Flashers

No I am not talk about dodgy men in raincoats. These flashers are worm like blobs of intense, pure-white light which wriggle or zoom across my vision like fireworks before disappearing. They occur with my eyes open or closed. I used to see brief flashes of white light inside my eye like a flash bulb going off. It felt just like someone was taking sneaky photos of me when I wasn’t looking. I read up and realised that it was my doolally, slightly incontinent, retina discharging electrical impulses at random.

I was quite frightened by my private light show. Each burst seemed to be the swan-song of a cell dying in my retina. A furious, ostentatious death that said, “Hey! Look at me, I’m not going without a fight”. Then after 20 years of flashing, I thought a more positive way at looking at it, would be to believe that my retina is just hungry for missing information. Instead of putting its feet up and getting lazy, it is putting on a pretty display instead.

The lights are more obvious when I cough, sneeze, yawn or blow up a balloon. RP has turned my retina into a sensitive little flower. A mere sneeze creates enough pressure or jarring to make it dazed and see stars, like a cartoon character after a sharp blow to the head.

I sneeze and there is no time for a ‘bless you’ – a dozen white lights shaped like curved lozenges are already moving in front of my vision obscuring bits of the whatever I am looking at. The fat glow-worm shapes move with purpose in all directions at a steady speed until they burn themselves out and disappear.

The lights have a unique neon intensity – a bit like what you might see if you could look straight into the sun. It has a purity that reminds me of reports by people who have survived near death experiences, who describe a blinding, serene tunnel of light.

I don’t see anything as trippy as a subway to heaven or glowing angels, but I do wonder whether seeing this special intensity of white light is something that happens when the eye is low on oxygen. Certainly my light show seems to ramp up if I am: struggling to breath; exercising heavily; leaning down too suddenly; or when I have a heavy cold. All these things probably cause my eyes to be starved of oxygen because the blood flow to my retina is narrower and less efficient than everyone else’s. I just have to wait, and when the blood flow gets going again, the lights will stop for a while.

After a particularly spectacular light show my vision feels patchy with after effects like those you get when someone takes a flash photo too close by or when you are dazzled by a torch.

I never thought I would get ‘flasher envy’ but mine do seem quite boring compared to those that some other people with RP describe: catherine wheels; swirls; smoke rings; half moons; and volcanoes in all kinds of fancy colours. These sound so much more exotic than ones shaped like plump maggots!

Damon Rose wrote this fascinating article about his experience of total blindness being full of colour and light, rather than blackness.

This set me thinking- my eyes certainly plays some weird tricks on me. Sometimes sitting in the dark, I will see a glow out to my sides in the blind bits of my vision. I feel surrounded by light as if someone has put a lamp on, or the sun has suddenly risen. I sometimes feel that the lights are on, even when they are not. I won’t notice if someone flicks the lights on or off, because to me it’s already bright round the edges. It is very strange, as if the dodgy bits of my retina have solar panels and are firing up in the dark all by themselves.

Some people with RP describe waking up in the middle of the night convinced that it is morning because sunlight appears to be pouring into the room. They might even get up before realising that it is actually still dark outside.

This is the paradox of sight loss. As my eye stops seeing, the blind bits manifest more as sensations of light than darkness. Things are slowly changing. I am losing something but also gaining a whole new dimension that other people just are not aware of.

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Is it possible to have a big B in DeafBlind?

For people familiar with the Deaf Community you will probably know all about the big D/ little d debate. As much as I think it may have had its day as a metaphor, I do think it is a useful comparison for adjusting to life with Usher.

People who are Deaf with a big fat out and proud ‘D’ shout about their deafness from the rooftops, they often also embrace sign language and Deaf culture and are not embarrassed telling the umpteenth hearing person that day to speak clearly or write things down. They generally see it as the hearing person’s problem if they cannot communicate with a deaf person.

Some little d deaf tend to be more reticent. They might not be so upfront about their communication needs, they might try to blend in and pretend their deafness isn’t really a problem. They might try to wing it and bluff their way through conversations. They might be a little bit ashamed of being deaf.

Is there an equivalent for Usher people? Is it possible to be big ‘DB’ DeafBlind or little ‘db’ deafblind?

