Jaguar in the garden

Self-driving cars, bionic retinas, sight-restoring stem cells, gene therapy, talking phones. If there's ever a time to go blind, this seems to be it. There is so much hope on the horizon that it's tempting to put my head in the sand for a while and wait until science waves its magic wand. Or at the very least, drives me around in a snazzy car while I sit back, relax and enjoy the view with my Google Glasses...

However, when I saw a new consultant in San Francisco on Friday, she said it was very much time to be 'prepared'. My good left eye is going to go the same way as my right eye very soon. Apparently, Stargardt's disease is nothing if not symmetrical and my days of relying on my left eye are numbered.

So, alarm bells ringing and with a new sense of purpose, Steve, the girls and I went to a low vision conference on Saturday. We thought we'd save time by stopping by the venue so that I could register on our way to a friend of Sophie's birthday party. Bad plan. In our rush to get out of the house, we didn’t think through how Sophie would fare beyond keeping her at a safe distance from all the dogs, canes and the whizzy computer screens on display.

Predictably, Sophie’s eyes widened in terror when she saw the guide dogs (we're hoping her aversion to dogs is just a phase) and it was no surprise that she wanted to know what the canes were all about. But the look of panic when we reminded her that my eyes are bad stopped me in my tracks.

Since I was diagnosed with Stargardt's  a couple of years ago, I've spent hours agonizing over how different things will be for the girls growing up. I feel so sad that I won't be able to  read stories to them or see them smile, or help with homework, or catch a ball, do jigsaws, or help choose a prom dress if we're still in the US by then (and if they'd let me anyway!). But on Saturday, I suddenly realised that all of these things only matter if we let them matter. Now, more than ever before, there must be plenty of ways around them. Probably even an app or two. All Sophie and Alice need to know is that I'm fine, and as long as that's the case, then the rest will into place.

And it's also good for me to remember that while I may be dragging my heels about adapting to the situation, Sophie is far quicker off the mark. Not every toddler has to tell her mother what she’s looking at (apparently, we had a jaguar in the garden the other day), what she’s doing or how she’s feeling, but she’s taking it all in her stride. She’ll  even describe to me exactly what she's doing when she's being really naughty. For now, at least!

Hat shop

Sprinting to catch a flight at Vancouver airport on Friday, I asked a security guard where to go. He told me to read the sign right in front of me. I retorted that I'm visually-impaired.  Confused, he pointed at the sign again. With just two minutes to go until my flight was due to leave, I gave up and raced off in what looked like the right direction leaving him muttering about people 'trying it on' to get priority boarding. I'll bear that in mind for next time...

Then there was the very nice lady dressed in a bright red suit who greeted me in the lobby of my opthamologist's office last time I visited. So nice in fact, that she took my hand, led me to the elevator and rode with me to my floor. Still holding my hand (which was becoming more and more awkward by the minute), she told me to check-in for my appointment using the touchscreen computers which had apparently replaced the usual front desk staff. I explained that I might need some help navigating my way around the screen and she looked entirely bewildered. She made a hasty retreat and whispered to a colleague that I appeared to be 'visually challenged'. I couldn't help but wonder how on earth she'd managed to avoid dealing with 'visually challenged' people in a retinal disease clinic. And if she wasn't there to help patients like me, what on earth was the hand-holding all about?!

All very odd, but then I suppose that this transitional stage between being sighted and not is confusing - for me and everyone else. How can somebody who can run at full pelt through an airport be unable to follow directions? How can somebody read texts on her phone (in an attempt to forget about holding hands with a complete stranger) struggle to use a touchscreen computer? Why would she bother writing a shopping list when she complains about no longer being able to read her own hand-writing? Why does she insist on buying magazines she can barely see?

Recently, I've been experimenting with different ways of describing what's going on. I've tried on a few different hats for size, starting with the entirely nebulous 'horrible eye-disease' and 'terrible eye-sight' approach, followed by a brief attempt to brave no-nonsense labels such as 'visually-impaired' and 'low-vision' before retreating to the comfortingly meaningless 'almost legally blind'.

At some point, the blindness might speak for itself but now and in future, I'm so happy to have friends and family who don't seem to mind the ambiguity of it one bit: People who'll listen to me moaning about not being able to see ANYTHING. AT ALL. ANYMORE one day and still be happy to take me to see a ballet or an art exhibition the next.

Shifting sands

While the end destination is fixed, I have discovered that the journey towards blindness is haphazard and unpredictable. In my case, my sight is in the hands of the millions of tiny photoreceptor cells responsible for transmitting visual stimuli to the brain.

