for Usher Syndrome.”

Without taking this to a totally political place, I'll say that having a disease like Usher Syndrome - a disease with no treatments or cures - is terrifying in the age of this new president. I've been disappointed by a lot of what I've seen so far, and I've feared that the administration would cut essential funding to essential research and slow the progress that's got us so close we could maybe get out of the car... and walk.

We probably can't rely on politicians to do what's right for us (oh, that was a news flash, right?). Fortunately for those of us with Usher Syndrome, there are donors who are generous with their funds who are willing to support progress.. And I am so grateful.

For the past 26 days (y'all... that's a LONG time for an awareness campaign!), so many of us have been doing what we can to raise awareness. And when the Own The Equinox campaign is done, the Usher Challenge goes on. I'm throwing links to both of those in the bottom of this post - both benefit amazing organizations doing different work - for the same group of people just like me.

In the past year, my hearing seems to be essentially the same, as does my vision. I had a horrible experience with a stupid eye doctor this winter when I went to get contact lenses. I had to explain to him what retinitis pigmentosa is. And while I may not have actually uttered the words, "Are you SURE you are a real doctor?!" out loud, I sure was thinking them. And then bless his poor heart when he tried to upsell me with photos of my retinas! Nah, I'm good, man. I've seen 'em. They're broken. For now, anyway. (That janky eye exam is how I will happen to spend a portion of Usher Syndrome Awareness Day actually GETTING AN EYE EXAM - it's not just commitment to the cause, but a need for new contact lenses because I'm pretty sure the dude wrote me the wrong rx months ago, and well, I've been too busy to fix it but now I'm out of contacts and HEY RUN ON SENTENCE HOW ARE YOU?).

Admittedly, not experiencing a significant decline helps my frame of mind. It really is progressing slowly, at least for now, and that's a huge relief.

Research needs to keep moving faster than the loss of my vision. That's pretty much all there is to it. Research just needs to keep being faster.

And that's why these campaigns - as annoying as they may seem to some - are sooooo essential. Because they give Usher Syndrome, and those of us with this disease, a bit of a moment, if you will. Our chance to tell you about it, to ask you to read our stories, and encourage you to be aware of what this disease is. And sure, if you felt so inclined, maybe you could support one of those organizations below.

I'm training for my second half marathon - it takes place in about four weeks. I can do hard things, I can still reach goals. I'm still tough. In two months, I'm taking my mom to Chicago where once again, I plan to sit on the edge of my seat during "Yorktown" and realize yes, how lucky we are to be alive right now. Next year, I won't be in Iowa. Dr. Stone says I can come every two years... and that to me feels like a bit of a reason to breathe a moment and let go of some of the fear.

(I'm trying)

I barely remember my life before my Usher diagnosis and that seems a bit of a shame really. But you can't go back - only forward... so here I am, one foot in front of the other. Probably in running shoes.

That time I lost a year

I think I spent most of last year in a fog. It goes beyond the fact that the older I get the faster time moves (time being a cruel beast who has me hurtling towards 40 at breakneck pace). Most of 2015, and the early part of 2016, was spent in a the kind of thick pea soup fog that makes you feel like you're walking through pudding.

I cried more days than I didn't.

I was angry more days than I wasn't.

I felt scared more days than not.

In short, it was easily the absolute worst year of my life.

That's a hard bit of information, I'm sure, to the people who love me, who strive to make my life special and wonderful and meaningful. People have a tendency to take it personally - the unhappiness rooted in the startling news of a scary diagnosis and the havoc it wreaks on your state of well being.

Because sometimes there are no right things to say. You can't smooth a jagged edge, you can't unravel the tangles of the thoughts of someone who is in the midst of muddling their way through figuring out what a new normal could look like.

The shock of receiving the diagnosis of Usher syndrome was compounded by the way it was delivered: without compassion, without hope, and, worst of all - without eye contact. 