Picture shows a man opening his shirt to reveal superman's chest with a fingerspelled D from the British fingerspelling alphabet and a printed capital B to spell DB

I think so, yes. I know which one I would rather be. It’s got to be healthier to accept both deafness and blindness – than to try to conceal either disability – to wing and bluff, and crash your way through life is exhausting. It makes me feel like a prize ‘A’ clutz. It erodes my confidence and eats away at my self-esteem.

I think that many people with Usher, even those who are proud of their deafness, are little b blind. They are often ashamed of their vision loss. We might try to hide from it and pretend that it is not happening.

So what is preventing us from all becoming big DB DeafBlind?

If I were suddenly marooned alone on a dark planet – I would not hesitate to use a cane to explore. Who cares? No one can see me – right? So what makes it such a difficult thing on planet Earth?

In two words: social embarrassment.

I would say that the biggest barrier is actually the reaction of people around me.

Here are two scenarios.

A little d deaf person discovers their Deaf identity and decides to embrace their inner Big D. Members of the Deaf Community slap them on the back and think ‘Good for you!’, ‘What took you so long to  find the way to Deaf nirvana?’- or something along those lines. The person announces that they are going to start using communication support at work, perhaps an interpreter. The Big D deaf all think “hallelujah! About time! You won’t look back”.

Is a deaf person using an interpreter for the first time ‘brave’, ‘heroic’ or ‘inspirational’. Would people say, “We still love you even though you have decided to use an interpreter”? Well Deaf people certainly wouldn’t – because they see it as a positive thing.

Compare this with another scenario. An Usher person decides to start using a cane. They want to embrace their inner big DB and  to make a positive adjustment. They are not congratulated in the same way. There are platitudes, grunted commiserations and lumps in throats. These often come from other D/deaf people. Using a cane is seen as a negative thing, as if a sad threshold has been crossed. It’s awkward like someone died.

Canes, guide dogs, hands-on sign language, communicator guides are all seen as a symbol of what has been lost, rather than the new independence that has been gained or maintained. Surely these things should be celebrated as keys that unlock doors. They are tools for making the best of bum cards.

Yes of course I am sad to have lost a lot of my vision and to know I will lose more.  But using a cane or a dog if and when I need one can only be a positive thing. Perhaps the earlier the better so that I can glide into being DeafBlind without beating any retreat from the lifestyle that I have now.

By the time an Usher person is ready to take action to equip themselves with the tools of a full and independent life, they have probably done a fair bit of private grieving and feeling sad. They are moving on a bit and taking control. This is a hugely positive step. It should be a cause for celebration not commiseration. Dealing with other people’s sadness at this time makes just coming right out and saying, ‘This is what I need’ a lot harder.

At this point, I need people to just say – ‘hey, good for you for having the guts to make life easier for yourself’, ‘you won’t look back’, ‘kudos’, ‘respect’, ‘it will open doors for you’.

If people stopped reacting with surprise, pity or condolence then it would not be such a huge psychological leap to make. More DeafBlind people would find it easier to rock their cane.

And if every DeafBlind or Usher person who needed a cane or a dog, had one. The Deaf Community would be full of them, the stigma would disappear making it easier for the next generation of confident DeafBlind people who want to strut their stuff.

Sorting out terminology

DeafBlind.

This word has a kind of finality and dread about it.

But the alternatives are clunky.

Visually impaired is to blind, what hearing impaired is to deaf. Clunkity clunk.

The same goes for ‘vision loss’, ‘partially sighted’, ‘visual disability’.

Blind is a lot more rounded and much easier to say or sign. However, I find it a heck of a lot more scary. Blind is terrifying, right? Well it was until I found out that blind covers a whole gamut of sight loss, just the way that deaf covers a range of hearing loss. You do not have to see nothing to be blind. It fact you can see a whole lot and still be blind.

So being DeafBlind just means you have combined hearing and sight loss of whatever degree.

Right now, so that word just became a whole lot less scary. It ain’t a gremlin or a taboo word to be feared anymore.

Maybe people with Usher should reclaim it like gay people reclaimed ‘queer’ and Deaf people reclaimed the sign for ‘Deaf and dumb’.

I am going to use it in this blog – cos if everyone started using it in its proper sense then it would lose its potency for striking fear into the hearts of all deaf people. It would just mean any deaf person who also has any degree of sight loss. No biggy right?

It would no longer conjure up images of people leading lonely lives in total darkness and silence. That just is not the reality for most DeafBlind people.

