You don't have to see it the way I do.

in

In just over two weeks, I am going to be 40 years old. This is a fact that I'm having a difficult time warming up to and it's also causing me some distress because I never claimed I was going to age gracefully and I most assuredly am NOT.

I can run a lot further than I could at 30. I'm not changing diapers or chasing a toddler like I was at 30. But 40 is a different animal for a lot of reasons. Mostly because at 30, I thought I was just fine... and now I have to remind myself that I will be.

Last week, my employer handed me an insurance form and asked me to have my doctor at Iowa fill it out and return it. Again, it seems the insurance company saw my appointments and got a little freaked out: "Uh oh, this woman is gonna cost us money." They are now quoting my employer an astronomical hike in the cost of our company insurance plan. Affordable insurance for my employers to provide for us hinges a lot on this form.

I hate that feeling.

And while the folks at Iowa promptly filled out and returned the form, it is a reminder to me that no, I will not be costing the insurance company a bunch of money because there are currently no treatments for Usher.

It's an ugly feeling - this sadness about what is beyond my control blended with this irrational sense of responsibility for costing my company so much money (I didn't even hit my deductible this year. That's how much money I DIDN'T cost them).

I have been in a funk ever since.

-

Foundation Fighting Blindness has been running a campaigh - #HowISeeIt is supposed to drive awareness and raise funds. It's been pretty amazing to see celebs active on social media promoting FFB and driving awareness. It's no ice bucket challenge, but I'm not a fan of being cold, so I'm alright with that.

It's been a bit disheartening to see the backlash against the Foundation and against the campaign.

I read the comments on Facebook (oh, silly girl, never read the comments) and I'm... frustrated. I'm angry.

There are some people who take issue with the #HowISeeItChallenge because they see it as fear mongering, they take issue because they feel it implies there's something wrong with being blind - as though that makes them "less than." The criticism of the Foundation has been harsh and it's been confusing to me because since my diagnosis, I have found the FFB to be tremendously supportive, helpful and committed to their cause.

Look: It says it in their name - Foundation FIGHTING Blindness.

It is their mission to end vision loss caused by retinal disease. To expect their mission focus to be anything less than trying to eradicate disease is ridiculous - as it's WHAT THEY DO.

No, it doesn't mean you're less than or inferior if you're blind.

It's just that NOT ALL OF US WANT TO LOSE OUR SIGHT.

(Let me say that again)

Some of us are TERRIFIED of losing our vision. Some of us DON'T WANT to lose any more sight. And some of us are going to fight like hell to support the people who are trying to find treatments and cures.

Why this came as a shock to me, I'm not sure. The same sort of culture is prevalent in the hearing impaired communities as well. But I've never had deaf pride. I've always hated not being able to hear well.

And you better believe that I will fight this vision loss as strongly as I can.

-

Fall is here and it's getting darker earlier and the sun is taking longer to rise in the morning. I love fall - the end of sweating every time I step outside. The darkness is already getting to me, though.

I may never regain what I've already lost but I don't want to lose anymore. Every time I start thinking I'm doing okay, I hit a patch of not-okay-ness. At 30, I had no idea any of this was coming and I'm staring down the barrel of 40, all too aware of how much has changed for me.

It's a tough pill to swallow, and I'm just not ready to accept it yet.

I'm not going to allow others to make me feel guilty for not accepting it. And that's how I see it.

That time I lost a year

in ,

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.

-

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.

 

 

 

 

Every once in awhile I feel like I could potentially be a bit more positive and uplifting than I really feel

in

Today is the last Tuesday of 2015. Tomorrow will be the last Wednesday. This week contains the last days during 2015 that I will go to work and when I walk out the door at the end of my work week, that will be the last time I'll walk out the doors until next year. Always seemed fun to say, back when we were in school: "See you next year!" as if next year was a substantial amount of time away instead of mere days.

Tonight, I was looking at a link someone shared of beautiful underrated places to travel. I kept thinking to myself, "I want to go there...." I think to myself, maybe in this year ahead, maybe this could be the year I go somewhere big.

(And then I mentally catalog all of the other things that I will need to spend money on before I spend money traveling to far off places and then I wonder what the odds are of winning a lottery one doesn't play and then I think, "Well maybe I really don't need to go to Andalusia anyway... Maybe I should just be content with what I have.")

Some days, being content is actually pretty easy.

I wouldn't have thought so, earlier this year, that I would ever have days where I didn't cry, that I wasn't constantly gripped with some sense of feeling sorry for myself - but here we are at the end of the year, and I'm pretty sure I haven't cried today. Probably not yesterday either. I make no promises about tomorrow - but who can promise tomorrow, anyway?

I think a lot about luck and the lack of.

About time and the passing of.

About fear and the abundance of.

Some days, if I'm not careful, the mind weighs down and I can feel so scared about what I don't know. I don't like those days.

Yesterday, I saw an article about a study where scientists created retinal cells that worked in rhesus monkeys and I hesitate to feel optimistic because a) I'm not a monkey and b) even if it does work, human trials are probably well down the road.