No longer able to envision my future, with every doctor's appointment, I was sure that would be the day they'd pull the plug on my independence: no more driving, progression too great, vision too far gone. The anxiety some people feel about the dentist, I would feel about the eye doctor, much to his dismay. 

"This is better than the dentist," he would say.

"My dentist is actually pretty awesome," I replied.

I couldn't envision my life a year down the road, and certainly not five to ten years in the distance. What could be left? Surely nothing.

[This is where I'll leave out all the grim thoughts one has when they tell you that you will lose both your vision and your hearing because it is a HUGE bummer and no one wants to read that.]

And so.

I was told by several people to visit the University of Iowa, to see Dr. Stone there. I heard a lot of really amazing things about Dr. Stone, but the most important thing was that he was positive, that he might give me some hope. Well, hope is certainly worth a road trip.

In April we went, stopping along the way to visit a dear friend who filled us with love and carnitas before the first of two appointments.

-

From the beginning, my experience at University of Iowa was different and in the best possible way. I began with a field vision test - oh, how I hate those - and the stress of the test was overwhelming. I didn't want to fail, I didn't want them to say, "You can't see, you can't drive, you are so much worse than you were last year."

The technician paused the test. Told me to just do my best. Assured me I couldn't "fail." She got up and walked around the machine and stood next to me, gently patted my arm, was encouraging.

Oh. I am a person. I could feel it in the way she treated me.

I received the results from another doctor who not only assured me I still had a very wide field of vision and he was not at all concerned about me driving, he provided me practical tips for how to best function in lower light situations, where my test indicated I had the greatest difficulty. How to navigate dim spaces, crowded spaces.

Oh. This is empowering. This allows me to feel safer in places where I can't see as well. I am a person. I could feel it by the way he treated me.

-

We celebrated that night with dinner from a local restaurant, a plate piled high with onion rings and I can't even remember what else. One hurdle down, but I still had the next day ahead of me.

-

I met Dr. Stone the next morning. I expected a day full of tests and awfulness, but my appointment was quite short. He assured me the loss would be slow. He assured me that there is an army of people researching cures and treatments. He assured me that it was his job to know what the latest developments are so that he could let me know what I needed to know.

"Your job," he told me, "is to live your life. Put your worries in a box and leave them in this office, and we'll come back and look at them next year."

I think I floated out of the office.

It wasn't at all what I expected. I was so prepared for the worst - for a year, I was prepared for the worst, and what I got instead was: Keep on keeping on because this ain't over yet. This is a monkey wrench, but you're still exactly who you were. You will still be who you are tomorrow.

-

That was over two months ago. If I think about it too hard, amidst the relief is a bit of anger over the year I lost while I drowned in fear. I feared so much would be ripped from me - my ability to drive, to keep a job, my independence. And... well, someday it still might be. But not today. And not tomorrow. Probably not next week or next month. I am finally feeling like: I have time.

I'm not entirely sure what to do with myself now. I mean, I've got this life in front of me and I better go live it. For so long, I couldn't envision what much future held - and to be honest, I still can't, but at least there's a bit of hope hanging out there.

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On the back of my right arm, just below my tricep is a scar. Often, I touch my arm, feeling the smooth small knot of scar tissue. I find myself doing it often, touching the scar.

It's odd, I know, and it's funny to imagine that just over two months ago, it was a bleeding, oozing puncture wound in my arm. I didn't feel it when the nurse punctured my arm to take the sample for testing. Days later, though, it ached and, well, it wasn't pretty.

That sample is probably in a research lab somewhere. Smarter people than me are working to make retinal cells out of stem cells. Like some day, that chunk that was taken out of my arm can be used to make cells that might help me or people like me. Not that exact chunk, I'm sure. That chunk may or may not already be hanging out in a biohazard waste bin.

When I think of it, though, I can allow myself to feel hope. That there's something bigger going on. That small sample could some day be something very big and that... that's something to me. That's huge.

It's something to feel hopeful about.

Finally.