Canada Says Prion Disease Unstoppable

Editor’s Note: As we have suggested before on this blog, prion disease is impossible to stop. As a cluster of prion disease appeared last week in the human population near Vancouver, Canadian official’s today have announced that eradicating prion disease (chronic wasting disease or CWD) from the deer population will be impossible. Now, the goal is to control the spread in the wild deer, elk and moose populations. If only they would make such admissions on prion disease in people and livestock. (Known as Creutzfeldt-Jakobs and Alzheimer’s disease in people and Mad Cow disease and scrapie in livestock. All are caused by the same contagion–prions).

chronic wasting disease caused by prions

Unfortunately, deadly prions are deadly prions regardless of the species victimized. Therefore, making different assumptions about the characteristics of prion disease in different host species is flawed. Claiming that there is not a common denominator in prion disease between species is reckless. In other words, if prion disease is unstoppable and incurable in wildlife, we can and should make the same assumptions regarding prion disease in people and livestock. The stakes are too high in this life-and-death race with a contagion that migrates, mutates and multiplies. 

Hunters should take note that it is not safe to even touch an animal that has Chronic Wasting disease. Deadly prions are known to be in blood, saliva, feces, urine and tissue of infected mammals. Your knife and saw will become infected and permanently contagious. Same goes for the butcher shop that processes one. If your game processor has ever processed a contaminated animal, the facility is exposing every carcass that follows it down the production line. Despite what officials say, it is impossible to sterilize these facilities.

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CWD A Lost Battle

Experts say it may not be possible to eliminate chronic wasting disease (a deadly and contagious prion disease) in deer and elk in Canada. The fatal infectious disease is so well established in Saskatchewan and Alberta that the federal government and some provinces are rethinking how to deal with what is commonly known as CWD.

In 2005, Ottawa announced a national strategy to control chronic wasting disease in the hope of finding ways to eradicate it. Now the emphasis is shifting to preventing CWD from spreading, especially in the wild.

“We have to realize that we may not be able to eradicate this disease currently from Canada, given that we don’t have any effective tools, so we may be looking at switching from eradication to control,” said Penny Greenwood, national manager of domestic disease control for the Canadian Food Inspection Agency.

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The agency says it is working with the provinces and the game-ranching industry to come up with a better plan, perhaps by next spring.

“We feel that the current program that we have had in place for chronic wasting disease … is not effective in achieving its goals,” Greenwood said.

CWD is caused by abnormal proteins called prions and is similar to mad cow disease. There is no vaccine against it. Symptoms can take months or years to develop. They include weight loss, tremors, lack of coordination, paralysis and, ultimately, death.

Some scientists believe infected animals can pass the disease to other animals through saliva, blood, urine or droppings, or indirectly through prions in the environment. The prions can remain active in dirt for years. There is no evidence that the disease can affect people (there is no evidence that it can’t), but the food inspection agency recommends against eating meat from infected animals.

chronic wasting disease and moose

Prion Disease In Wildlife

The latest report from the CFIA shows the disease is active in herds of deer on Saskatchewan game farms. Scientists say the more pressing challenge is the growing number of infected wild deer and elk in Saskatchewan and Alberta. The disease has also been found in a moose. Researchers believe that in one area of Saskatchewan, up to 50 per cent of the deer have chronic wasting disease.

“This is a disease that is now established in wildlife, and when you have a disease that is established in a wildlife reservoir, it is always extremely difficult to eliminate it,” Greenwood said.

mad cow disease and prions

British Columbia and Manitoba have had no confirmed cases, but have put up billboards on highways warning hunters not to bring in deer or elk that have been shot in other jurisdictions. The caption on the signs that B.C. put up this spring in the Peace and Kootenay regions near the Alberta boundary read: “Stop Chronic Wasting Disease. Do Not Import Intact Deer Carcasses. Keep B.C. Wildlife Healthy.”