I think about how there's a presidential election coming up in the next year and people are so focused on their righteousness and sound bites that there are big big issues that aren't getting any air time at all - like medical research. I think of how many people who get so bogged down on what the news tells them matters most, that they don't think of the implication of what happens if someone is elected who doesn't see the hope in science. Progress could be stalled and that stalls hope for me. People like me. And people who are dealing with far worse things than me. People who need science and hope and progress.

And when I think of that, well, I get a bit angry.

Just so you know, anger feels better than fear.

(But both feel a bit powerless, if you really wanna know.)

-

I really didn't expect any of this when I rang in the new year nearly twelve months ago. I feel cheated out of the year I envisioned for myself - a year that was still full of good things but marred by this dark cloud.

I overheard something a few weeks ago about choosing to feel joy, and the truth is, I don't choose joy as often as I should these days and that's probably something I'm gonna have to work on next year. Perhaps scientists can manufacture some of THAT with stem cells.

I really am trying.

It doesn't always look like it.

It doesn't always feel like it.

But I am. I am trying every day to keep on putting one foot in front of the other and to not let myself get bogged down with a diagnosis that changes my life, yes, but does not end it.

And so it goes.

And this is why research is important.

in ,

A family friend recently shared this with me, and I was pretty much blown away by the type of research that's going on.

Please support the folks who are doing the work.

I've set up a fundraising page over on FFB - they're an amazing organization run by people who have blown me away with kindness - with the heart to sit on the phone with me and talk to me about the questions I have and the fear I've been feeling.

To support research, you can donate here.

Eye thank you. 

And suddenly my vocabulary is different.

in

It's been five weeks since I met with the team of eye peeps at Kellogg and about four months since my eye doctor first told me she spotted pigment changes in my retinas. A lot has changed in those four months - namely, the amount of time I spend thinking about (and fretting about) my eyes.

If I'm being perfectly honest, I don't think I gave my retinas a single fleeting thought before - and the only time it might have even crossed my mind was in terms of the Retina Display on my iPhone 6 (thanks, Apple). 

Before January, I don't even think I knew what my retinas did, let alone devoted hours of my waking time being upset that mine don't work.

My brainspace has changed a lot. So has my vocabulary. Now my vocabulary is full of...

 

Retinas.

Your retinas are light sensitive tissue lining the back of your eye. Light rays are focused onto the retina through our cornea, pupil and lens. The retina converts the light rays into impulses that travel through the optic nerve to our brain, where they're interpreted as the images we see. Fun fact: if you mix up the letters of retina you'll spell retain - I only know that because I've typed retain four times in the last paragraph.

Visual Acuity.

This is the eye test we are all probably familiar with - where you need to identify letters or symbols from a fixed distance. I feel pretty lucky here - this is the one part of the eye exam I feel like I can still kick butt in.

Visual Field.

This test stresses me out. It doesn't hurt at all, but it reminds me much of the hearing tests that I should be having far more frequently. It's a test to see how much you're seeing in the periphery... so it's a series of flashes of light in different points of your vision, and you click a button when you see a flash. 

Electroretinography (ERG).

If you read the description of an ERG test, you might get a bit weirded out because, hello? An electrode on your cornea? And then, you think to yourself, "Well, I wear contacts, so... it can't be THAT different." 

Let me repeat that: electrodes on your corneas.

Nope, it doesn't at all hurt, but it's a bizarre feeling. Also? The gel-goop they put on the electrode dripped out of my eye, ran down my face, and smeared my makeup so I likely looked like the worst nightmare vision of a clown - WITH ELECTRODES.

Usher.

Not just the guy who sings that "Yeah!" song that my family likes to dance to at weddings. Who knew. 

Autosomal Recessive Disorder.

We may all remember from basic biology class that we receive genes from both of our parents. This whole thing has made me wish that I had paid closer attention in biology class.

In the case of an autosomal recessive disorder, it means that I received a copy of the abnormal gene from each of my parents. Neither of them have RP, but both were Carriers of the abnormal gene. There was a 25% chance I'd receive two normal genes and a 50% chance I would receive one normal and one abnormal. Annnnnnd, lucky me, the 25% chance of getting the two abnormal ones (gosh, no wonder I hate Vegas - in the genetic crapshoot, my luck? Not so hot).

You can't control what genes you pass on. You want your kids only to get the super great things about yourself but sometimes... that doesn't happen. For example, I had hoped my daughters would get my naturally curly hair - no go. However, one of them has my nose and one of them has my not-so-endearing tendency to get brutally grouchy when she's hungry. Luck of the draw. Not always good luck.

Retinitis Pigmentosa.

I had never ever heard of this before this year. And that's probably true for a lot of people. Partly because it's not that common and partly because no one has done an ice bucket challenge for it that's gone viral and raised a ton of cash.

I've linked to the definition of RP before, so I won't again - but what I will say is that if I have to have this stupid thing, I'm going to work to make sure that it's part of the vocabulary for more people. Why? Because awareness brings change. Also? Because I'm going to complain about my retinas, and these tests, and wearing floppy hats, and having trouble seeing at night, and my fears, and...... [What? Like you expected anything different?]

