TORONTO - Who would have suspected that the federal government is in the mortuary business? Or at least the mortuary transportation business.
The Public Health Agency of Canada is seeking bids from funeral services companies for transporting the bodies of people suspected of having died from Creutzfeldt-Jakob disease, a rare but always fatal affliction that is contagious with certain types of exposure.
In its online tender notice, the agency says the successful bidder would be required to collect bodies from hospitals, long-term care facilities and residences throughout Canada and transport them "by ground, air or rail" to pathologists for brain autopsies to test for Creutzfeldt-Jakob disease, or CJD.
The number of cadavers to be transported varies but could reach 60 or more per year, the notice says. Services would include returning the post-autopsy remains to a funeral services provider "in keeping with the needs of the deceased person's family."
This isn't the first time Health Canada has contracted out transportation services for cadavers with suspected CJD; the program is part of PHAC's Creutzfeldt-Jakob Disease Surveillance System, which has long been tracking the incidence of CJD and other human "prion" diseases in Canada.
Prion diseases, also known as transmissible spongiform encephalopathies, are rare, progressive neurodegenerative disorders that affect both humans and animals. Prions are infectious agents that cause abnormal folding of specific proteins found most abundantly in the brain. These misfolded proteins cause progressive, irreversible brain damage.
In animals, prion diseases include scrapie in goats and sheep, and bovine spongiform encephalopathy — or mad cow disease — in cattle.
Dr. Neil Cashman, a world-renowned expert in prion diseases at the University of British Columbia, said Canada has had two confirmed cases of what's known as variant CJD, the human version of mad cow disease. Both occurred in immigrants to Canada likely exposed to the disease in their home countries, the U.K. and Saudi Arabia, through BSE-contaminated cattle products.
"The bulk of CJD in Canada and around the world is due to other causes," Cashman said from Vancouver. "Most of it is sporadic; it comes out of the blue for reasons we don't understand. Some of it is familial, due to a mutation in the prion protein gene, but it's about 90 per cent sporadic here and everywhere else.
"It's a spontaneously arising infectious disease, so the people who have CJD can transmit it through brain surgery or other very specialized modes of transmission, but they didn't catch it from anyone or anything," he said, explaining that most people who develop symptoms of the disease are in their 60s or 70s.
With classic CJD, such symptoms as depression, abnormal vision and balance problems eventually lead to dementia and an inability to move or speak, followed by death. While CJD can have an unusually long incubation period — even up to 50 years — death typically occurs within one to two years from onset of symptoms.
When CJD is the suspected cause of death, the person's remains are transported to designated pathologists in each province, who perform an autopsy and remove the brain for testing to confirm the diagnosis, said Michael Coulthart, director of the PHAC surveillance program.
Between 1997 and 2012, there were 1,294 suspected cases of CJD in Canada. The tracking system has recorded 589 deaths from confirmed and probable cases of CJD, of which 540 were classified as sporadic.
There are typically 40 to 50 suspected cases of classic CJD a year in Canada, although 60 were reported in 2012, said Coulthart, noting that incidence appears to be gradually rising, possibly in part because of the aging population.
Pathologists must wear protective gear while performing an autopsy — due to exposure to brain and spinal-cord tissue where the infectious prions reside — and embalming isn't recommended because of a small risk of transmission.
However, the risk of contracting CJD by anyone transporting a body "is nil," said Cashman. "It's the same as somebody who has CJD in a hospital. They're not transmitting the disease from bed-to-bed contact. Even intimate contact is not something that transmits disease.
"There is a huge fear out there from funeral directors about doing anything with CJD, but this has no basis in fact for transport," he said.
"And there are lots of cases of funeral directors refusing to accept people who've died of presumed CJD. This is a huge torment to families, so if all the t's are crossed and i's are dotted in a contract to transport this completely safe cadaver, then that will help expedite the system," he said of the PHAC program.
Phil Fredette, a spokesman for the Funeral Service Association of Canada, said he has never heard of a funeral home refusing to handle the care of a deceased person with suspected CJD.
"We typically use universal precautions to begin with when it comes to any decedent, which we should because we don't always know if there's a communicable disease with a certain person or not," Fredette said of staff wearing gloves, gowns and masks.
"Creutzfeldt-Jakob has been around for many, many years," said Fredette, the manager of a Saskatoon funeral home. "We've all been aware of it ... There's no more fear of that than any other communicable disease."
Coulthart agreed that the notion of fear being an impediment "is a bit of an overstatement. I think people are rightfully concerned about handling the remains of anyone who has died of potentially transmissible diseases," he said, adding that the risks of prion diseases are often poorly understood among people who work in mortuary services.
"And a lot of stories seem to be circulated about how strange and dangerous prion infectivity is. In fact, there are some particular features that need to be recognized of the prion, and that is that its ... proteins of infectivity (can be) very resistant to inactivation" by most conventional methods.
"But there's no particular risk recognized for handling an intact body."
Since the surveillance program began in 1998, Toronto-based funeral services provider MacKinnon and Bowes has transported the remains of Canadians who died of suspected CJD — the only company to bid on the contract — which is now up for renewal and posted as part of federal transparency policy.
It may be that the company, which has worked with the military repatriating the remains of fallen soldiers, will again be the only bidder, Coulthart said.
He is not surprised at the paucity of bids, as requirements are stringent: companies must be able to transport cadavers in communities across the country, do so in a timely manner and with staff who can provide services in both English and French.
In the end, the goal of the program is not only to track cases of classic CJD, but also any variant cases that might arise.
"What we do is provide a kind of assurance that careful monitoring is being done on the possibility that Canadians have acquired variant CJD from exposure to Canadian beef," Coulthart said. "And that, of course, has turned out not to be the case, and we believe it is not likely to be the case.
"So not seeing it is a very important result."
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