Of course many people with Usher prefer just to refer to themselves as having Usher, rather than being DeafBlind. Fair dos. I do too. This will get you a long way in the Deaf Community where most people know a bit about Usher, but try telling the cinema cashier you have Usher and you will get a blank look. Telling them that you are DeafBlind will get you a nice reduction with zero faff.

Floaters

In the earliest stages of RP I used to get a whole ton of floaters, those annoying little specks that hover inside your vision. Everyone gets floaters sometimes, particularly as we age, but people with RP tend to get them in crazy numbers. Now that my vision has worsened, they seem to have disappeared. Perhaps they’ve stopped or maybe I just don’t see them anymore. Good riddance, my pesky, transparent friends, you were quite harmless but I do not miss you.

Although, my floaters have taken a trip, the cloudiness shown in this video is the real deal. It shows very accurately how I see the world on a bright day.

Floaters are bits of waste floating around in the eyeball itself. Tiny pieces of jelly get wanderlust, breaking free and going for a cheeky explore right in front of your eyes. There are a host of shapes: spots like dead gnats; little fibres; cobwebs, clouds and circles. It is actually the shadows of these that we perceive, like some kind of trippy projection by amoebic silhouette-puppets. You only see them when bright light passes through, so they are most noticeable when looking at a blue sky or a computer screen. As your pupils contract with the light, they tighten their focus, and you suddenly see these adventurous strands.

Picture of a tree in a field with a cloudy blue sky and lots of floaters, which look a bit like sperm

Floaters are often annoyingly suspended just where you don’t want them. You can try as hard as you like to catch them with your gaze but they drift insolently, in concert with your eye movements. They never stay still. The slippery blighters just can’t be pinned down. Interestingly, if floaters did not move, your brain would tune them out and you would not see them at all.

For Joe Public, seeing a whole batallion of floaters could mean that you need to see your eye doctor, but those with RP are used to these visual vagabonds, and when all is said and done, they are the least of our worries.

What is it like being Night blind?

I am night blind. Twilight is my worse time of day. This awkward in-between light is when my vision starts to pixelate, becoming more and more grainy as my funnel vision closes in. My eyes stop seeing as well as they can in full daylight, but my brain still thinks it can see enough to skip around normally. Twilight is when I am at my clumsiest and I feel my chest tightening as I realise my vision is fading with the sunset. Only when it becomes fully dark does my brain finally put the brakes on and make me more cautious.

First picture shows a twilight scene with a winding road and a house in distance. The second picture shows the same scene as it would be seen to a nightblind person. It is almost completely dark.
Even a twilight scene can look totally dark to a person who is nightblind (pixshark.com)

Stepping into a dimly lit room has the same effect on my blundering brain. It does not know what it can and cannot see. Shadows will be drawn across my vision and darkness will descend, even if the lighting is just subdued. Your eyes will adjust in seconds, mine will take minutes and probably will not adjust at all. A dimly lit restaurant looks properly dark to me. The detail and colours dwindle. All sources of light become intensified and flare in the darkness.

Some people with RP will suddenly be unable to read the menu and cannot see the food on their plate. I do not have this problem yet but I struggle to communicate so sometimes I bring along a portable light to help me illuminate people’s faces. I have discovered that I can see my companion more clearly via the camera of my mobile phone. On screen they suddenly grow a body, I can see their hands, the table, the condiments and the rosiness of the ambient light. I can pan the room and see everything at once. Everything seems so fresh and bright. Lifting my eyes to look into the gloom once again gives me a jolt as I realise my dining partner looks fragmented, incomplete and shadowy.

Street lights are beacons in a dark street. Imagine camping in a dark field and seeing only torch beams illuminated against total blackness and you will get a sense of the all or nothing that we see in the dark. Walking down the street, it is easy to see car headlights and street lamps, but I have to strain to make out the detail of anything else. The intensity of a light has to be strong to register on my eyes. I will not see the weak glow of a bike light unless I know where it is and look straight at it.

