According to a brief report in the journal Neurology, two hunters in the United States died of Creutzfeldt-Jakob disease (CJD), which was likely caused by eating the meat from deer that had prion disease–also known as chronic wasting disease (CWD). The report is unconfirmed, but it does provide a chance to illustrate how badly prion disease, including CWD, is being managed. Answers begin with the truth.

What Is Prion Disease

Prion disease, including CJD and CWD, is a progressive, infectious disease, which means that it gets worse over time and that there is no cure. Prion disease is caused by a misfolded protein called a prion. Prions are infectious and vicious. They migrate, mutate and multiply. They kill with extreme efficiency.

In wildlife, prion disease affects members of the deer family (cervids) such as deer, elk, moose, and caribou.

In livestock, prion disease is often called mad cow disease. In humans, it is usually called Alzheimer’s disease, Parkinson’s disease, ALS and other names to help cloak the fastest-growing cause of death in the world–neurodegenerative disease.

Prions infect the entire body of victims (man or beast). Prions are in the bodily fluids and cell tissue of each victim. Blood, mucus, saliva, skin, urine, feces and organs are all infected. Prions infect everything in their path, which means that there isn’t such a thing as safe handling of an infected (dead or alive) cow, deer, elk, moose or reindeer. Caregivers for those who have neurodegenerative disease face the same risk. Prions are unstoppable and prion disease is always fatal. Infected items cannot be sterilized.

The only difference between CWD, mad cow disease or the human forms of prion disease is the species impacted. In humans, the only difference between prion diseases is the region of the brain that is under attack. If the prions start their assault in the hippocampus region of the brain, for example, the person will experience memory problems, which should generate a diagnosis of Alzheimer’s disease.

If the prions start attacking the brain in the cerebellum or the primary motor cortex, the person will have difficulty coordinating the complex muscle movements we need for smooth, controlled actions. This will likely generate a diagnosis of Parkinson’s disease. If prion disease is advanced and attacking several regions of the brain, it should generate a diagnosis of Creutzfeldt-jakob disease, like the hunters in the study. Yes, they are infectious.

The spread of prion disease is not fully understood, but it’s safe to say that there is no evidence of safe prion exposure. In other words: 

Prions + Pathways = Victims

Despite this basic logic, wildlife officials want misinformed hunters to be part of the problem. Hunters have been told that CWD is a serious disease for deer populations, but there is no strong evidence that it can infect humans or livestock. This is a lie. Prion disease is prion disease. There is no disagreement that mad cow disease is highly transmissible to humans from livestock. There is no species barrier to prion disease, including chronic wasting disease. Don’t trust any agency or anyone who claims otherwise.

Unfortunately, prion pathways are abundant. In the case of the hunters who died of CJD recently, they could have been infected by many pathways: 

1. Assuming they harvested and dressed the animals, they could have exposed themselves and others in several ways. The surviving family members still should take precautions. For example, when hunters harvest animals with prion disease, they contaminate their hunting knives and saws while cleaning and skinning the animals. The knives and saws can’t ever be sterilized. 
2. The hunters probably got blood on their hands and clothes and washed the last of it off at home. They probably got blood and hair from the carcass in the back of their truck, which cannot be sterilized and safely used again. 
3. If the animals were butchered at a local processing plant, the plant is permanently contaminated. The tables, knives and saws are spreading prions to every animal that comes down the line–not to mention the threat to the workers in the plant. They take prion contamination home every day. Meanwhile, the prions are migrating, mutating and multiplying.

The hunters and or their wives cooked up their meat on grills and in frying pans that are now contaminated and spreading prion disease with every meal. Forks, plates and more are now contaminated and spreading disease. The sink and dishwasher are contaminated because of washing these contaminated items. You get the picture.

Prion Disease Threatens Family, Caregivers

The patients themselves were likely mishandled. They infected their homes and communities before they died. Cups, plates, phones, door knobs, light switches, steering wheels, etc. are all infected with prions. Healthcare professionals probably protected themselves and the truth without warning the families and caregivers about prion infectivity.

Meanwhile, wastewater systems from meat processing plants, medical and dental facilities and other high-risk waste streams collect and incubate prions. Wastewater treatment can’t stop prions, which means that reclaimed wastewater and sewage sludge are still infectious waste. Despite the infectious and toxic nature of these waste streams, we are injecting prion disease into our food and water supplies. We are dumping tons of it on fields and in forests every day. We are reclaiming and reusing infectious wastewater like it’s gold. This prion loop recycles neurodegenerative disease from humans to humans, humans to wildlife and from wildlife back to humans. It’s no wonder that neurodegenerative disease is exploding around the world.

The symptoms of CJD can vary depending on the type of the disease, but they generally develop rapidly and worsen over time. Common symptoms include:

• Memory problems
• Difficulty thinking
• Changes in mood or behavior
• Problems with coordination
• Muscle weakness
• Vision problems
• Seizures
• Coma

There is no cure for CJD, and the disease is always fatal. People with CJD typically live for less than a year after symptoms begin. People with Alzheimer’s disease and Parkinson’s disease can degenerate to a point that resembles CJD.

Prion disease is a spectrum disease, which means that no two cases are identical and diagnosing exactly where a case falls on that spectrum is a guessing game. Neurology is not an exact science, especially when it involves neurodegenerative disease.

Prion disease has been found in wildlife in 33 states in the United States. It also has been found in Canada, Norway, Sweden and Korea, so far.

Gary Chandler is the CEO of Crossbow Communications — an award-winning public affairs and government affairs firm. We specialize in public health and environmental issues. We serve the world.

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