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Monday, August 1, 2011

The Only Certainty

by Mark Dunning

Forever is composed of nows. ~Emily Dickinson

I have a friend with Usher syndrome. She worries about it. I know she does. I can see it in the way her eyes brighten when we talk about potential treatments. I can feel it in her enthusiasm for knowledge about the disease. Yet she does not live like someone who worries about Usher syndrome. She stubbornly refuses to let the future decide her present. She lives for today.

She has long been my hope for my daughter’s future. When she was young she drove thousands of miles with a vanload of friends to exotic locales, scraping by, sleeping on floors and in hostels. She lived crazy, carefree. She got her nose pierced. When she was older she fell in love, got married, had children. She travelled for her career then, dressed stylishly, pulled a rollerboard and used a smartphone. But she still has that same young smile, she still has her nose pierced. She has always been alive. That’s the best description of her. Alive. I want my daughter to be that way. I want Bella to be alive.
I am training to run a half marathon. I ran ten miles this past weekend and when I finished, I felt more like a corpse than an athlete. I have never been a runner before. Just read this if you don’t believe me. The irony is that I have taken up running to escape death, rather than court it.

When Bella was diagnosed with Usher syndrome, I found myself laying on the floor in the dark, my mind racing uncontrollably into the future. In a blur she was giving up horse riding, struggling in school. Then she was out of work, living in a sterile apartment. Then she was grey haired with a cane sitting at a family outing oblivious as her brother’s kids scurried around her. Then, just like that, she was dead.

Wait a minute. She was dead? That’s it? Four sentences then dead? The diagnosis had turned out to be far more dire than I ever imagined. It was even worse when I put myself in the story. If Bella was grey haired at her brother’s party, where was I? Gulp.

I put down the Ring Dings and took up running. I did it so I could be alive to help Bella. I wanted to be there to help her find a way to keep riding horses. I wanted to kick her out of the house to go to parties and socialize and make friends. I wanted to embarrass her when she felt sorry for herself and make her go to college even if she didn’t want to. And I wanted to be at that party to see grey haired Bella smiling as her children played with her nieces and nephews.

More than anything I swore to do my best to live in the present. That is where I am content. In the present Bella can see and in the present I am alive.

People with Usher syndrome are not miserable. They are not unhappy. Oh sure, they might get frustrated with their condition from time to time, but I don’t think they curse their fortune any more than, say, the average middle-manager cubicle jockey. The distinction lies in the future. The cubicle jockey envisions a brighter future in the corner office while people with Usher tend to fear the future. They dread a darker tomorrow.

The truth is, though, that we all face a darker tomorrow. If we look too far in to the future, we’ll find that we’re all dead. Sorry, but it’s true. A thousand years from now we’ll all be dirt.  But none of us look THAT far in to the future. To think a thousand years ahead would cripple all of us. We couldn’t function. We are much better served living today, trying to improve tomorrow, and not thinking about the distant future.

I often wonder why we families with Usher struggle so hard to take that same approach to the disease.  It’s not like we are unfamiliar with the process.  We all ignore the future to some degree.  Today, we are happy and functioning.  Do we really need to concern ourselves with more than that?  As Albert Einstein once said “Never think of the future. It comes soon enough.”

My friend inspired me to write this post. She always inspires me. She was recently diagnosed with cancer. I don’t write this as a eulogy for her, but I am very worried about her. I know it is constantly on her mind. I know she sees it in the mirror in the morning, in the eyes of her husband, in the faces of her children.

This is a painful irony. She has spent her life refusing to believe she would lose her vision. Now that might be true, but for the all the wrong reasons. I want Usher syndrome to be prominent in her life once more. I wish more than anything that it could be the reason she enjoys today. She has always been alive, thriving in the present. I wish she could stay that way forever. She is my hero. I want my daughter to be just like her.

I want her to be alive.

EDITOR'S NOTE.  My friend passed away a few weeks ago.  She never lost her vision to Usher syndrome.  Somehow that doesn't seem to matter at all.


kb51kw69 said...

Thank you for sharing this. It's so true. Most my younger sister and I were diagnosed with Usher Syndrome 15 years ago. My sister was panic-stricken over the idea of being both deaf and blind. What would happen to us? Well, my sister died 7 years later of cancer at 49 - well before the horror of being either deaf or blind. This spurred me on to living in the present - instead of waiting until I could retire to move to Colorado, I moved 4 years ago. I've taken up backpacking, kayaking and just started horseriding lessons AT 60! There is life after Usher Syndrome. We all need to live it!

Donnie said...

I am so glad that I foudn this blog! My husband and I have a neighbor that is very near and dear to us. He, too, was born with Usher. It seems that his vision has deteriorated considerably just within the past year. He is SUCH a joy of a man! He helps us in just as many ways as we help him.
However, I do worry because he seems in so much denial. He puts off learning tasks now while he has his sight that will assist him once it is gone. I pray that there is some kind of cure, but until that day, what are some other ways that my husband and I can encourage and support him?

KMU said...

To Donnie - My 20 year old son with Ushers II is also in this same denial. But maybe it's a good thing. He says, "Mom, I'll learn it when I need it - I'm too busy living right now." So I let him live.

UbAh said...

I have been following your blog for a short time now, due to having people with US in my life. My girlfriend, her twin sister, and their cousin all have US.

Unfortunately what has finally inspired me to finally comment is some bad news we heard from an oncologist. The girls found out that their cousin has colon cancer a short while ago, and in the course of treatment his oncologist told the family that a link has been found between usher syndrome and colon cancer. As you can imagine this news hit hard with my girlfriend expressing "I am just now getting used to the fact that I am loosing my eyesight and now I have this to worry about"

I am sharing this news because after I read this posting a while ago and heard about the cancer diagnosis of the cousin, I started to wonder if there was a link but I could find nothing online stating there was in fact a link. So even though I had seen many people with US having cancer, the absence of anyone reporting a link convinced me I was imagining things. Now with the news from the oncologist I feel that I need to share it online so more people with US hear about it and start a screening routine like the girls and I are doing.

Mark Dunning said...

I have not heard of a link between cancer and Usher syndrome, but I will ask the question of the genetic experts in the field.

UbAh said...

Thanks, I look foreword to hearing what the experts have to say besides just "there is a link", because I could find nothing online about it.

Mark Dunning said...

To follow up to an earlier comment, as a member of the Coalition for Usher Syndrome Research, I am fortunate to have e-mail access to almost all the leading experts on Usher syndrome. I asked them about a link between cancer and Usher syndrome. Of the dozen or so responses from some of the leading Usher experts, none of them knew of a link between Usher syndrome and an increased risk of any kind of cancer. - Mark