RE-VISIONING THE FUTURE

It’s been an interesting time for THE HOPE LADY, improbable brushes wit the unexpected, the impossible, etc. Many of you have been asking me questions, having read my posting of October 30. So here’s a little more of the story. But I warn you, it does go on a bit.
A couple of weeks ago I happened to come across a little article in the newspaper. This alone smacks of science fiction. I never quite get over the wonder of reading the newspaper. Once upon a time I was a child, playing on the living room floor, getting no attention from my family. They were in their own worlds, having divided the newspaper into sections, one section for each of them. At that time I never imagined that I would some day read the newspaper on a talking computer—I had never imagined a computer. But these days I take the computer for granted, only occasionally pausing to marvel at it, usually when I read the newspaper.
Anyway, I happened to be reading the newspaper when I came across a little article about the disease that caused my blindness
GENE THERAPY RESTORES VISION IN LEBER CONGENITAL AMAROSIS
I read the article a few times, half suspecting it would disappear. Sometimes things you thought you found on the computer do disappear. But this one didn’t. So I did a little more investigating on the Internet—have I mentioned how amazing it is just to search for things on the Internet—and that led me to a website for the University of Pennsylvania, a university that already stood high in my affections seeing as how it is a very prominent site for research in positive psychology.
I wrote to a doctor there who referred me to Toronto, and eventually I was referred to Dr. Ian MacDonald here in Edmonton. He was just getting ready to chair a research conference in Edmonton sponsored by the Foundation Fighting Blindness. Last Saturday my sister and I attended that conference.
At this point I really ought to introduce my sister Donna. We’ve been in this together since my beginning. She was in it first. Our parents were teen-agers when they got married. They were young, but they were anxious to settle down to a quiet life on the farm. Within the year they had a daughter, and soon they noticed her darting eyes. The first doctor they saw said she was totally blind. It was not true, a fact which soon became apparent to them. They were relieved, since another baby was already on the way. That one had normal eyes.
Donna’s vision was a bit of a puzzle. She could see objects, yet she walked right into an open trap door in the neighbour’s kitchen. There’s a scar on her chin to prove it. Along with the normal activities of farming and child raising, our parents busied themselves with everything that made sense to them. They went to doctors and faith healers. They read magazines and made inquiries about articles that relatives sent to them. One such article led them to New York, where Donna got some enormous lenses that enabled her to read print up close. In this way she devoured the World Book Encyclopaedia and the newspaper—an activity that blackened the tip of her nose.
I came along a little before her 8th birthday. There again were those darting eyes. Logically, they embarked on my raising with the sensible theory that she and I would be the same. We weren’t. My vision just wasn’t as good, though I did love the sun—a peculiarity since the sun all but blinded her. My love of the sun led Mom to believe that I would need a strong light for reading. They got me some of the New York glasses and Mom sat me down in a sunny window. She drew huge numbers in different colours. I stared at them in the sunlight and thus learned two things at once—my numbers and my colours. This was a bit of a victory. Donna couldn’t do colours.
School just didn’t work out for me the way it did for Donna, though it wasn’t for lack of trying. The janitor adapted a school desk with a large board and they clamped a lamp to the board. Then they placed my desk near a plug-in, far from all other desks. As a child one of my most fervent wishes was to sit close enough to somebody else so that I could get in trouble for whispering. They bought scribblers with huge black lines. They gave me my own black (really black) blackboard for my desktop and they drew things on it in bright white chalk, because it was easier for me to do math that way. At some early point in all this, my sister Sandra, a born teacher, circumvented everything and taught me touch typing, a skill she was learning in Grade 8.
With all this effort being expended on my education, it’s little wonder that people got frustrated. I would not do my schoolwork. Teachers said I was lazy. They said I wasn’t trying. Yet it was confusing, because I was learning the material. I was literate, I could do math. I simply refused to read more than a few words at a time.
Glasses were an issue. My day glasses broke on the playground. Mom was too busy to get them fixed right away. That’s when a canny teacher noticed that my playing vision seemed to be as good without them. That ended the saga of day glasses. My reading glasses prevailed for longer. Without them I could read nothing from a printed page. But my Grade 5 teacher sent them packing also. He sent the lamp away, moved me to a normal desk in a normal row, scolded me for whispering to my neighbours and started a process that got me reading Braille. By the time junior high came along, I was at Jericho Hill School for the Blind in Vancouver, and Donna’s black-tipped nose was tracing the lines of university textbooks. Thus we have lived parallel lives, she as a sort-of-sighted person with really bad vision and me as a blind person with a little bit of totally unreliable vision. I can, for example, see the light through the open train door of the LRT well enough to step through that door—but only sometimes. Other times I don’t see the door at all. Colours were lost somewhere in my forties, not a big loss, except at Christmas. Christmas lights just aren’t much fun these days.
Donna is still a really big reader. “Did you see the article in the paper about gene therapy for our condition?” I asked her a couple of weeks ago. She hadn’t seen it. Score one for me. But when we went to the conference last Saturday, white canes in hands, she put on her reading glasses and read us the agenda. Score one for her.
They believe that 350 Canadians have Leber Congenital Amarosis. At the conference we learned that they have isolated 12 genes for LCA. Somehow these genes mutate and if two people with mutant genes happen to meet up, one out of four of their kids might be expected to have the condition. Our folks beat the odds. They got two out of four. Having identified some genes, the researchers began experimenting on dogs, injecting genes into their eyes. The new genes turned on some photoreceptors that would have been turned on in a normal eye, and the dogs’ vision improved in the tiny spots where they made the injections. Now they are experimenting on people, people who have LCA2, one of the twelve genes. The results are very promising.
So now Donna and I are at the beginning of a new process. We have to be examined and re-diagnosed. Then our genes will have to be identified. Our names will be added to a registry, the FFB/CIBC registry. That will make it possible for us to be contacted if something comes up that can help us. Things are moving quite quickly, and though Donna and I may well be too old to benefit, our mother’s cousin’s child might benefit, being somewhat younger.
In the meantime I’ve been dreaming about what I would do with a little bit of extra vision. I could appreciate a few Christmas lights. I could step gracefully through a few more train doors. But even if nothing else comes of it, I’ll still have the thrill of being the only person I’ve met so far who noticed that little article in the newspaper.