The Secret Deafie is a series of anonymous columns written by different writers. Our fourth contributor tells us about living with Usher Syndrome.
When I was younger, I was just Deaf.
I, like other Deaf people, held my vision sacrosanct. The idea of losing my sight was an unthinkable terror not to be contemplated for even one moment. The idea was too overwhelming. Too horrifying.
I utterly took for granted being able to see. It was my most effortless sense. My way of being in the world.
From the beginning, I have been a visual being. Vision was – my unshakable companion; my vaulted treasure; my room with a view; my blanket; my Annadin; my karabiner; my anchor; my solace and joy. Life with diminished sight was simply unimaginable.
Back then I did not know what was to come.
I knew a couple of people with Usher Syndrome. Words like tunnel vision and night blindness didn’t mean much and being around them stirred conflicting emotions.
Part of me wanted to do the right thing – to communicate with and include these unfortunate deafies who happened to be slowly going blind – the other part of me wanted to run a mile.
To not feel obligated or hemmed into a corner struggling to communicate with someone who reflected so starkly my own, scarcely acknowledged, darkest fears.
I know I was not alone in this reaction. I saw it in the eyes of Deaf people around me. In their reluctance to really get to know a person with Usher, the person in the background who always stay an acquaintance. It rang clear in their comments, which usually went something like ‘I would rather die than go blind’.
Then the unthinkable happened. I was diagnosed with Usher Syndrome.
I’d been deaf all my life and now I was told I would slowly lose my vision. Suddenly the slightly irrational fear of all things Usher – became rational- it was real, it was happening- a Hiroshima in my retinas.
I finally became proper friends with my Usher acquaintances. They became a source of wisdom. But, still I feared becoming like them. Even more, I feared seeing pity and avoidance on the faces of other Deaf people. I knew that deep down many Deaf people are a bit afraid of people with Usher, just as I had been.
I watched for every clue that my vision was deteriorating. I needed to make sense of the spectre looming on the horizon. I needed it to be less of a huge and scary unknown. I was desperate to know what the changes to my vision would actually ‘look’ like?
Would I be aware of the sides of my vision closing in? Would there be an evil black blobby frame staining my view? I grilled my Usher friend, ‘What does it look like?’, ‘Do you see black round the edges?’. His answers were unsatisfactorily vague. ‘I don’t know it just feels like it has always been this way. The corner of my eye has gone. It’s kind of like looking down a toilet roll but there’s no black tunnel walls. I am just not aware of space to the sides. It is like it doesn’t exist. I don’t see anything instead – just nothingness’.
Tunnel vision crept up on me. First at night time and then in the day. I lost the first 160 degrees of vision without really noticing. My eyes compensated by scanning from side to side, leaving me slowly more and more exhausted without really being aware of why. Suddenly people a little ahead of me in the street bobbed along without feet and then they bobbed without legs. Then I had no lower vision left.
But hey, It really isn’t that bad. I can still see. It is just a different kind of seeing. A slower and more considered way of seeing. Like a cameraman with a restricted viewfinder, I have to think more carefully about what I want and need to see. I get to direct the shots and see the world differently.
The fear was far more crippling than the reality. Bring it on- at least then I am dealing with it, rather than worrying about it.
What I see is far less intimidating than those harsh black inkblots on opthalmology simulation pictures. I think these are over-simplistic, rubbish and obviously invented by a sighted person.
My experience is much nearer to that painted by the artist, John D. Lewis.
He paints his blurred periphery in a startling sluice of colour, with a face that is fading out rather than obscured, whilst my vision seems to be encroached by a kind of grey mist. Like dirty net curtains that I can’t really see but just know are there.
There is no sanctuary in a room full of Deaf people anymore. That palpable and utter sense of relief and belonging no longer applies. Without peripheral vision, people’s hands dart in and out of view. Their handshapes blur, although their faces are clear as day. Deaf people now mumble their signs like hearing people swallow their words. All communication is stressful.
In dim light, it is easier to read lips and face expressions, than to try and understand hands that disappear below eager faces. I piece fragments together. I can see enough to get by but I will miss a sign in every utterance and the guesswork is exhausting. I am always filling in the gaps. I’m have become ‘hard of seeing’. It’s tricky, stressful and sometimes quite frankly I’d rather stay at home.
Now I scan the Deaf faces around me for every wince or shadow of fear or obligation as I stumble on a curb or missed something that was said. They are only etched in the glances of those who don’t really know me. They grab my arm without asking and don’t trust me to say what I need.
Usher is perplexing and hard to explain. I can see perfectly well when walking along alone but as soon as I have someone walking beside me, I can’t keep track of where they are. I can no longer do that unconscious synchronised tandem stroll that deaf people do when chatting along the street. If I glance round I lose my place on the view ahead and become disorientated and clumsy.
Years ago, an Usher friend asked a group of deafies, who were having a restaurant meal, would we mind if he walked back to the station alone, and meet us all there to catch our train. It was dark. We were in a busy city centre. We all exchanged looks and someone made a comment about him being stubborn in not accepting help. Now I understand. We offered the wrong kind of help. We needed to trust that he knew what he needed. We needed to be guided by him, not trying to lead him in our well-meaning but thoughtless and clunky way.
Those who really know me, know that I can deal with it. To ask for help if I need it. They trust me to get on with it. Life just isn’t that bad. There’s a hell of a lot worse things than Usher. Try dying for one. Or constant pain, depression or chronic fatigue. These are the things that would really stop life being fully lived. Usher simply makes life a lot more inconvenient, it doesn’t stop it in its tracks.
As for wishing you were dead rather than deaf and going blind. I have only one amazing word for people who think like that, “Resilience”. Humans have an astonishing capacity to adjust.
Fortitude is a new word in my vocabulary. Each time I stub my toe for the second time in an hour, I try to think of it as a tiny bit of fortitude I never knew I had. As some things become more difficult, new hobbies and interests present themselves. Things I would never have contemplated before. In fact I contemplate a lot more about everything.
I’ve learned that life has to be lived in the present moment or it will be wasted. A lot of people do not seize the here and now until it is too late. They spend too much time dwelling on the past or the future. Worrying too much about next week, next year or getting old.
The only certainty with Usher is that ‘tomorrow’ will get a little harder than today. I like to see Usher as a rude nudge into a more positive mindset. My ‘todays’ are a lot more satisfying, now I can’t take my ‘tomorrows’ for granted.
Yes, being told I would lose most of my vision felt like the rug had been pulled from under my feet, but once I found the ground again it felt more solid than before. There’s a perverse sense of challenge in seeing differently, in living through an unusual lens. As I look up at the sky, I can not only see nimbus clouds and aeroplane trails, but also flashers, floating specks and quivering crackle glaze. My vision has a new dimension. Strangely it helps me to see more clearly, that we are just a sum of our parts, our bodies are fallable and life is what you make it.
Seeing out of step with everyone else is strangely liberating. I’m different but free to see my own way. It’s frustrating but fascinating. It’s certainly never dull. As my vision dims, I sometimes feel I am floating in a surreal bubble, but from this bubble I actually get to see what seeing is all about.
Do you have a story or experience you’d like to share? If you’d like to write a Secret Deafie column, just email email@example.com
The Limping Chicken is the UK’s deaf blogs and news website, and is the world’s most popular deaf blog. It is edited by Deaf journalist and filmmaker Charlie Swinbourne.
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