First picture shows a night scene with a car parked in a driveway and an empty road with streetlights. The second picture shows the same scene seen by a nightblind person. Only the lights and reflection in car window can be seen.
Normal vision and night blindness (pixshark.com)
Picture of town centre street scene in dark. Picture is divided into 3 panels. The middle panel shows what person with nightblindness sees. It is totally black apart from car and street lights. The side panels show what someone with normal vision can see- including shops, bollards and street signs.
Middle section of this picture is what someone with nightblindness would see (pixshark.com)
Very dark road scene only car headlights can be seen
http://www.autoinsurancequotes.org

In the dark, I used to be able to see the outlines of furniture and my sleeping children. Now the diameter of my vision is so small that I can see only a tiny part of what I am trying to look at. It’s impossible to scan in a gloom-filled bedroom because I have to stare really intently to see anything at all. I keep losing my place on the scene and it disappears before I can stitch anything meaningful together. The light from the landing scarcely helps because it just makes the rest of the room look blacker.

I have to focus and squint for ages to pick out the brightest of stars in a star-filled sky. There may be thousands but I will see just one or two at a time. I quickly lose them again because I have no point of reference in the dark. There is nothing to anchor my sight to. My funnel of sight is simply too narrow at night.

Trips to the theatre or cinema mean arriving very early because the beam of tepid light from the Usherette’s torch is useless for finding my seat. I make sure my children have been to the toilet before the show so there is no risk of having to navigate the auditorium in, what to me looks like, pitch blackness. I bring my cane which gets me to me seat without problems.

Night blindness perplexes people because you see so much better from one moment to the next depending on the lighting. In the daytime, standing in the queue for a museum, I whip my cane out of my bag, anticipating the murkiness inside, and attract puzzled looks from people who just saw me walk across the street.

It snowing outside as I write this. I’ve always liked snow but since I lost my night vision I really dig those falling flakes. I see so much better when there is a blanket of white to bounce the light around at night. It’s like someone suddenly put the lights on.

What do the blind bits look like?

What happens to the bits round the edges of my vision that I cannot see – the cropped off bits that my eyes send to the cutting room floor? Well, my brain fills in the edges with what it thinks it there.

I might be sitting in a restaurant chatting after a meal, and as I look at the person I am talking to, I will feel that I can see the empty plate below my nose. My brain knows the plate is there, so I see it, even though it is right in the blind part of my sight. Later I look down and am perplexed to see an empty place mat. The plate has gone. I did not see the waiter take it away. I should not be surprised, after all I am registered blind, but it never fails to startle me.

Lady is toasting her male partner in a restaurant and looking directly his face. The image is blurred to show what she can see. She can see her partner and their clinking glasses but not the table. There is a blurry image of a plate in front of her on the table.
Seeing a mirage of a plate that the waiter has already taken away

My brain does a wonderful thing, it takes what I can see and uses it as a template for the rest. I ate from the plate and saw what it looks like – its shape and pattern – so my brain simply uses memory, to maintain a credible image in my blind edges when I look up. It is a mirage – a very convincing one. My brain does not refresh the image, that it thinks it sees, until I realise the plate is no longer there.

I really have to introspect to make myself aware that I am not seeing all I should. My blindness is often unconscious, although now that my sight has worsened I am becoming more and more aware.

There is no edge or frame to my vision, just as there is no edge to yours. In my blind periphery I usually see nothing. People with RP sometimes describe it as like looking out of your elbow, or what other people see from the back of their heads.

At other times, particularly when it is bright, I can see haziness at the edges, a smoky light-grey or white blur, like an invisible curtain encroaching on my sight without me really being aware it is there. It feels a bit like someone rubbed vaseline round the edge of my eyeballs, or like looking through scrunched up cling-film.

I am interested in the experiences of people who have less sight than I do. There is a grim vernacular that surrounds blindness, people talk of ‘darkness’ and the ‘lights going out’. Is this what it is really like? I asked people on a RP Facebook group whether this rang true. Nobody reported seeing black edges or total blackness after they lost their sight. The consensus was that they do not see anything at the edge – there is no extra darkness – just that the edges are a lot nearer the centre. Those with no sight at all say that they see blurry light, or complete blankness – no white or black, just nothing.

People seem to struggle with the idea of partial sight or blindness, much more than they do with deafness. They can block their ears but when their eyes are open they cannot block their sight. Someone clever, probably Shakespeare, said, “The eyes are the window to the soul”. There is something existential about seeing, which means people equate how they see the world with the very essence of themselves. Vision is the portal through which they physically interact with others. Perhaps, they worry that without sight, their own essence would somehow be diminished. I wonder if this is why people flounder when they try to imagine seeing nothing – not blackness or whiteness – just nothing, because it seems close to death.

