At the age of 11, Jeannette Shannon was stricken with polio. Her toes curled until they touched her heels, and her other limbs contorted in similar pretzel-like fashion. Most of the girl's body was paralyzed, and she was encased in an iron lung.
But Ms. Shannon's symptoms abated, earning her a place among a group known as the "lucky polios."
"I made a complete recovery and I danced through the next 35 years of my life," said the Mississauga woman.
Ms. Shannon operated a sporting-goods business and raised three children, leading a highly active life. But in the early 1980s, she started to suffer aches and pains, and cramping that was eerily similar to her childhood experience. Over the years, the symptoms have grown progressively worse.
"I went from being a very active woman to being in a wheelchair, and it's all because of polio," she said.
After numerous misdiagnoses, including rheumatism and arthritis, Ms. Shannon learned she was suffering from postpolio syndrome. The luck of the "lucky polios" has run out.
Forty years after the dreadful disease was defeated in the Western world, it is rearing its ugly head anew in the form of a degenerative neuromuscular condition that strikes decades after the initial bout. According to a new report, PPS has already affected 12,000 people in Canada and another 250,000 in the United States and it threatens up to 20 million people worldwide in the coming years.
"The causes of PPS are not thoroughly understood," said Lewis Rowland, a professor of neurology at Columbia University in New York and chairman of the March of Dimes steering committee on postpolio syndrome.
During the initial bout of polio, some of the nerve cells of the spinal cord that control muscles (called motor neurons) are damaged or destroyed.
Dr. Rowland said patients can lose 60 to 70 per cent of their motor neurons, but the surviving nerve cells find muscle fibres that still work and attach to them, restoring muscle function.
"After 15 to 40 years, the ability to maintain function seems to be lost, but no one is certain why this happens," Dr. Rowland said.
The most likely hypothesis is that the surviving motor neurons wear out prematurely from having to do much more work than Mother Nature intended. Aging also plays a role, because the number of motor neurons in the spinal cord decreases after the age of 60, even in those who have never had polio.
To date, PPS seems to have affected fewer than half of polio survivors, but some experts predict it will inevitably strike all of them.
Dr. Rowland said it is difficult to judge the real breadth of the problem because too few health professionals are aware of the condition, and many cases are likely not diagnosed.
To counter the problem, the March of Dimes released three new publications yesterday: a set of clinical guidelines to help physicians identify and treat PPS, a booklet to help former polio sufferers identify the symptoms of PPS, and a report looking at the growing problem of PPS in developed countries.
The information is available from the Ontario March of Dimes or on-line at http://www.dimes.on.ca or polio@dimes.on.ca
There are about 40,000 polio survivors in Canada. They include some of the country's most recognizable public figures: singer-songwriter Joni Mitchell, actor Donald Sutherland and Finance Minister Paul Martin (whose father, Paul Martin Sr., lost the sight in one eye and the use of one arm to polio).
There are, however, only four postpolio clinics in Canada -- in Vancouver, Edmonton, Toronto and Montreal.
Ken Pearn, a Winnipeg building contractor, said he went to half a dozen general practitioners and specialists before finding a doctor who recognized his illness as postpolio syndrome.
"A lot of people aren't getting assessed properly because they don't live in the big cities," he said.
Mr. Pearn said many polio survivors also neglect to mention the condition in their medical history because they consider it a long-ago childhood illness. He contracted polio in 1952, aged 9, and spent 14 days in hospital with the left side of his body entirely paralyzed.
Then, about five years ago, he noticed a strange weakness in the left side of his body, and a growing lethargy. Today, Mr. Pearn can barely lift his left arm, and has to pace himself to conserve strength.
Still, he considers himself lucky. "There are people with PPS who, 50 years later, have to start wearing their braces again, or using the iron lung. It leads to some real psychological problems," Mr. Pearn said. (The iron lung, the enduring symbol of polio, was an airtight chamber that pushed the chest up and down for victims who suffered damage to their lungs.)
There is no cure for PPS, but it can be treated with lifestyle changes to lessen muscle strain.
Polio is a viral infection. There are three distinct strains. Poliovirus enters the body through the mouth, and invades the bloodstream, attacking motor neurons and causing lesions that result in paralysis. Most deaths are due to respiratory paralysis, but the disease can paralyze a single limb, one half of the body, or all four limbs. Most people infected recover, but some sufferers are left with life-long damage, up to and including quadriplegia.
The Salk vaccine was licensed in Canada in 1955, virtually eradicating polio. In 1962, the improved Sabin vaccine was approved, and has been a basic childhood immunization since then. Canada was declared polio-free in 1994.
The World Health Organization predicts that polio will be eliminated from the world by 2005. But postpolio syndrome will linger for many more decades. -*** Beyond polio The World Health Organization estimates that there are 10-20 million polio survivors worldwide and up to 8 million may develop postpolio syndrome
Country Polio survivors Postpolio syndrome United States 750,000 250,000 Germany 100,000 40,000 Japan 90,000 30,000 France 60,000 24,000 Australia 40,000 16,000 Canada 40,000 12,000 United Kingdom 30,000 12,000
Source: World Health Organization
André Picard