‘Living with Ushers’ by Michelle Hauschild http://www.mivision.com.au/living-with-ushers/
Edan Chapman was born with the rare genetic disorder known as Usher syndrome. He is – and always has been – profoundly Deaf. Now he is going slowly blind from retinitis pigmentosa. This is part one of a two-part story on Edan Chapman’s battle with Ushers.
We are all defined by our genetic makeup– 25,000 pairs of genes that determine the colour of our hair and eyes, our height, our sex, our physical and mental health… the list goes on. Within those 25,000 genes there are many mutations, some insignificant, others that cause problems and lead to diseases.
Two of the leading causes of adult blindness – glaucoma and age-related macular degeneration – are caused by a complex combination of both lifestyle and genes.
Sixty per cent of infant blindness can be put down to congenital cataracts and glaucoma, optic atrophy and eye malformations and retinal degeneration.
Retinitis pigmentosa – the collective name for a whole group of genetic retinal conditions – is known to be caused by over 55 specific genes and more are being discovered all the time.
Quite simply, if mutated, these genes damage the light sensitive photoreceptors; known as the rods, found at the periphery of the retina, as well as the cones at the centre the retina. Initially retinitis pigmentosa diminishes peripheral and night vision. Later it affects a person’s central, detailed and colour vision.
The symptoms typically appear between age 10 and 30.
Edan Chapman, who lives with Usher syndrome Type I, first became aware of the symptoms of retinitis pigmentosa when he was 13.
Cup your hands either side of your eyes and it may start to give you some idea of how limiting it is to have just 5 per cent of your peripheral vision.
But it is no longer just the peripheral vision that is being stolen away. Over the past couple of years Edan’s central vision has been getting more blurry. It is harder to focus. Sometimes he has a film over his vision that just refuses to go away.
Describing himself as a ‘photographer of all trades’, Edan says while fading sight has limited his working lifespan, “that doesn’t mean I’ll ever stop shooting photos… you’ll have to pry the Canon 5D out of my stiff fingers in my coffin to stop me from continuing with that passion”.
Edan’s story is so powerful; his journey so unique, mivision has chosen to tell it in his own words in a two-part series. In this first article, Edan relates his struggle to accept his fate, and how he discovered his passion for photography.
For years I struggled with the knowledge of my doom. Being Deaf is NOT an issue. I don’t give a rats. It’s great. I have a beautiful language and culture.
Blindness is the snake on the apple tree for me. The horrible knowledge… aahh.
I had one of the worst introductions to Usher syndrome I know of.
I’ve been a nomad since I was a kid. Dad was the ‘grass is greener on the other side’ kind of man. The parents split when I was a baby and mum lived in Byron so that was my base and I would spend a year with mum then go to wherever dad was – in Germany, Australia or New Zealand (Wellington.)
I got a cochlear implant when I was seven. My mother insists that she explained it was because of my Ushers but all I remember is the doctor saying “You’ll be able to hear”… the doctor probably thought it would be interesting to see if it worked… unfortunately, I threw out the external part when I was 11 because all I got was white noise and I was sick to death of the whole thing.
My first trigger that something was happening to my sight was when I was 13. You need to remember, your brain is an amazing tool. It adjusts to an incredible degree and can fool you into believing you are fine. I was playing spotlight with the usual gang of kids. I started to notice I was always getting caught because I simply could not see properly at night. Once I tripped and scratched the corner of my temple and broke into a cold sweat – I had almost stabbed my eye out on a tree support post. That night I asked mum in frustration why I couldn’t play properly. She stopped, looked at me and said “Don’t you remember?”… I cried for hours that night and just wanted to die. ALL she (and now I) knew at that point was this ONE SIMPLE FACT:
I was going to go blind.
No whens. No maybes. No NOTHING. Just “You’re going to go blind”.
The thing was, mum had no answers either. When? How fast? Tomorrow? The answer to all of those was “I don’t know”. I cried for hours.
