(Photo supplied)

It may be hard to believe that although bovine spongiform encephalopathy (BSE), or 'mad cow disease,' has cost the Canadian economy around $10 billion in lost trade and compensation, little is known about the disease.

Indeed, no solid numbers exist as to the human risk of exposure to this untreatable and fatal neurodegenerative disease. But this month, PrioNet Canada, a federally funded network that studies prion-based diseases like BSE, moved to change that by announcing $590,000 in funding for two related projects at the University of Ottawa.

The funding is part of an $8-million injection into 19 projects across the country examining prion diseases and their effects, and will fuel research conducted by U of O's Dr. Daniel Krewski and Dr. Michael Tyshenko.

"This research will bolster the knowledge base required to predict and manage the deadly impacts of prion diseases," explained PrioNet scientific director Dr. Neil Cashman. PrioNet is backed by several industry heavyweights such as GE Healthcare, ThermoFisherScientific, Epsilon Chemicals and Idexx Laboratories.

When in 2003 a single cow on a northern Alberta ranch was confirmed to have BSE, what resulted can only be described as hysteria – borders were slammed shut, thousands of cattle slaughtered and sales of beef everywhere, especially Canadian stock, slumped. During the crisis prices fell from $1.10 per pound to about 30 cents.

And when one cow is found sick the entire country is affected, said Lianne Appleby of the Ontario Cattlemen's Association. "We don't yet have the ability to close down one province, the entire U.S. border is closed, so that affects Ontario as well," she said.

Prions are a "very new and very strange" form of disease, according to Dr. Cashman. With obstacles like drought and the high dollar also affecting the industry, farmers can't afford another outbreak.

"Every infectious agent that we're used to, bacteria and viruses, have DNA or RNA to pass their genetic codes on. Prions appear to pass on at a protein-level only. We don't know the rules yet," he said.

Experts know it's possible for BSE to be transmitted to humans in the form of Creutzfeldt-Jakob disease (CJD), but no one knows the exact risks of BSE. That's one thing that Dr. Krewski, director of the Centre for Population Health Risk Assessment at the University of Ottawa, and his team hope to discover.

Dr. Krewski will look at public knowledge of prion diseases in Canada and how farmers have been affected, and will also study the much larger outbreak in the U.K. in the 1980s and 1990s.

In that case, an estimated two million to three million possibly diseased cattle got into the food supply undetected, and around 200 people developed CJD.

But it's not just a simple ratio of 200 to two million, however – several factors go into predicting a reliable number, explained Dr. Cashman.

"I'm confident that the risk will be infinitesimal and when you define the risk as one billionth, we'll all breathe a sigh of relief. But if the risk is higher, it could have an impact," Dr. Cashman said.

"All of this will go into an integrated risk management framework, which will take all of the (research) and we're going to make that available to policy makers in Canada and internationally to provide guidance to make sure we do the best job in the future in containing future versions of the disease that might evolve," Dr. Krewski said.

In a similar vein Dr. Tyshenko, a research associate at the University of Ottawa, is looking at the risk of a new treatment for diabetes that was halted once found to possibly transmit prion disease to humans.

"This is really important because parents will have to be making decisions for their children as to whether the risk is too great to be using this kind of treatment," he said. "This research will help to fill in the gaps in the research already out there."

It's one more example of BSE's huge socioeconomic impact in Canada, said Ms. Appleby. "The beef industry definitely supports this kind of research. The Canadian public needs to know that the food supply is safe and part of that is better understanding how prions work."

Dr. Cashman agreed. "Cattle ranchers never want this to happen again and what we are looking at ... is how we can prevent (this) in the future.

By Julie Fortier

Special to the OBJ

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BOVINE BUGS

Chronic Wasting Disease is a contagious neurological disease affecting deer, elk and moose. It causes a characteristic spongy degeneration of the brains of infected animals resulting in emaciation, abnormal behavior, loss of bodily functions and death.

Source: Chronic Wasting Disease Alliance of Canada

Creutzfeldt-Jakob Disease is a degenerative brain disorder that eventually leads to dementia. Symptoms resemble Alzheimer's and Huntington's disease, but Creutzfeldt-Jakob disease usually progresses much more rapidly. Worldwide, doctors typically diagnose one case of Creutzfeldt-Jakob disease per million people each year, most commonly in older adults. Treatment of Creutzfeldt-Jakob disease focuses on relieving symptoms and maximizing comfort.

Source: Mayo Clinic

BSE is a progressive, fatal disease of the nervous system of cattle. It is what is known as a transmissible spongiform encephalopathy (TSE). Scientists believe that the spread of this disease in cattle in Great Britain was caused by feeding protein products made from infected cattle or sheep, specifically brain spinal cord matter. There is no treatment or vaccine currently available for the disease.

Source: Canadian Food Inspection Agency