By JILL MAHONEY

From Friday's Globe and Mail

Friday, August 9 Online Edition, Posted at 2:03 AM EST
Fears that the human form of mad-cow disease would strike North America have been realized with the death of a Saskatchewan man from the incurable brain-wasting illness. The continent's first confirmed case of variant Creutzfeld-Jakob disease alarmed dozens of people who may have been infected through a medical instrument used on the man, while unsettling untold numbers of Canadians who lived recently in Britain, where the ailment originated.
After the cause of the man's death was confirmed as vCJD on Tuesday, health officials telephoned 71 people who underwent endoscopies with the same tool used on the victim at Saskatoon's St. Paul's Hospital. Endoscopies are performed with long scopes and are used to diagnose internal ailments. "The risk of these people having that disease are so minute," said Dr. Antonio Giulivi, who is in charge of monitoring CJD at Health Canada. "This was a precautionary call, only a precautionary call." However, the assurances have done little to calm the fears of some of the patients who had endoscopies in the eight-week period between the man's procedure and when his doctors stopped using the instrument in April because they suspected vCJD. "It was very upsetting, because suddenly, in one phone call, somebody's telling me you could be tainted with mad-cow disease. As little as I know about mad-cow disease, I know that it's an awful thing," said Jo-Anne Engele, a 41-year-old Saskatoon teacher. The endoscope used was cleaned, but could not be sterilized because the heat would melt the rubberized instrument. However, if prions — the deadly agents behind CJD — contaminated the endoscope, the cleansing process would not likely have been effective. Still, scientists have not definitively established whether the neurological disease, which has a long incubation period and is spread by eating infected processed meat, is also spread by blood. As well, there is no known case of transmission through contaminated medical instruments. However, as a precaution, health officials told the 71 people not to donate blood, tissue or organs. Dr. Steven Whitehead, deputy medical health officer for the Saskatoon area, said officials agonized over whether to notify the patients. "We have done this after a lot of deliberation because we believe that they have the right to know, to have this information," he said. Citing the wishes of the man's family, officials would say only that he died earlier this summer, was under age 50 and ate processed meat while visiting and living in Britain during the 1980s and 1990s. The cause of his death was definitively confirmed Tuesday after intensive tests of his brain tissue in Toronto and by a British expert. Like the other three strains of CJD, variant CJD can be diagnosed only with a brain biopsy or autopsy, meaning a definitive finding is most likely after death. In April, U.S. health experts reported the first suspected case of vCJD in the United States in a 22-year-old British woman living in Florida. They said the woman, who is alive, most likely contracted the disease while living in Britain. Public health officials said Canadian cattle are not suspect because it is likely the Saskatchewan man contracted vCJD in Britain after eating contaminated beef products there. "There is no risk to the general public from this case. The infection is not passed from person to person and as far as we can tell it was acquired in the United Kingdom. There's no reason to believe that it's connected to Canadian livestock or to the Canadian food supply," said Dr. Ross Findlater, Saskatchewan's chief medical officer of health. Canadian cattle have never been found to have mad-cow disease, known as bovine spongiform encephalopathy. However, in 1993, an infected British cow was imported to this country and was destroyed. BSE, which spreads when cows eat feed made from the ground parts of infected animals, was first diagnosed in Britain in 1986 and has affected nearly 200,000 cattle there. There have also been cases of infected cattle in nearly 20 other European countries. The epidemic among British cattle peaked in early 1993, when about 1,000 new cases were reported weekly, and has not been eradicated. The human variant of the degenerative neurological disease claimed its first confirmed victim in Britain in 1996: a 20-year-old vegetarian who ate beef burgers as a child. In all, there have been about 125 confirmed cases of variant CJD in humans, the vast majority of them in Britain. Windsor's Hotel-Dieu Hospital had a similar scare over a year ago, after a woman underwent brain surgery and was later suspected of having a possible case of classic Creutzfeld-Jakob disease. The hospital's trauma centre closed its operating rooms for a month, and officials tried to follow Health Canada's guidelines to sterilize their instruments — about 400 sets of 70 pieces each. However, the chemicals ruined the tools. With a report from Canadian Press
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