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Unrestricted Vision

Editor's Note: Editor's Note: Lisa McGauley holds an MSW, and works at the Fraser Health Authority, Peace Arch Hospital.

It was April of 1961. I was born into a world of boundless potential. The Russians had beaten the Americans in landing the first man on the moon. Martin Luther King was in full swing with the desegregation movement . This was also the year the Berlin Wall was erected, severing east from west Germany.

I was the eldest of four children and an active grade 2'er with a voracious appetite for the written word. My parents describe me as being compelling and driven, with little need to be directed. I was driven to school, picked up and taken home for lunch. One day I ran over to my mom's regular spot and jumped into a green car, not my mother's car. My mother wasn't sitting in the driver's seat. Surely a mistake of childish excitement, but it was repeated over the next few weeks as my mom struggled to understand my behaviour.

A visit to the eye doctor made the family none the wiser as there seemed to be no measurable deficit in my vision. Grade two ended, and we headed off for summer vacation. In the car, I insisted on reading my books very close up, but my parent's insisted that I read them at an appropriate distance.

"Be firm," The eye doctor had encouraged, "as there seems to be no reason for her to read so close." Six hours later , strong-willed and determined, I had had all of my comic books and readers confiscated.

As a rule, I was a compliant, happy child so this dogged determination to read so close puzzled my parents.

The fall of grade three wasn't any better. I had even more trouble with the smaller print textbooks, and struggled excessively to distinguish the yellow chalk on the green board. The year before it had been white chalk on a blackboard, providing greater contrast. Once again, a visit to the eye doctor confirmed no measurable vision change despite the significant change in my visual functioning at school. The only explanation was a "conversion reaction". I was choosing not to see. The diagnostic equipment in 1969 could not measure subtle changes in visual acuity as they can today. Conversion reaction was a large label to put on an active and otherwise healthy eight-year-old. It suggested a loss or alteration in physical functioning from a physiological cause representative of a psychological conflict or need.

I was watched closely by my family and teachers. Reading the small print was definitely a challenge. It was hard. I had been a voracious reader and was frustrated by my inability to consume the amount of written material I desired. Gym class and social playing seemed unaffected.

At nine years old, I sat again in a darkened ophthalmologists office with my dad. I was fighting with a nurse to keep my arm stretched straight in a cold metal sleeve . She wanted to inject dye so the machine could take pictures of the back of my eye as the dye was absorbed. This part of the eye is known as the retina.

"I am sorry. It looks like your daughter has an extremely rare case of juvenile macular degeneration. For some unknown reason, her retina is dying prematurely, and there is no known treatment. I am sorry." He spoke clinically, arousing no concern in me.

The doctor and dad looked at pictures for a while, the doctor pointing out patterns and using a lot of medical jargon. "Nothing too different from other visits," I thought as I patiently sat looking at my lap.

The specialist continued, "In the best case scenario, she will retain her present vision. The disease is progressive, and in the worst case scenario, I would expect her to lose all her central vision. The peripheral vision will not be affected at all."

"And there is absolutely no treatment, nothing we can do?" my father asked.

"I am sorry. There is nothing at all."

Juvenile macular degeneration (also known as Stargardt's disease or fundus flavimaculatus) is the name given to a group of inherited retinal dystrophies that typically involve a loss of central vision over the first few decades of life, although peripheral vision usually remains. Stargardt's usually affects one in every 3000 people. Visual loss in Stargardt's disease usually occurs prior to noticeable clinical changes. Over time, however, physical changes in the retina appear, including degeneration of the central retina or macula.

By my late 30's , my world looked like a shattered mirror. Pieces of fragmented light, images and shadows. Splinters of movement and vision completely dependent on high light levels and contrast. In the dark I could not see anything. Quiet candlelight dinners in restaurants meant feeling for the chair, eating invisible food from an invisible plate and holding conversation with invisible partners. My central vision was a large, growing blind spot with ragged edges. The area was black with thousands of honeycombed, flashing, pinprick lights and floating shadows.

This was the worst case scenario the ophthalmologist had talked about.

Following the birth of my second child , my vision began to deteriorate peripherally, and by the age of 41, it became difficult to ignore the functional changes. My peripheral vision was continuing to deteriorate.

Another visit to the retinal specialist and more tests. This time, there was news, not the familiar "I am sorry, but it is a natural progression of your disease". Instead, it was reported that this degree of vision loss was definitely not juvenile macular degeneration, as thought for the past 30 years.

My diagnosis was changed to retinitis pigmentosa. Again, I was the odd man out. I had an extremely rare case of RP, where the macula dyes first, and then islands of blindness form in the periphery, grow, and eventually join the central blind spot, creating a black continent. As before, this disease was progressive and with no treatment. The prognosis, however, was much worse. I would go completely blind.

RP, a group of inherited retinal conditions characterized by degeneration, typically begins with peripheral vision loss, and gradually encroaches on central vision over decades. RP is estimated to affect at least one in every 3000 people, and is caused by mutations in any of a number of genes. Generally, RP is considered to be a "rod-cone" degeneration, where the rods begin to die off first, followed by the cones.

"Researchers are always investigating a variety of possibilities to restore vision in persons with RP and Stargardt's, including gene therapy, artificial retinas, computer chip implants and specialized low vision devices."

So Macular Degeneration or RP? Perhaps One is superimposed on the other. The reality is I am going blind. Does the name really matter anymore?

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