Surviving the COVID-19 pandemic is on our minds. With no antiviral agent to stop this coronavirus infection, the real hope is to survive long enough to receive one of the effective vaccines to generate antibodies to block infection. In addition, protective masking, hand washing, and social avoidance are necessary.
However, of grave concern, we have another untreatable always fatal disease spreading epidemically through wildlife around the world and locally. The disease is caused by structurally abnormal proteins and is called Chronic Wasting Disease (CWD) based on the appearance of the sick animals. Every infected animal dies.
No human is known to have been infected by eating meat from wild game animals infected with CWD, but based on prion science, it could happen.
My interest in prion disease spans decades and prompted me to write a novel about its spread. If it’s spread through zoo animals that consumed infected beef, will it jump species barriers and infect others?
In the UK, numerous exotic zoo ruminants and bison died after eating infected meat and bone meal. Prion disease also killed captive wild cats throughout Europe. In the outbreak of human prion disease in Great Britain, it also killed hundreds of pet cats. Outbreaks in farmed mink have also occurred. All infected animals develop severe behavior changes, restlessness, sometimes aggressiveness or timidity. Infected deer lose their fear of humans. They develop excessive salivation, tremors and staring. Their inability to eat results in muscle wasting and starvation, thus the CWD descriptive term.
In the 1990s, a protein prion form crossed the species barrier from cattle to humans killing hundreds of people who ate the infected beef.
When humans developed the disease from eating infected beef, the incubation period was often years and occurred in younger people. Symptoms ranged from early visual changes, progressing to memory loss, motor disturbances and balance problems, followed by advancing dementia, coma, and death. This human disease is called variant Creutzfeldt-Jakob Disease (vCJD). Variant correlates with the form of CJD from diseased beef.
Prions are unlike any other infections, not a viral or bacterial. Prions are highly stable structurally abnormal proteins that transmit the fatal disease through contamination and consumption. This means, if you eat beef from cattle with Mad Cow Disease, you may die. Cooking the meat well done does not inactivate prions. The structure is so stable in tissue it must be incinerated to kill it.
The abnormal proteins can survive on surfaces for long periods. The usual soap and water washing of knives, truck beds or counters where meat is butchered with is not adequate to decontaminate the areas. Research shows 40% Clorox can inactivate prions if soaked for five minutes.
Prion infected carcasses left in the field after removing consumable portions of a game animal contaminates the soil. Depending on soil type, prions may not decompose and could be infectious in the ground for years and can contaminate the watershed. Ideally, the infected game and carcasses should be burned.
Hunting is big business. When purchase of specialized clothing, weapons, vehicles and licensing fees are included, hunting enthusiasts spend billions each year. Devoted sportsmen are unlikely to give up eating game meat or stop hunting in spite of prion risks, but all hunters are encouraged to follow guidelines published by state hunting websites. All big game should be tested for prions before consuming.
Infected game animals appear normal for 1-2 years before showing symptoms, so eating a normal appearing animal without testing places you at risk. Testing tonsil or brain tissue reveals the abnormal structures, but the abnormal prions are found in muscle tissue, too, not just in neurological tissue. Brain pathology shows characteristic microscopic holes.
CJD is a complex of fatal neurological disorders first described by the two physicians whose names they carry. Some of the disorders are inherited.
Betty Kuffel, MD
Fatal Feast is available on Amazon
Hey all, I just published my solution to Forrest Fenn’s hidden treasure. Hope you like it!
About Michael J. M. Kuffel
through the The Thrill of The Chase many times, sometimes analyzing every image with a magnifying glass, other times researching references in the book, such as Billy The Kid’s .41 caliber revolver was named “Thunderer” like a mountain in the Lamar Valley. Periodically, I discovered deliberate hints that would narrow the search area and ultimately lead to the final solution.
had arrived. We planned our trip for June 2020, but on June 6th Forrest announced the treasure had been found. We were shocked and in disbelief.
I had seen this shape multiple times in Forrest’s books, including roof lines with extended overhanging roofs and attic doors, particularly a small building among boulders.
Follow this link to my Amazon Author Page: 
researcher Dr. Callie Archer vows to find a cure for the aggressive variant of mad cow disease that killed her father. Like unstoppable super-bugs, the deadly prion proteins infect livestock and wild game, threatening world food supplies. Unknowing humans who eat infected meat become paranoid, violent and die horrible deaths.
Dr. Kelly McKay struggles to complete her ER residency at Seattle’s Harbor Medical Center. Ferocious competition, burnout and an unpredictable lover complicate her life. Besides unexplained deaths of patients under her care jeopardizing her career, a sudden increase in stabbing victims points to a serial killer stalking women near the hospital. Will Kelly be next?
s or two to help prepare for the beginning nausea and exhaustion as the process starts. Then, the middle with expanding girth and files, till finally the painful labor of weaving words to reach the end. But writing those wonderful words actually mean there is some serious work to be done in the form of final tedious editing and only after that, finally, a book is born.
and my first ER based medical thriller Deadly Pyre set in Seattle.
the fuel flow in our composite home built Prospector resulting in engine failure near the summit of Lost Trail Pass on the Montana-Idaho border. With nowhere to go but down, Tom turned away from the mountain. As past search and rescue volunteers with the Alaska Civil Air Patrol, we knew survival was unlikely.
death of Valentine, our beloved German Shepherd. Valkyrie was our sixth do
g and one that proved to be the biggest challenge to train. The super-alpha female ran like the wind, lept from couches to tall bookcases in a flash to check out interesting books or toys on high shelves.
called out. Rescuers stomped down the slope in deep snow following our little dog. The skilled rescuers told us they’d tracked the impact radio transmitter with difficulty to find the crash site, but when they saw Valkyrie had hope they were closing in. (This photo was taken two months before the crash.)
people, dogs thrive with social involvement and love.
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lifetime. In her eighties, she remained active with senior groups in her community and was teaching cribbage to students when she suffered a fall and died following complications.
Best wishes on Mother’s Day.
in Montana at the NIH Rocky Mountain Lab in Hamilton, searching for ways to stop prion disease. The current concern is saving the wildlife.
Author Betty Kuffel, MD 