The beginning

I lived for many years as a fully sighted person knowing that I would lose my vision, when I am already deaf. Receiving the news in my late teens, that some day in the future I would go blind was surreal, because the eye disorder that I would go on to develop – retinitis pigmentosa –  had not yet claimed any of my sight. I only knew that it would, because I carried the gene for Usher Syndrome. It was a bit like being told that you were HIV positive back in the days when developing full-blown AIDS was inevitable. It was a ticking time bomb. I did not know when it would go off, or how it would look when it did.

I saw myself as ‘Usher Positive’, knowing that one day the RP would combust. I lived with uncertainty and fear, which echoed the way people talked about HIV in those days. Usher is something you have in you that you cannot get rid of – knowing that it will wreak havoc and make life pretty damn inconvenient, but unlike AIDS back then, it wouldn’t kill me – and that’s pretty damn positive too.

Red centre which looks like a pupil and could be a planet or hot centre surrounded by blue exploding matter which looks like an iris. The whole image looks like an eye.

I spent my twenties looking for any conceivable sign that sight loss had begun. I was hypervigilant, always questioning my eyes.

Back then I was a visual person. A very visual one, and inside my head I still am. For most of my twenties I had really sharp peripheral sight. I could sometimes speechread snatches of familiar jaw movements or catch signs in the corner of my eye. I mastered that great game of London transport, clocking the glances of other people on the tube and reading their intentions without looking up. I was one of those annoying people who walked around London reading the newspaper, without bumping into anyone – using just the sides of my eyes to see where I was going.

In my early twenties my vision was still normal. I aced the field tests and was comfortable even in the darkest of places, but it was also around that time that I started to get little clues. I saw black cats, shadows that darted across my lower vision, and when I looked down for these feline friends there was nothing there. Each time I looked at the sky, my vision would be teeming with floaters, little specks, circles and strands, and at other times I was startled by flashes of light like a camera bulb going off near my face. Over time the display developed into a psychedelic show of white lights inside my eyes.

Glare has always been a problem for me. Photographs of childhood sunny days always showed me squinting. Now, I developed a hatred of naked bulbs and a preference for softly lit rooms. This was around the time that ambience lighting became fashionable in pubs and restaurants. Gone were the bright wall lights and swirly carpets. Dinginess and dimmer switches were in. Mood lighting was my friend then, as it soothed my eyes, but later, as night blindness crept upon me, I would come to feel my heart sink as the lights dimmed.

The first tiny hint of problems in the dark came at age 23. I lived on a narrow terrace street cluttered with wheelie bins and parked cars mounted on the pavement. I regularly enjoyed drinks in smoky, candle-lit pubs and walked home with friends. I realised that I needed to pay a more attention to the street furniture than they did, they could see it out of the sides of their eyes as they looked at each other in the dark. I could see it, and them perfectly, but I could not see everything at once.

These indications were fleeting and I put them to the back of my mind, as I rushed to fit it all in before sight loss began. I cycled everywhere and ran – in the dark too – and travelled and studied hard. At 28 I started a training course – one bloke who often sat next to me always jiggled his feet which drove me mad when I was trying to understand the lecture. The constant peripheral movement was distracting. By the end of the course, three years later, I was no longer so bothered by it. I could still see his foot but the movement had faded.

Around this time, I started to question myself – is this the sight loss starting? Is this really it? I remember asking a friend to show me how far in front of her she could see the floor when she looked straight ahead. It seemed to me that my floor was blurring but I could not be sure. On some days I would look at the sky and see cracking, flickering and shimmering and wonder if it was my imagination that my sight seemed to be breaking up. It was so subtle it was hardly perceptible. I look back now and realise that it was the start.

Night blindness crept up. I went on holiday to Croatia with a deaf friend and while we were chatting on a humid evening wandering around the dark harbour side, I bumped into a bollard and then a small child, and caught an expression of concern on her face. I now needed to concentrate on where I was stepping but as long as I was not distracted there were still few mishaps and everything looked normal.

Soon afterwards, I stopped driving at night. Taking control of the wheel had started to feel strange and unreal – I couldn’t judge speed so it seemed like the car was floating because I could no longer see the blur of passing tarmac. The oncoming headlights started to flare at me, making my eyes hurt.