I think that existential angst accounts for a lot of the deep-seated fear that surrounds going blind. I certainly felt something along these lines before I lost some of my sight. Then I realised how ignorant I was being. The essence of your person does not live in your eyes, or your ears, or your muscles or any other sense. It lies in your experiences, your memories, your imagination, how you communicate and interplay with others, and how you make sense of all of these things.

Acquired vision loss, or deafblindness does not change your person, but the person you are will determine how you deal with it. The brain has a remarkable capacity to adapt and so do people.

What does tunnel vision look like?

I have lost the sides, top and bottom of my vision to retinitis pigmentosa. If I look straight ahead I cannot see the floor or what is immediately above, or to my right or left. It is a bit like looking through a letterbox. Tunnel vision is a misnomer for what I see. There are no big black ugly sides like toilet roll tubes. Instead I have funnel vision, perhaps in time my funnel will change to a tunnel, but right now it’s like watching the world through a video camera lens – things that are far away look normal but close up things get cropped. I can see the whole of a scenic view but only a bit of my computer keyboard.

Diagram of a head showing peripheral and central fields of vision
portcapacity.portofmelbourne.com

By scanning a little, my eyes see the rest of the keyboard, and my brain puts it together so I feel that I can see the whole thing. It is a clever trick like stitching together a panoramic photo. I have to really stop to think about how I see. It is not always a conscious thing. My mind just does it automatically.

The problem is if I don’t scan, I won’t see things that are close. There is a big margin for error. I can see the tiniest bit of fluff but will fall over a chair getting to it. When I am moving around I have to consciously tell myself to look – because the moment I don’t, is the moment I will end up making a wally of myself.

RP eyes are constantly putting together a puzzle, and over time the jigsaw pieces will get smaller. In very severe tunnel vision there may not be any overlap between what each eye sees resulting in two separate jigsaw pieces. In the early stages, while the pieces are still big  – you don’t really notice the scanning – but as they shrink in size it starts to overwhelm the brain. That is probably when it is time to starting using a cane.

Every Usher person has a different visual field, some will have islands of vision in their far peripheral field and will notice someone waving if they hit the sweet spot.  Some will bump their heads on everything but still see the ground. Others may notice overhanging branches but have poor lower vision, making it hard to see signing hands, or to move about. This does not mean that one has person worse damage than another, it is just that we use different parts of the visual field for different things.

Some people with Usher have poor balance due to differences in how their ear canal works, others do not, but since balance relies on a triptych of signals from the eye, ear and muscles (particularly in the ankles and feet), worsening sight can make stability more difficult. Some people report feeling momentarily unsteady even when standing still. They may get dizziness from quick changes in their eye or head movements, and lose track of direction. As vision narrows there may be a sensation of camera shake or juddery vision. I have occasionally experienced this standing at the top of the stairs.

The brain stabilises the images we see, otherwise quick eye movements would make us dizzy. You can try this for yourself. If you fixate on a point and move your head from side to side, the world will appear to stay still even though your head and eyes are moving. The brain is calibrating the image for you.You can override this effect by gently tapping the side of your eye to make your eyeball move slightly (keep your head still). Your vision will jerk and the world will appear to move. By manually moving your eyeball, you are are causing a disconnect between the brain and the eye, and it does not realise that stabilisation is needed.

I wonder with tunnel vision whether there is sometimes not enough visual feedback from our eye movements reaching the brain, which means our sight cannot stabilise quickly, and it takes a little while longer to feel orientated. The less a person can see, the more prone they may become to instability, or dizziness, so for a short time – until the brain catches up – they struggle to see, like trying to steady a zoomed in camera, only it is not zoomed in just narrowed.

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.

Are you baffled by what an Usher person sees?

Ever been baffled by what an Usher person sees? Or wondered what it would be like to climb inside their eyes through a John Malkovich style portal to see just what they can see? People with Usher are born or become deaf but then develop Retinitis Pigmentosa usually as teenagers or adults – an eye condition which can baffle even those who have it. Most are registered blind by around 40 years old. Yet most people with Usher do not feel blind, they can still see plenty. I am registered blind but my world is not devoid of sight, far from it. In many ways the way I see the world looks just the same as before. It looks normal. It is not a scary or dark place. It shouldn’t be feared. I just see differently. My vision just doesn’t do all the things it should.

