Approaching Blindness

I have terrible eyesight. I cannot see in the dark at all. Pubs, bars, restaurants… any place with subdued lighting is out of bounds. Even a dull wintry afternoon presents a challenge. As does, perversely, a bright summer’s day as I cannot deal with brightness and need special glasses to cut out blue light. My colour vision is so poor that I cannot tell green from blue, purple from brown, or pink from orange. My peripheral vision is non-existent. Things seem to magically appear in front of me as if out of nowhere, and navigating through a crowd of moving people is hazardous for all concerned. But once, y’know–although it seems so long ago as to be in a previous life–my vision was fine. The deterioration has come about mostly because of something called Usher Syndrome, named for Charles Usher, a British eye doctor who recognised that the condition was hereditary. The following is a personal ‘map’, an account of those landmarks by which I measure the gradual loss of my eyesight.

I was born in 1966 with a hearing loss. Back in the early 1970s that meant I had to have a pocket sewn on to all my shirt fronts so I could clip on one of those pink-grey boxes, with a thin cable leading up to an ear piece. Morrissey made them a fashion accessory in 1985, but by then I’d long given mine up for a more discreet behind-the-ear model. I’ve never really considered the hearing loss a disability. I struggle with phone conversations and prefer to watch TV with subtitles, but that’s about it. No big shakes.
The loss of sight, though, is something else. Syndrome means a disorder or disease with more than one characteristic, and for people with Usher Syndrome, that means the loss of hearing and sight.
For many sufferers of Usher’s the Retinitis Pigmentosa (RP) doesn’t kick in until around puberty, and this was true for me, although it wouldn’t actually be diagnosed until much later.

The first landmark on this map is at fourteen: I recall struggling to complete my paper round on dark, wintry evenings. Delivering to those houses with which I was familiar was fine, but if I was given someone else’s round to deliver, then it was a nightmare. I remember returning to the papershop on one occasion in tears of frustration, dumping a sack of undelivered newspapers on the counter and storming out. I knew my night vision wasn’t as good as other people’s, but there was no real inkling that there was an actual problem there. It was just one of those things.

After school I started work in the stores of a local HGV garage. One day, around my sixteenth birthday, I noticed a strange impairment in my right eye. It looked a little bit like water coursing down a windscreen. If I moved my head the vision wobbled slightly. Mum made an appointment with the doctor for the next day and he immediately referred me to the eye clinic department of the local outpatients with a suspected detached retina. This diagnosis was confirmed and after a day flat on my back to let the retina settle down, I underwent surgery to tack it back into place. The result was a mixed bag. Without surgery I would have lost all the sight in that eye. As it was, I recovered most of it, but with some creasing (I can’t explain it any better than that) to the central part of my vision. However, with both eyes open this creasing disappeared as my left eye compensated for the damaged right.

At seventeen I bought my first motorbike, a little Yamaha FS1-E. It possessed a 6V headlight scarcely brighter than a candle, a fact brought home to me the first time I tried riding it at night along one of the local country lanes. I nearly ended up riding through a hedge when I mistook a left-hand bend for a right. Decision made: no more sorties down unlit country lanes, a decision only reversed when I got bigger bikes blessed with twin halogen headlights, bright enough to crinkle leaves and melt frost.
An inkling was starting to form that I had something wrong with my vision, but if I mentioned my night-blindness to anyone–and I’m including my doctor and optician here–it got brushed aside. Eat more carrots was the advice my doctor gave, despite there being no other evidence of vitamin A deficiency, and my optician told me that it was likely a side-effect of the lasers used during the operation on my detached retina.

At twenty-five, I wanted to do a bungee jump for charity (God knows why), for which I needed a doctor’s certificate. My doctor passed the buck to the eye specialist, worrying that a bungee jump could somehow cause my retina to become detached again. By pure chance, as I waited to go in the consulting room, I didn’t see or hear the buzzer or flickering light that indicated the specialist was free to see me–mainly because I was engrossed in a book (Robert Rankin’s ‘The Antipope’, in case you’re wondering). The specialist, noting my hearing aid and complaints of night-blindness, mentioned the possibility of me having something called Usher Syndrome. This was the first time I’d heard of it. He asked me if I had difficulty finding objects: say, if I dropped a pen or coin? Yes, I did! He examined the back of my eyes and confirmed I had Retinitis Pigmentosa and explained what it was. My retinas were degenerating. Where a normal person’s retinas are a healthy uniform pink criss-crossed with capillaries, my retinas had lots of black speckles where the cells had died off, mostly those around the outside of the retinas. This is the classic pattern of RP. Remember your biology lessons at school, where you learnt about rods and cones? Put simply, the 120 million rods are the photoreceptors responsible for detecting light and the 7 million cones take care of the colour. With RP, the rods die off first and they are most abundant on the periphery of the retina. So, the sufferer loses night vision, followed by the peripheral vision and eventually, as the cones degenerate, colour vision. I took a field of vision test which said I was still within the limits to safely ride my motorbikes, and it was down to me to use my own discretion as far as travelling at night. Which was something I’d been exercising for nigh on ten years anyway, so I went away and lived my life as normally as I could, while I could .

By the age of thirty-two, I became aware of something I had not been prepared for, and that was an acute aversion to brightness. At first I thought it was just the glare suffered by all spectacle wearers, and then maybe a slow response to changes in light, as I noticed it particularly when leaving the dimly lit second hand bookshops I frequented. Stepping out onto the pavement I would have to stand still for several moments to allow my eyes to adjust to the daylight. And even when they were adjusted, I still found myself shielding my watering eyes. It didn’t have to be sunny, a bright sky could be overpowering, or the surface of a lake, or snow, or even fog.

At thirty-four I sold my motorbikes. No amount of concentration could make up for the kind of sight deficit I was now experiencing. I never suffered a single accident–or caused one, I might add–as a result of not seeing something, but it would only have been a matter of time if I continued.

At thirty-seven, I complained to my optician that the new glasses she prescribed didn’t seem to be suiting my eyes so good. She took a closer look and told me that the problem was not the prescription, but the actual lenses of my eyes: they were clouding over. Cataracts. This hardly seemed fair; cataracts on top of everything else. A year later the cloudy lenses were removed and artificial lenses inserted. The upside of this was that I now no longer needed glasses, except for close work like reading or using a computer. The downside is that my eyes now no longer have any focussing power. The artificial lenses are not attached to muscles the same way the real lenses were. They are fixed. Normally, the irises help a little by dilating or restricting, but my irises are almost permanently dilated thanks to my retinas insisting on every spare photon. This also leads to another unfortunate side-effect where light strikes the edge of the artificial lenses, causing haloes to appear round points of light. RP and artificial lenses do not make good bedfellows.

At thirty-nine I had a field of vision test which confirmed that in twenty-five years I have lost around 80% of my eyesight. Whereas once I complained of not being able to spot a dropped pen or coin, now I can easily lose larger objects, like a pair of shoes or a small daughter.

I’m unsure what the prognosis is. One specialist seemed to think I would go completely blind while another said total blindness from RP is unusual. I guess I’ll just have to wait and see (haha). Optimists point to the advancing light that is stem cell technology, but I refuse to take heart. Maybe the next generation will benefit, but … better to accept your lot than raise your hopes, I feel. Even so, deep down, I do hope. You have to.

December 2005