I started doing drugs then. Developed insomnia. Became a loner. Felt sorry for myself. I was suicidal from 13–16. If I could have just disappeared, I would have. Life seemed pointless.
I love art but gave it up except for my perennial sketch pad and pencil I would have in my bag when I hitched all over the shire in my late teens. I did stupid things.
It took me years to realise I was still okay and it was not just some Russian roulette of waking up without sight. I wish I had known that all that time ago, it would have made things vastly easier. I often wonder if the stress actually contributed to making my vision disappear faster.
As it is, I’m looking at a 13” laptop screen from roughly 50–60cm and I can just see the whole screen and the tips of my knuckles as I type. So that’s about 5 per cent of my peripheral vision left.
The interesting thing I’ve found is that the information I have read about Ushers is pretty misleading. It is NOT JUST the peripheral vision. Over the past year or two I’ve noticed it’s the central vision too, it’s getting more blurry, harder to focus, sometimes I have a film over my vision that just refuses to go away, I have to blink several times and rub my eyes to get it to go away and sometimes it just doesn’t. Daytime is getting harder to see – despite my night blindness, I prefer night. The light is stable. It’s consistent. So my pupils can adjust to that level and STAY AT THAT LEVEL.
Cloudy overcast days are the worst. The glare! UGH. I detest these days. Ironically, the ‘headquarters’ of deaf blind services is in Melbourne, the city with the most consistently overcast days in Australia.
Finding the Camera
Over the years though, I realised something important. I can still see.
I had wasted over 10 years avoiding art and stuff because I thought it was pointless but in the end, all I did was rip myself off.
At 24, I got a scholarship to The Learning Connexion (a School of Creativity and Art) in Wellington, and decided to go for it. I completed the course then got side-tracked by life… one day I simply realised I was still taking photos with my old Sony 3.1 megapixel camera that my uncle had given me when I was 21 and that I loved taking photos. I got angry with myself and enrolled into TAFE when I was 27 and did Cert II in Tools for New Media and then moved to Melbourne to DO SOMETHING WITH MYSELF. I also went and bought a Canon 5D Mark II DSLR. Expensive as hell but worth it.
In December 2011, I bid for and won the position of the Official Photographer of the 2012 Australian Deaf Games in Geelong. It was one of the hardest jobs I have ever had to do but I kicked ass. Yeah, you heard me right. A nearly blind Deaf guy got given the job and did a fantastic job at that.
I’ve tried to get more work as a photographer but sadly, the Deaf community is not a big one in general terms and most don’t have great jobs so we’re not a rich demographic so I endlessly get asked to do work for free.
To be honest, if I didn’t have the blind pension (ha!) I would never be able to continue my passion for photography or be able to do what I do. It’s amazingly ironic. If I wasn’t going blind, I wouldn’t be able to be a photographer!
These days I’m just focusing on trying to take photos of things I like, I don’t make any money. I’ve pretty much given up on ever making any kind of living from it.
That’s the worst part of what’s happening for me now. I’m just taking stuff one day a time, a few months ago I almost broke down with stress and all so I cut out everything and started again. That was the best thing I ever did. Went to Cambodia for a month alone and that was quite the experience. Cambodian people are so beautiful. I love to smile. I love to hug people. The western world is a bit sad that way, if you smile at people in general they react first with suspicion or just ignore you.
In Cambodia, hurtling down the dirt roads in a tuk tuk, everyone who looked up at me and saw me grinning my head off and waving did the exact same back to me. I loved them and they somehow just knew that and loved me right back. It was EXACTLY the tonic for my tired soul and I came back with new hope.
Sure, it’s s***. It’s f*****g hard. I cry a lot. More each year it seems. But the funny thing is sometimes it’s actually tears of appreciation, of being able to see 18 years after I thought I would go blind any moment. God, what a gift! A flower. A dead insect. A blade of grass. The top of a mountain. The mist. A woman’s eyes. Happiness on a face. Hugs. Smells. Touch. I’m still pretty alright and I try very hard to remember that every day.
Next issue we will cover the second part of Edan’s journey with Usher syndrome.