Scientists say a big challenge in tracking the spread of CWD is a lack of research into the disease and surveillance programs to track its spread. The federal government ended a program on March 31 that was established in 2005 called PrioNet Canada that was conducting research into CWD and mad cow disease.

Surveillance in the wild is difficult because there is no way to test live animals for the disease. Provincial governments rely on testing the heads of animals turned in by hunters. But in Saskatchewan and Alberta, the provinces where the disease is well-established, it is not mandatory for hunters to do that.

sewage treatment plant and disease

More needs to be done, said Trent Bollinger, a CWD expert with the Canadian Co-operative Wildlife Health Centre at the University of Saskatchewan.

“It is a long-term commitment to both research and management to see how to best approach this and those are difficult things for governments to buy into,” Bollinger said from Saskatoon.

Last year, the Manitoba government ordered conservation officers to kill as many as nine elk that escaped from a Saskatchewan game farm into the province. Bollinger predicted that with infected wild deer close to the provincial boundary, it is only a matter of time before wild animals with CWD wander into Manitoba.

Alberta is funding research into the disease through the Alberta Prion Research Institute.

Debbie McKenzie, a University of Alberta biologist, said there is concern that CWD could eventually infect other species, including caribou. She said researchers hope to develop a vaccine to stop the spread of the disease, but coming up with an effective way of vaccinating animals in the wild will pose another challenge.

“It is one thing for a farmer to vaccinate his cows. It is another thing to come up with cost-effective ways of vaccinating all of the deer and elk in an area.”


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Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform

Chronic Wasting Disease Being Mismanaged

Chronic Wasting Disease Unstoppable

Editor’s Note: As the following story illuminates, the global response to chronic wasting disease and other forms of prion disease demonstrate incompetence, negligence or criminal misconduct. I will debate anyone in the world and I will consult with anyone in the world. Mismanagement of this predatory disease is not acceptable and it could change life as we know it–if it hasn’t already. 

Initially the concern about Chronic Wasting disease (CWD) was focused on the health of the white tail deer population and the economic implications CWD could have on the deer hunting industry. New science has raised the possibility that we should be looking more closely at the potential effect CWD could have on human health, as well as hope that a new vaccine might stem the spread of CWD.

chronic wasting disease caused by prions

Chronic Wasting Disease has been a persistent issue among hunters and resource managers in all 25 states in which it now exists, including here in Wisconsin, since the first CWD positive elk was discovered in Colorado in 1967.

A positive doe found just west of Shell Lake in Washburn County in 2012 has rekindled discussion about CWD here in Wisconsin.

Initially the concern about CWD was focused on the health of the white tail deer population and the economic implications CWD could have on the deer hunting industry. New science has raised the possibility that we should be looking more closely at the potential effect CWD could have on human health, as well as hope that a new vaccine might stem the spread of CWD.

Wisconsin has more than 600,000 deer hunters who regularly harvest 300,000 to 400,000 deer annually, according to DNR Regional Wildlife Manager Mike Zeckmeister.

“Deer hunting … generates more than $500 million in retail sales and over $1 billion in total impact to the state’s economy,” he said. “A healthy whitetail deer population is critical to the state’s economy.”

land application sewage sludge

DNR CWD Wildlife Biologist Tim Marien said the state has spent roughly $43 million on the CWD program since the first whitetail tested positive in 2002. That money came from a combination of federal and state agencies including USDA and USFW. The most money was spent in 2003, $12.6 million, immediately after the initial detection of CWD in Wisconsin. The funding for surveillance, testing and research has decreased steadily since then to $600,000 in 2012.

If you do what a lot of folks do and just read the first couple of sentences of a paragraph and assume you’ve got the gist of what’s being said, this is what you’ll likely read in the first paragraph, of informational materials about the impact of CWD on human health, “there is no evidence that CWD poses a risk for humans,” followed shortly by, “The World Health Organization has reviewed available scientific information and concluded that currently there is no evidence that CWD can be transmitted to humans.” That’s not exactly what the science is saying.

prion disease epidemic

“I would not eat a deer that has not been tested (for CWD),” said retired veterinarian David Clausen, chairman of the Wisconsin Natural Resources Board.