Stargardt's disease  creates a hostile environment for photoreceptor cells which gradually degenerate and die. If enough of them die at any one time, then I'm left with another  blank spot in my vision. And if the cells happen to be near the fovea, the sacred cow of sight, then the vision loss I experience is more pronounced and more disruptive. 

I don't usually think too much about the physiology of it all but I suppose it explains why I can see something perfectly well one day and then find myself frantically cleaning my glasses in an attempt to see it the next. 

Sometimes, the degradation is so subtle that I can blame it on tiredness (not unusual with a newborn baby) or hornones (ditto) or bad lighting. Other times, it's scarily obvious that something big just happened. Then it's time to begin adapting to the new status quo all over again. 

On bad days, it can feel like a game of musical chairs - getting up and starting again in the knowledge that there'll be one fewer chair to sit down on next time the music stops.

On better days, I know that I'm becoming far more proficient at doing some quick mental triage to work out what's changed, how it will affect me, and how I need to deal with it. I've learnt that it's okay to persevere with some things until the bitter end, that others are easily fixed with some resourcefulness, help from family and friends, or a piece of adaptive kit. And then there are other times when it's just fine to gracefully admit defeat... I never liked doing my tax return anyway.

Brain training

Last weekend, I tripped and fell quite spectacularly during a leisurely jog around the block. Five stitches and a cracked rib later, one of the nurses at the hospital asked if I was training for something. I haven't signed up for a marathon but I suppose I am doing training of sorts - training my brain to rely less heavily on visual information and to 'feel' my way around. 

Sensory compensation is a well-known phenomenon amongst the congenitally blind but what does it mean for beginners like me? My feet and my brain clearly weren't in the mood to connect last Saturday, but is it possible to experience and process the world using different senses?  Can I really rewire my brain? 

Until recently, I have been able to plug the gaps in my vision with lots of useful visual memory. Give me a visual clue and I'll fill in the rest. Lately, however, the gaps have become larger and the guessing game more challenging.

It's not quite time to learn how to use a white cane or to switch to screen reading software, but it's definitely time to find ways to prepare for my future while making life a little easier in the meantime.

Here's what I've learnt so far: 

...that it's possible to hear when a person is smiling, frowning or raising an eyebrow even when you can't see their face 

...that being organized and ordered is really important. If I don't put my keys, phone, wallet etc. down in the same place each time, then it's unlikely that I'll find them again

... that toddlers and tidiness don't mix. Said keys, phone and wallet may well end up in the fridge

... that sometimes, the simplest solutions are the best. Putting raised stickers on the dials of all our appliances has saved no end of frustration, shrunken clothes, burnt food...

...that the right lighting makes all the difference. And it can become quite an obsession as I turn lights on or off or angle them this way or that simply to chop a carrot...

... that pretending to look at a toddler's pretend spider is okay, but that pretending to read the words of a story won't be okay for much longer...

... that while I may feel stupid asking for help, most people are amazingly willing to read the things I can't see

... that it's actually quite interesting to 'see' the world through other people's eyes

...that none of these things truly compensate for not being able to see, but they're a jolly good start.

'Blinking on the Brink' is the story about losing my sight with Stargardt's disease. It's not meant to be a miserable read, simply an account of the day to day trials and tribulations as I make my way towards a new kind of normal.

A bit about me: I live in  California with my husband, Steve and our two daughters, Sophie (two) and Alice (two months). We moved out here a couple of years ago from England and we still can't quite believe our luck that it only rains a few times a year!  Life is pretty hectic with two tiny children at home but in rare moments of quiet, I love art and music. I'm not sure how my art history degree led to a career in market research and branding, but it's been a fascinating decade spent traveling the world while working with some great brands.

I was diagnosed with Stargardt's disease in 2010. I vividly remember struggling to see a painting in a London art gallery and wondering if I had put my contact lenses in the wrong way round by mistake.

It turns out that I was in the early stages of Stargardt's, a genetic retinal disorder which affects the macula, the part of our eyes we use to see detail like print or people's faces.  Stargardt's causes progressive vision loss to the point of legal blindness and beyond. 

The world hasn't gone completely black, rather fuzzy in the middle and a bit weird around the edges. I don't have a cane or a dog just yet. Those will come in time. For now, it's about getting used to seeing without seeing - filling in the gaps, piecing together snippets of vision into something that makes sense.

My days are spent wondering whether that really was a man skating past wearing nothing but his inline skates; whether those really were poodles being pushed along in a double stroller. Only in San Francisco!