Cassiopeia. Orion. Ursa Major.

in

I listen to a lot of podcasts when I drive lately. My patience for the radio is low - the same songs over and over again. My drive home coincides with the local top 40 station making a dance mix of everything for an hour and I can't stand it, so… podcasts. 

It started with the podcast of some friends of mine. Then Serial. Then other things. Some are great, some are…less great. It takes up time, makes the drive easier. 

I recently started listening to NPR's "All Songs Considered." In college, I loved how I was always able to find new music - I loved being ahead of the curve, to have a library full of more than just what got radio play. "All Songs Considered" is giving that back to me - and it's ultimately going to end up costing me a fortune in iTunes downloads.

I was driving home from my daughter's gym meet. It was dusk when I pulled out of the parking garage. I'd have been on the road sooner but the garage attendant insisted on collecting everyone's parking ticket as they departed. I had hoped to be on the road sooner.

Twisty roads, "All Songs Considered," and a new song - Gacha and "Waterfalls."

My eyes scanned the sky - and nothing.

Darkness. Darkness. Darkness.

As I drove, my eyes would periodically shift towards the sky - looking for something, anything. And yet… nothing.

Agitated. Scared. On the edge of tears.

Well, maybe it was just cloudy.

You see, when I went in for my doctor's appointment in Ann Arbor, one of the questions they asked me was, "Can you see the stars?"

"When it's not cloudy," I responded. But as I drove Friday night I realized: I can't remember the last time I noticed the stars. Was it because I've just not noticed or because they weren't there for me anymore?

By the time I pulled into my garage, I was eager to run into the house, throw down my purse and race to my back deck to look out. Well. I couldn't see the moon either. I know I've been able to see the moon, lately.

How relieved was I - when I finally got the nerve to ask - to find that there were no stars to see, it was too cloudy?

-

I've tried not to read much about retinitis pigmentosa - mostly because it all terrifies me. One of the things I've read several times is that so many people can no longer see the stars. Can you imagine the night sky above you - a dark, blank canvas?

As I stared into the sky Friday evening, I was wracked with fear - Is this how it's going to be? and Did I lose the stars and not even notice?

-

We went out to dinner Saturday night. Chris and I tried a new-ish taqueria in Grand Rapids. Margaritas, chips loaded with queso, pork belly tacos. We chased dinner with handmade ice cream from a local creamery (vegan ice cream? What is it made of?). 

When darkness fell, i looked up. I saw nothing. 

"Are there stars?" I asked him. 

He scanned the sky, "Not much. Just one." He pointed.

I tipped my face upward to where he gestured.

The stars aren't gone for me. Not yet. I saw it. And I've been looking ever since. 

I can still see them and I will keep noticing them and I will keep counting my blessings that they're still there shining on me. 

I hope to never stop seeing them. 

There are days when I have a lot of hope and there are days when I want to dive face first into a box of Cheez Its.

in

A friend told me recently that since I wouldn't have known anything was wrong if I had not offhandedly mentioned it during a routine eye exam that perhaps I should just pretend that this thing doesn't exist - that I don't know about it. And she is so very very right except… that's not how my brain works. I am a glass half empty, assumes the worst, the sky is falling kind of person.

I can't pretend it doesn't exist because I'm too busy being scared of it.

And the thing is, I've been fortunate enough to have been given hope by people who care enough to inform me that listening to my brain may not be the best idea. There's a lot of research going on right now - some of it nearly ready for clinical trials - and who knows, maybe in ten years there will be a treatment that stops all of this before it gets too bad. Or brings back anything I lose.

I have been angry at the world. I have been scared. I have been hopeful. I have been loved. I have been heartbroken. I have a whole bunch of feelings - sometimes all at once - about this diagnosis.

One of the things I hadn't anticipated was how hard it would be to not truly know my diagnosis. I have currently been diagnosed with retinitis pigmentosa - but my diagnosis is quite likely Usher Syndrome which would mean that the hearing loss that I've had all my life is tied to this vision thing I've only recently started experiencing.

Weird, right?

The onset of the vision stuff is fairly late for me, I'm told - which should bode well for me overall. This makes me hopeful in the moments when I dare feel hope. Sometimes I dare.

The statistics say that about 50,000 people in the United States have Usher Syndrome, which means that this is a pretty rare thing, because of course. 

And all of this is even more of a shock to the system because the things I love most to do - photography, reading - rely so heavily on my stupid eyes.

By the way, that's the name of the folder in my Gmail where I store all my messages with the genetic counselor, and other people relating to this eye hooey: "My Stupid Eyes." 

I've never really been one to let life kick me in the teeth though. I feel like I've been run over by this diagnosis, and I've probably cried a gallon or two of tears in the past several months, but the moments when I can let myself envision making a difference somehow, those are the moments I feel strong, the moments when I feel like maybe I still have a little bit of control here. 

Because I am still at the point where this is often sitting right in the front of my brain. I am told that I'll get to the point where it's not all that I think of, but right now - I'm just not there yet.