Prion Disease in Humans



Luckily, prion disease in humans is rare, like one in million. However, the rapid spread through wild game animals and the history of transmission of a similar disease to humans from eating prion-infected beef means knowing about the disease is important.


Misfolded proteins called prions cause a group of deadly neurodegenerative disorders in humans called Creutzfeldt-Jakob Disease (CJD), named for the men who first described them. Three main forms are:

* Sporadic CJD – most common (85% of cases), unknown source/cause but the microscopic prion structure is like the type in sheep

* Hereditary CJD – 10% of cases; in families with a history of the disease and test positive for a genetic mutation

* Acquired CJD – transmitted by medical procedures or eating contaminated meat, or as in kuru from cannibalism.

The diseases are collectively known as Transmissible Spongiform Encephalopathies (TSEs) in animals, a descriptive term depicting the holes in brain tissue from abnormal protein accumulation. CJD is the most common of human prion disease; others include Fatal Familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).

Symptoms of CJD

Neurologic symptoms in cattle bear some resemblance to prion-infected humans. Variant Creutzfeldt – Jakob (vCJD), the human form caused by mad cow prions, begins with visual changes, color variation and distortion of figures. Progression occurs over months and is varied depending on the area of brain most affected.

Symptoms include emotional instability, (crying, laughing, anger outbursts), visual hallucinations, slow thinking, memory loss, and impaired judgment. As the disease worsens, tremors, poor balance, stiffness and jerking muscles cause trouble walking. Ultimately, the person is bedridden and lapses into a coma. Symptoms overlap with other neurodegenerative diseases including Alzheimer’s and Parkinson’s.


Neurological evaluations for these symptoms often include brain MRI or CT scans and spinal fluid testing. A brain biopsy provides the definitive diagnosis, but new blood and urine tests are available. Microscopic findings show characteristic protein accumulations with holes, making brain tissue look like a sponge. To identify the origin of the disease, differences in the abnormal folding of prion proteins can be determined at the Human Prion Surveillance Center in Cleveland, Ohio.

We have hope for rapid diagnosis and treatment with researchers around the world andBaby black ears.edsquare.jpg in Montana at the NIH Rocky Mountain Lab in Hamilton, searching for ways to stop prion disease. The current concern is saving the wildlife.




More information can be found online at the NIH Fact Sheet website:

I am finishing final editing on Extinction, a prion medical thriller and will soon be ready to query agents.

Thanks for stopping by.


Betty Kuffel, MD

Posted in Beef, Biological Thriller, Creutzfeldt-Jakob Disease, Deer, Medical Thriller, Prion Disease | Tagged , , | 3 Comments

Prion Disease in Man and Beast

I just finished writing the second of a two-part series on prion disease for my Montana Woman magazine monthly Lipstick Logic health and lifestyle column. The prion topic is important, and few people really understand this fatal disease and potential for contagion.

Over two-hundred people died after consuming prion-infected beef during the epidemic in the 1990s. Today, 1 in 2000 British people show evidence they carry mad cow prions but have not yet developed symptoms.

The cause of the mad cow epidemic in Great Britain was from animal food contamination. Rendering plants accepted prion-infected cattle and sheep carcasses and processed protein slurry carrying the deadly prions was made into animal feed. By the time the source was identified, it was too late. People died and millions of cows had to be destroyed.

Feeding cattle animal protein was banned and is still banned in the U.S. The few cows detected positive for prions today are thought to be from mutations and not spread from other animals. Just as the many types of prion diseases in humans, some are inherited mutations. Sometimes in the past, prion disease was caused by infection from contaminated surgical instruments, tissue grafts, corneal transplants and human-based growth hormone. Surgeons and pathologists have died from accidental contamination. Funeral homes have special safety regulations for body handling. Cremation to destroy the prions is encouraged.

The disease first made news in the 1920s when members of the Fore tribe in New Guinea were found to have kuru from ritual cannibalism. They called it “laughing death” because of the terrible neurologic symptoms victims experienced before finally lapsing into coma and dying. Parts of the contagious corpse were sometimes eaten by tribal members and spread the disease.

The Centers for Disease Control and Prevention (CDC) considers prion infection in wildlife an emerging disease. Epidemic spread in wildlife is occurring. Currently 25 states have identified infected wild game animals including deer, elk, antelope, moose and reindeer. Captive bison have also been infected. It is also in Canada, Norway and in imported elk in South Korea. Hunters and anyone who eats game meat should know about the disease and use caution.

