About Claude St-Jean:

Courtesy of the Globe & Mail 2006:

Diagnosed at 15 with Friedreich's ataxia, he raised funds and relentlessly encouraged research into a deadly disease that claims one in 20,000 Quebecers.

He was the driving force behind a campaign to find a cure for a fatal disease of the nervous system called Friedreich's ataxia. A teenager when his own symptoms first developed. Claude St-Jean once confided to his diary "I feel like a dog whose chain is gradually being shortened." He was 15-year-old when his life was suddenly and irreversibly changed. Yet, for all the relentless progress of the disease, he never accepted his predicament.

"Logic tells me I won't make it," he once wrote. "I know it has accelerated in the last two years. But I will at least have the satisfaction of knowing that I have tried everything, of having helped others, of having fought to the end. And who knows? Perhaps even winning."

When he was first diagnosed with ataxia, there was little or no research being conducted into the nature of the illness, which causes slow, unrelenting damage to the nervous system. First described in 1860 by German doctor Nicholas Friedreich, symptoms included coordination problems, such as clumsiness, awkwardness, slurred speech, and frequent falling and unsteady movement.

There is no effective treatment to the disease, which is inherited by about on child in 25,000 and begins to affect the heart and nerves around puberty. Sufferers eventually lose the ability to control even simple gestures, and can develop diabetes and foot deformities.

Typically, Claude St-Jean enjoyed a totally normal childhood. "I was happy, carefree, enterprising, jovial and looking forward to a promising future," he wrote. "One evening, on my way home form my student job, I suddenly experienced a loss of equilibrium, causing me to stumble and walk unsteadily. Passerby, believing me to be drunk or stoned, began casting insults at me."

Humiliated, Mr. St-Jean first attributed the attack to fatigue. But when the same thing happened a few days later, his parents took him to meet a top neurologist specialist who, after subjecting the teenager to a battery to tests, diagnosed Friedreich's ataxia.

"You would think that I might have drawn some reassurance from knowing exactly what the problem was, " Mr. St-Jean recalled. "But the doctor informed me that it was a serious, progressive, genetic and hereditary disease for which there was no cure."

His symptoms, he was told, would worsen and result in an increasing paralysis. His doctor told him that he would soon be confined to a wheelchair. If he was lucky, he might live another 12 years before an infection or hearth attack killed him. "My physician compared it to playing the lottery, where the chances of winning are slim. In a way, I guess I won the jackpot!"

His reaction was to explode in anger. Young Claude returned home and promptly smashed a large aquarium onto the floor. Unable to accept a prolonged fate. He seized a knife from the kitchen and locked himself in the bathroom. Police were summoned, and eventually he calmed down and was taken to hospital. While under observation, he established a meaningful relationship with a psychiatrist named Marie Caron. They later became friends.

Claude St-Jean never wanted for pity, Dr. Caron said. "What he was looking for was answers. So I put him in touch with a neurologist named Dr. André Barbeau, who was renowned for his work on Parkinson's disease."

Mr. St-Jean repeatedly called on Dr. Barbeau to convince the neurologist that he should, instead concentrate his research on ataxia. At first, Dr. Barbeau quickly tired of the young man's repeated and time-consuming visits on what was a seemingly hopeless mission. Dr. Barbeau, who died in 1986 at 54, responded that it would be unrealistic to throw himself into costly research into an uncommon disease. His young patient refused to take no for an answer.

Impressed by his persistence, Dr. Barbeau finally offered the teenager, a sliver of hope. He told that if he really wanted to fight ataxia, he had to do it the right way and recommended setting up an organization that could raise the necessary money for the research.

And so was born the Association Canadienne de l'Ataxie de Friedreich (ACAF). On March 18, 1973, the 250 people at ACAF's inaugural meeting elected Claude St-Jean president, and his father Léonard, as associate-secretary.

Mr. St-Jean, who was just 21, threw himself into his work. By October, he had raised enough money to put together a team of specialist who, led by Dr. Barbeau, began the serious work of investigating the origins of Friedreich's ataxia.

Engaged in a media-awareness campaign ever since, the ACAF remains at the forefront of ataxia research. Dr. Massino Pandofo, who in 1996 was credited in identifying the Friedreich's ataxia gene, says it was Mr. St-Jean's single-minded determination that had fuelled the groundbreaking discovery.

"Thanks to his effort, since the 1970, many fine researchers from all over the world became interested in the disease," says Dr. Pandofo, who at the time was professor of neurosurgery at McGill University. "I directly witnessed Claude St-Jean's constant activity in supporting FA research and awareness, despite the advanced stage of his disease.
In a subsequent finding, Dr. Pandolfo and his team went even further and pintpointed how iron accumulates in cell that lack the frataxin gene. In a healthy cell, the gene produces a protein that regulates iron, which the cell uses to make energy out of fuel and oxygen. If the gene of the chemical react with oxygen and put stress on the cell. Eventually it dies.

During his lifetime, Mr. St-Jean raised about $6 Million for ataxia research. Not only was he a superb fundraiser, but he also played the part of the selfless test-patient, submitting himself to endless physical examinations and to new and often unproven medicines and procedures.

Mr. St-Jean refused to give-up, Dr. Caron says. Despite acute physical pain, he never complained and instead devoted himself tirelessly to his dream of an ataxia cure. He always believed that despair, and the depression and inactivity that often go hand-in-hand with it, too often shortened the lifespan of Friedreich's ataxia patients. Over the years, he went out of his way - once crisscrossing Canada and the United States in a specially equipped van - to spread a message of hope among fellow sufferers. "I tell these people, listen, you have the disease but you can live an almost-normal life. I tell them to continue their studies, to keep active. It is certain that one who keeps active will live longer."

Perhaps as an example, perhaps because of his inner resolve, he kept the disease at bay and added 27 years to his initial 12-years death sentence.

Claude St-Jean was born in Montreal on March 27, 1952. He died in Montreal on June 13, 2006. He was 54. His parents and sisters, Sylvia, and Nicole, who continues her brother’s work at ACAF, survive him. He was predeceased by his brother Luc, and sister Francine, who both died form ataxia in their thirties.

Order of Canada Claude St-Jean, C.M.

Member of the Order of Canada

Awarded on July 11, 1977; Invested on October 26, 1977

Founding president of the Canadian Association of Friedreich's Ataxia. By dint of his outstanding courage, tenacity and vitality, he has encouraged medical science to make significant progress in finding the solution to this disease, not to mention the support and hope he has restored to its victims.

Above from: http://www.gg.ca/honour.aspx?id=1617&t=12&ln=St-Jean


Thank you Claude!