Bear with me while I do my best to explain by taking a little detour into the visual world of birds of prey. Random but relevant.

Close up of kestrel eye surrounded by feathers

Kestrels flying so high that they look like a speck in the sky can see a tiny 2mm insect scuttling by your feet. Their colour vision is so sharp that they can see trails of mole urine highlighted in the grass.

The price the kestrel pays for such daytime prowess? Its vision is so poor in low light that it must go home to roost at night.

The huge blinking eyes of an owl allow it to see one hundred times better than us at night. Owls can scan a field in the dark and spot the wriggle of a mouse in the gloom. Impressive, right? The disadvantage is an owl can’t see colour as well as humans can. Their daytime vision is a bit rubbish and much less sharp than ours.

Owl eye

Why am I banging on about this? Well, human eyes are just as remarkable. They may lack the same wow factor but have none of the drawbacks. Human eyes are nifty mix – a little bit of kestrel, a little bit of owl. Humans can see well in the day and in dim light.

Imagine a retina – the middle of your eye is the kestrel bit. It is full of cells which give the sharp colour vision that you use for seeing details. These ‘kestrel cells’, otherwise known as cones, help you read, thread a needle and see people’s faces. They deal with the detail.

The owl bit of the retina is round the edge – where there are lots of cells called rods. Try paying attention to your peripheral vision. You can’t really see much in the way of colour or detail, can you? Things look pretty vague out to the side don’t they? But this vision is still essential.

Even in the day the rod cells tell you where obstacles are. They make you sense things moving quickly towards you and allow you to react even before you know what they are. If you are deaf they alert you to a hand waving for your attention or people in the room.

Rod cells let you swat a fly, catch a ball and let you know where the kerb is as you walk along daydreaming. You don’t have to move your eyes around as you walk because the owl bit round the edge of your retina is constantly scanning the environment for you, even when you are not thinking about it. It will tell you where the kerb is even when you are not looking directly at it.

When you are searching for your keys or sweeping crumbs off the dining table, you use this owl vision to scan before homing in on your target with your sharper kestrel central vision.

You rely on the owl bit of your eyes even more at night. It is this vision that alerts you to the silhouette of tents in a field or the outline of furniture in your bedroom. It let’s you see your sweetheart in candlelight or understand the signs of a deaf friend in the backseat of a dark car. It finds the light switch in the dark.

Every day you use both your kestrel and owl vision, switching between them effortlessly. When you cross the threshold of a pub for Sunday lunch on a sunny day, for a moment it looks really dark inside before your eyes adjust that’s your kestrel taking a break and your owl stepping up to the plate. They are a team – taking in turns to lead and to take a break, depending on the lighting conditions or the task at hand.

So what has all this got to do with Usher? Well for a person with Usher the owl bit of their eyes is refusing to cooperate. It’s got lazy or popped off it’s perch altogether. The owl cells round the edge of their eyes are slowly dying. Their peripheral vision is shrinking.

But the world still looks the same to the person with Usher because the kestrel is still wide awake and doing it’s bit. Usher people often still have sharp, clear central vision. It is the corner of their eye that is missing. This is why the Usher person often does not feel or look blind. In the early days they may not even be aware of their vision problems. They just move their eyes around more to compensate.

In good light the Usher person can see straight ahead but they don’t have owl vision automatically alerting them to sudden movement, like people waving or walking in front of them. But they can still see. It is bizarre. It can be baffling for them and those around them working out why they can see many things perfectly but totally miss others.

Back when I had some peripheral vision, a person in the corner of my eye would wave for my attention and I wouldn’t notice it, and then I would realise that I could actually sense the person stood there but I didn’t register their movements, there simply was no waving hand. The kestrel bit of my eye was working but the owl bit let me down.

The kestrel has to work flat out using only central vision to compensate for the lazy owl. The Usher person has to scan their eyes from side to side and up and down as they walk. Look ahead is there any dog poo or a kerb? Look up any branches? Look left and right for people cutting in front of you. They seem to appear from nowhere as if conjured from thin air. I have to remember what is up ahead, because the nearer it is, the harder it is to see exactly where things are. It is exhausting.