Clausen said he is concerned that public access to information, including the latest science, isn’t what it should be to enable hunters and other consumers to make informed decisions about CWD. He’s also concerned government officials have been less than transparent in their decision-making process and that their priorities have been questionable when it came to preserving the resource and associated economy versus the health and well-being of citizens.

“We have a responsibility to be honest with the citizens of Wisconsin,” he said. “If we are doing nothing to influence the progression of CWD, we need to say that. We need to be more up front about the likely ramifications of our inaction. We cannot continue to maintain to the public that we can manage this disease by simply watching it.”

On March 20, 1996, British Health Secretary Stephen Dorrell rose before the House of Commons to inform colleagues that scientists had discovered a new variant of Creutzfeldt-Jakob disease (a fatal nervous system disease in humans), in 10 victims, and that they could not rule out a link to consumption of beef from cattle infected with bovine spongiform encephalopathy (BSE), also known as mad cow disease.

To date 217 cases of CJD have been diagnosed, mostly in the United Kingdom. The mad cow outbreak demonstrated that prion infections could cross species barriers between humans and cattle, increasing concern about the possible transmission to humans of a CWD another TSE.

Pursuant to the Federal Food, Drug, and Cosmetic Act, material from CWD positive deer and elk may not be used in any animal feed, and animal feed containing CWD positive material is considered adulterated and must be recalled from the market.

“Why isn’t the same degree of caution apparently necessary regarding human consumption of CWD positive deer?” Clausen asked.

In Wisconsin, the Department of Health Services has maintained surveillance for human prion diseases since 2002.

“Hospitals and physicians who suspect a case are legally mandated to report those to us,” said Jim Kazmierczak, state public health veterinarian.

It is DNR procedure to inform hunters who have had their deer tested for CWD, by phone, that their deer tested positive and that it is their recommendation, as well as that of the CDC and World Health Organization, that they do not eat that venison and instead dispose of it properly.

Beginning in 2004, if it was discovered that the hunter had already consumed venison from a contaminated deer for whatever reason, his or her name was referred to the DHS. A follow up call was made by DHS officials to conduct a brief interview to obtain the names of all of the people who had consumed venison from that particular contaminated deer (friends, family members etc.).

Those names are added to a registry of all hunters and other folks who have been identified as having eaten venison from a CWD positive deer. Today that registry consists of roughly 1,000 names.

“Based on the small numbers (consumers of venison) along with the long incubation period, we didn’t think it (the registry) would be a very valuable tool for at least 10 or 20 years after we initiated it,” said Kazmierczak.

Those names are regularly compared to the names of people who have been confirmed to have died from a human prion disease like CJD in the state of Wisconsin.

“The longer we don’t find any venison eaters on our list of cases, the more sure we can be. To date, we don’t have any incidences of human illnesses being related to CWD,” confirmed Kazmierczak.

Scientists have been experimenting in laboratories around the world to try and better understand how prion diseases work. Prions are tenacious abnormal proteins and impressively resilient responsible for spreading CWD. They are typically transmitted between whitetail deer in saliva, urine and feces. They can also be transmitted from soil.

A team of scientists at the UW-Madison, led by assistant professor of soil science Joel Pedersen, proved prions have an affinity for a particular type of clay, montmorillonite, found in many common types of soil. Prions bind to the soil and can remain as infectious as those transferred directly by animals for at least two years.

This is just the kind of science Clausen thinks people should know about.

“We should be asking what are the department’s (DNR) duties and responsibilities regarding increasing environmental contamination of soil and possibly plants with a disease agent that both CDC and WHO recommend people not consume,” he said.

As a result of the Mad Cow outbreak in the UK, scientists made a significant discovery. Prion infections were found in extra neural lymphoreticular tissues (tonsils, lymph nodes and spleen). Until that discovery, health officials had only been looking for evidence of prion infections in the central nervous system (CNS), brain and spinal column.

