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Hope for Future Cancer Patients

I was born in 1952 and was a healthy baby. When I was about six months old, however, my mother began to notice I was having difficulty focusing when looking at something, and before long that I could no longer see her when she stood in my bedroom doorway. Although our family physician in the small northern Ontario town where we lived did not think that anything was amiss, he eventually referred us to a specialist in Kirkland Lake, the city closest to our home. It was there that my mother learned I had bilateral retinoblastoma, a rare form of cancer of the eyes that, if not treated, would be fatal. My parents had never heard of it before.

Retinoblastoma (RB) occurs in children from the time they are in the womb up to the age of five years, and accounts for about three percent of pediatric cancers in those under the age of 15. Approximately 60% of cases are unilateral (affecting one eye), with the remaining 40% being bilateral (affecting both eyes). While RB is curable if caught early, 87% of affected children worldwide die, mostly in developing countries. In developed countries, 97% of those who do live have moderate to severe vision impairment.

In the 1950s, when I was diagnosed with RB, there were very few treatment options. Radiation therapy was available in New York, but my mother was told that it created a great deal of facial scarring, and often didn’t work. The second option was radical surgery to the affected eyes, which could result in blindness. My ophthalmologist at Toronto’s Sick Children’s Hospital, where I was taken in the winter of 1955, felt my condition had progressed beyond the point where radiation would help, and so in order to save my life my mother signed a consent form to have both of my eyes removed and replaced with prosthetic ones. About two months before my third birthday, I entered the hospital with enough vision to remember seeing my mother standing just outside my room talking to a nurse. When I left the hospital, I saw nothing, and all I really remember is a feeling of extreme confusion at people being able to see me but I couldn’t see them.

But I adapted to my situation, and for the most part I had a happy childhood. I was a curious and adventurous child, and I taught myself to do just about everything my sighted friends did. I grew up, obtained a university degree, and after a few other jobs began working at Statistics Canada.

As far back as I can remember, I wanted to have children. For me, becoming a mother was more important than anything else at that time. When I learned at the age of 23 that the retinoblastoma that had caused my blindness was 50% hereditary, I was devastated. Between the ages of 23 and 30, I struggled with the numerous ethical issues this raised. Friends and family regularly voiced their opinions about people with hereditary conditions starting families, with one individual even saying that it would be “socially irresponsible” for me to have children. At the age of 30, however, I was married, and my husband and I decided to start a family.

Unlike my parents before me, and thanks to modern advances in genetic research and treatment options, my husband and I were able to inform ourselves about retinoblastoma before we had children. I contacted the National Cancer Institute of Canada during my first pregnancy and was provided with a great deal of literature on this particular form of pediatric eye cancer, as well as the name of a doctor at Sick Children’s Hospital who specialized in this condition. Under her guidance, every possible step was taken to ensure that, if the condition existed in any of our children, measures to arrest the progress of the disease would be taken as soon as possible.

We had two children--Philip in 1984 and Alena in 1987. Philip was monitored regularly from birth until he was two years old to make sure he was clear, and a later blood test showed that he does not carry the RB gene. Alena, however, was found to have two tiny tumours on one eye and three on the other when she was four months old. From then on, we travelled once a month from our home in Ottawa to Toronto, then every two months, every three months, etc. Lazar and cryo (freezing) therapies--treatments that were not available during the 1950s--were used to arrest the growth of the tumours. By the time Alena was two years old, the tumours had faded into scars that seemed to have had no effect on her vision. In April of 1990, Alena’s doctor said, “Yes, I would say this child is cured.” We then visited the hospital every year till she was 14 and every two years till she was 18. Since the scars have remained quiet and she has passed the age when any retinal tumours might occur, Alena can now make annual visits to a local retinoblastoma specialist.

Thanks to the great strides in cancer research, my daughter has full vision in both eyes and is a healthy young woman. We are fortunate in Canada to have access to world leaders in the diagnosis, treatment and genetic analysis of retinoblastoma. Consequently, the survival rate in Canada is now about 96%.

For further information, visit the Canadian Retinoblastoma Society’s website at http://www.rbsociety.ca or Retinoblastoma International at http://www.retinoblastoma.net.

Adapted from the March 2007 edition of @StatCan, the newsletter of Statistics Canada.