It’s a bit of a myth that Usher vision is like looking down a long tunnel. It isn’t. At least it isn’t for me, yet. It is more like a funnel. My vision starts narrow and opens out. I can see things in the distance just as well as you can, the world looks just the same as it always did, but if you are standing right next to me I can only see your mouth moving. No eyes, no hands just your mouth. I have to move my eyes around your face to take it all in and piece together your expression and the ‘mood’ of what you are saying. Just take one step back and I can see your whole face. Another three steps and I can see your hands as well. Ten steps and I might even be able to see your feet.

While playing badminton, I can see a perfect shuttlecock hurtling towards me through the air but around 6 ft away it just disappears, and more often than not ends up on the floor!  I can see a whole crowd of people ahead but when I am among them, I can’t keep track of who is where and end up clumsily bumping into folk. I’m only blind to things when they are very near.

This is why I can see things at the back of the fridge perfectly but completely miss those at the front. That is why Usher people knock over drinks placed within arms reach. They just don’t see the bit of the table nearest them.

Without owl vision, I am useless at finding things dropped on the floor. I can only scan with the middle part of my vision and it takes an age. It can be quicker to use my feet to search, or to get down to floor level and scan the horizon of the floor for something sticking up.

My good kestrel vision alone doesn’t help me see bikes zoom past, or notice steps, or avoid banging my head on cupboard doors. It doesn’t help me to find the dratted cursor blinking on my computer screen, or to check the table for crumbs. I have to step back and then I will notice the bit of dirt I have missed. It’s pretty irritating.

There is no owl to take over when I enter a dim pub so my eyes are incredibly slow to adjust, in fact they don’t really adjust properly at all and I often end up playing skittles with the bar stools.

At night things get tougher. I am dependent on a tiny circle of useless kestrel vision which was never designed for seeing in the dark. By scanning, I can see by piecing things together. This is just about good enough to navigate through a familiar well lit street if no one is around. In fact to my brain the street scene looks pretty darned normal. The kestrel sees enough to put the scene together. I do not feel blind. But as soon as people are milling around, pets, children, cars, bikes or those blasted trolley suitcases then I am scanning frantically to avoid collision and embarrassment. My brain can’t fill in the missing pieces fast enough and I become disoriented. That’s when it hits home how little I can really see – even though I still feel like I can see – which to be honest baffles even me.

Of course no two people with Usher or RP are the same. We all have different vision loss and are at different stages. Some will have very different vision to me, but I hope that this post reveals some of the mysteries of our vision and makes people understand that what we see is not frightening or desolate. It’s just a bit like doing a jigsaw with your eyes.

Watching and waiting

I’ve always been intellectually curious about people who experience the world a bit differently. I want to know how things looks and feel to them – how their perception may differ from everyone else’s in ways that are almost impossible to explain. Now I find myself explaining my own perception to others.

I have spent the last 20 years watching myself slowly lose my sight. I was diagnosed with retinitis pigmentosa (RP) at age 19 having been deaf all my life, but then I had close to a decade of completely normal sight until the earliest symptoms started to appear. Even then it was not clear, was this RP or was it me imagining it? It is a very hard condition to get your head around, even if you are the one experiencing it. RP creeps up on you, like a thief in the night. It is not immediately obvious that anything is gone.

After years of watching and waiting, my vision nose-dived in my mid-thirties and it is still in free fall. Hopefully the deterioration will level off soon. My peripheral vision has disappeared and it is much harder to see at night. Over time I will lose more sight and nobody can tell me how much or when.

I had a really good innings as a fully sighted person. For many people with RP, their vision loss is already advanced when they are diagnosed. I am thankful for those extra years of scrutinising my vision, as it went from normal to impaired, as it means that I can try to explain the changes to others. I will try to put in this blog the things that I wish I had known in those years of wondering what lay ahead.

Back then knowing that one day I would go blind didn’t feel real. How could my vision that felt so solid and reliable just peel away bit by bit? What would vision loss look like? The web was rudimentary then but even as it evolved and I regularly scanned the net looking for answers I couldn’t find anything that satisfied my need to know what to expect.

Simulation picture of RP

The only pictures of what tunnel vision would look like came from simulation pictures like this one. The idea of a black frame gradually encroaching my sight until it was all dark – terrified me. This people – believe me – is absolutely nothing like it looks. These black ink blot simulations are very misleading but it is incredibly hard to accurately simulate what people with RP see. This blog is my best attempt to describe what going blind from this condition actually looks like.

Observations on gradually losing sight – What do the visual changes in Usher syndrome or Retinitis Pigmentosa actually look like?