Recent research by French scientist Vincent Beringue using human and ovine transgenic mice, demonstrated that lymphoreticular tissue may be up to seven times less resistant to cross-species infection with prions than brain tissue. Beringue’s research also showed these mice lived normal life spans without showing any CNS symptoms but were none-the–less capable of transmitting disease when their splenic tissue was inoculated into other normal mice.

Because of this science and Clausen’s bringing it to the attention of the DHS, if a prion disease is suspected in Wisconsin, “both neural and extra neural tissues would now be sent to the CDC for examination,” said Kazmierczak.

CWD can cross the species barrier using a transmission mechanism called “serial passage.” Serial passage is occurring between deer naturally in nature.

“The longer CWD stays on the ground and the father it spreads,” said Clausen, “the more likely this type of thing is to happen.” In other words, the more adept CWD will become at crossing the species barrier.

At UW-Madison, the brains of hamsters were injected with CWD prions from infected deer and did not get sick. Hamsters appeared to be immune to CWD. Then the brains of ferrets were injected with CWD prions from infected deer and they got the disease and died. When infected brain tissue from the diseased ferrets was injected into the hamster’s brains, the hamsters got sick and died.

“So apparently CWD can make this end run around the species barrier,” said Kazmierczak. “It adds to our concern regarding the transmission and mutation of CWD when diseased deer carcasses are scavenged by other animals like coyotes, raccoons, and bears, even crows. We need to be guarded and cautious when we talk about CWD, we just don’t know.”

Dr. John Mapletoft works for Pan-Provincial Vaccine Enterprise Inc, (PREVENT). The Canadian-based organization connects and coordinates experts from public health, academic organizations, research institutes, the vaccine industry and the investment community to focus on the development of vaccine technologies.

PREVENT researchers have been working on a promising injectable vaccine to prevent the spread CWD among farmed cervids.

“Our goal is to prevent the disease in animals that don’t have it,” Mapletoft said.

Tami Ryan, a manager in the wildlife health section for the DNR, remembered being involved in several public forums back in 2010 when work on the vaccine was getting started.

“It did not evolve to fruition because at the time there were some pretty significant challenges to overcome with getting a vaccine developed in Canada into the U.S.,” she said. “About the same time, funding levels started to really decline (in Wisconsin) taking with it funds for research on CWD.”

A viable vaccine must meet three standards: it must demonstrate an immune response, it must be proven safe, and it must work. The CWD vaccine works by using a very short piece of protein that mimics a piece of the misfolded prion.

“We have demonstrated that our vaccine does induce an immune response in multiple species and that the immune response is specific for the misfolded prion protein,” said Mapletoft. “We also know it does not otherwise interact with or harm the brain.”

According to Ryan, one key question is if animals already infected with CWD tare vaccinated, can they still be carriers? Would they still be sharing prions and shedding prions into the environment?

Mapletoft said the current vaccine is “primarily seen to be a preventative (vs. cure) situation, so animals that already have the disease are not the target. That’s not to say it may not help them.”

The first vaccine tests began earlier this year on elk in Wyoming and another will be starting later this year in Saskatchewan.

One of the difficulties of testing a CWD vaccine is the long incubation period of the disease. Each of these tests will take a minimum of two years to determine if the vaccine is working, unlike a flu vaccine, which can be tested in a matter of days or weeks. Results from the Wyoming test aren’t expected until 2015.

“The vaccine has always been pursued with the idea that once the injectable vaccine is up and running and generating revenue, you would then have funds to proceed with a second generation oral vaccine which could be put out in the wild,” said Mapletoft.

“Frankly when the news came out recently about these trials in Wyoming, we were quite surprised,” said Ryan. “We’ll be paying attention.”

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized. 


public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area special expertise. Please contact Gary Chandler to join our coalition for reform