For a time, I had a bizarre sensation of strobe-like vision in the dark, like I was looking at a series of still photographs. A street scene would be normal, but people walking into view would suddenly appear and then move – staccato fashion – like a flick book animation. I could see them perfectly but not their movement. It was as if my brain had developed a judder, like a webcam gone jerky, because my eyes had slowed their upload speed. This effect only occurred at night and stopped after my peripheral vision deteriorated completely, or perhaps when my brain started to compensate.

There were clues that my daytime vision was about to be affected. I was skiing down a mountain in Andorra in sudden white-out conditions and completely lost all sense of contrast, I constantly fell while my friends skied on oblivious. It was disorienting. The sky and the ground became one, and I could not see which way was up or down.

My sight didn’t disappear, everything was still there but the resolution dropped – as if I had fewer pixels to do the job of seeing with. My eyes slowed down and became tardy. People would wave and I would take longer to react, I would see them but the information travelled sluggishly to my brain, so it seemed to take forever for it to tell my muscles to turn my head. It was almost like a few seconds of blindsight. If people were in the room, I would chivvy my brain to be more alert, so that I would not look like I was ignoring anyone. My eyes were weakening. My brain now had to be on full throttle.

Gradually my daytime vision became like a standard TV instead of widescreen – I just didn’t see as much out to the sides. It was not all that disabling at that point, but I knew RP had truly arrived because I had to hang up my squash racket. I started to lose game after game as the balls keep whizzing past without me seeing them until they rebounded off the wall.

I suddenly found it tricky if people used sign language with a toothbrush in their mouth – I was relying more on lip patterns and facial expressions to understand signers – because without realising it, my lower vision was blurring and I could not fully register their hand movements.

At night I was finding that I could see less and less. At 25 I had been hypnotised by a vast sky full of stars in the Jordanian desert, which layered into infinity as my eyes adjusted normally. Seven years later gazing up at another desert sky, I could only see the brightest. I found myself biting back tears of frustration while trekking out of a canyon at night in Peru. It was a mile deep with steep sides which blocked the moonlight and made the night blacker. I suddenly realised the weak beam of the torch was not enough to detect the end of each switch-back and I kept walking into the rocky canyon walls.

My first son was born. My vision had levelled during pregnancy, but a year later as my hormones were adjusting, my sight dropped. Now I was properly in between blindness and sight but my daytime vision was still good enough to drive, because the front of a car bonnet was well within my funnel of vision. It was not until three years later, after my second son had been weaned from breast milk, that I noticed the world had suddenly gone blurry. The ground fell away, people lost their legs and started to bob along. I needed glasses for the first time to read the subtitles on TV, but even with them everything was slightly out of focus and shimmering. There was distortion in the middle of my left eye where straight lines had gone wavy. I watched with dismay as that patch of vision vanished to nothing in just two days. There had been a bleed in the centre of my eye and I now had a hole in the middle of my left macular. My precious kestrel vision was gone, and reading without my right eye open became impossible.

The Usher moments were now coming thick and fast. I was 35 and immediately knew my driving days were over. I took the DVLA field of vision test and the optician’s face looked pained. I think he was surprised by my casual calmness, but for me, it was a question of when, not if I would stop driving. That day just happened to be the day that ‘when’ arrived. I’d gone from missing just a few spots in my field to missing nearly all of them. The Usher bomb had detonated. There was relief and no more waiting.

7 thoughts on “The beginning”

  1. Wow – what an incredibly powerful and brave account. Much of the RP account resonates but I have been neither as brave nor as stoic as you – all the very best.

  2. Oh the jiggling legs, I remember them well, and perhaps was also an offender! I am already a devoted reader of your blog xx

  3. I’m a fellow Usherite. Yes I recognise a lot of this. My daughter said “moon” for the first time today and could I find it? Looking for one small object in a big sky took a while!

  4. You’re a fantastic writer. Please don’t stop. Also this subject matter is especially riveting because I have a family member with RP. What a gift you are giving in helping others understand better.

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