Today, 1 in 2000 British people show evidence they carry mad cow prions but have not yet developed symptoms. This estimate is based on tissue biopsies appendectomies from people without neurologic symptoms. No one know what this means. Are they carriers who will someday develop prion disease? Can they transmit it to others? Is it in their blood? On the blood donor questionnaire there is a list of over 40 European countries where possible exposure to CJD may have occurred.

At this time, no human has been identified with prion disease from eating infected wild game, but a recent Canadian study confirmed primates (Rhesus monkeys) died from eating CWD infected venison. With a close genetic relationship to humans, CWD prions crossing the species barrier from deer to the monkeys suggest it could happen in humans like it did with mad cow infected beef. What can we do to prevent potential human prion disease from big game?

In the past two years, the Department of Fish, Wildlife and Parks detected 26 cases of CWD in Montana along the northeast Canadian border and in numerous counties extending from North Dakota into an area south of Billings. Surveillance of hunter kills, and planned hunts where infected deer are found are carried out to cull potentially infected animals to slow the spread.

Of concern is ease of transmission and the epidemic spread among wildlife. The Montana FW&P website details the process to test animals for hunters for a small fee.

Prions are difficult to destroy. To avoid the disease, you must avoid contact and consumption. Cooking the meat well-done does not kill infectivity. Incineration is recommended for contaminated items and cremation for all prion-infected human and animal remains.

Recently, studies showed hypochlorous acid (Briotech) can kill prions on surfaces. Before Briotech, lye solutions were effective in destroying prions but usual hospital sterilization techniques failed, leaving surgical instruments contaminated and able to spread disease.

In a press release from the University of Washington: “Briotech (BRIO HOCL PrP Formula) has been laboratory tested and proven to be the world’s first safe method to eliminate all detectable seeding activity of misfolded infectious proteins and is safe for skin and mucosal contact.”

For more information contact:

FWP Wildlife Health Lab at (406) 994-6357 (for testing information)

The next blog will be on disease symptoms and diagnosis.

Thanks for stopping by.

IMG_4281Betty Kuffel, MD

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Free E-book – Alaska Flight

Fly Away to Alaska!



If you would like to taste life in the wilds of Alaska, this is your free transportation! Take a thrilling trip with a flying doctor, and a nurse trying to escape her past.

Flight nurse Liz Elliot quits her Arizona job and flees to Alaska to cool hot emotions after a medical helicopter piloted by her lover crashes, killing him and two close friends. Her escape from grief backfires when she meets Paul Lasher, a charming flying doctor in need of her emergency skills in the Alaskan bush. They learn her friends were murdered. Will Liz be next?

I had fun reliving my life flying around and working in Alaska. I hope you have a safe trip. Please write a review on Amazon when you return.


Check out my site on Author Shout

Author Shout Author

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Writing and Technology

Technology Chaos and A Mouse


Before I embark on my tech story, allow me to introduce myself.

I am a retired medical doctor. My specialty was internal medicine and I spent most of my medical career in ERs making rapid diagnoses and interventions. Since retirement eight years ago, I have authored eight books and indie-published six of them. I am up most days before sunrise at my computer, writing.

A few weeks ago, without warning, my desktop computer gasped and turned blue. I immediately recognized it as the blue screen of death. With the speed of an ER doc, I grabbed my laptop, and sought a solution on Amazon. I found what I needed — a new one-terabyte hard-drive. This magical piece of technology that 40 years ago would have filled a room, arrived within two days, sealed in a small bubble-wrap envelope. My ill computer and the new hard-drive took an ambulance ride with me to a Tech ER.

The computer doc greeted us and immediately went into resuscitation mode. He surgically removed the old-hard drive and performed a transplant before my eyes. With a flick of the switch my desk top computer came to life, data saved, with no residual brain damage.

Experiencing personal relief with no residual Post Traumatic Stress Disorder, I returned to my passion of writing. Up before dawn, words flowed from my brain through my fingers and onto my screen with the speed of light. My new terabyte transplant operated at Mach speed and showed no signs of rejection. I believed my technology problems were over, but within a week, I experienced another technology catastrophe.

Without warning, my lifeline to the world experienced an energy failure. The battery of my cell phone refused to hold a charge.

When patients complained about old age, low energy, bad joints or a sleep problem, I understood. With age, the body slows down and sometimes needs a boost – a thyroid hormone, a joint replacement, or sometimes, just a good night’s sleep to recharge the body’s biological batteries.

Maybe all my cellphone needed was a good night’s sleep. I turned it off, plugged it in and checked it in the morning. Nothing. A visit to an urgent care battery clinic seemed simple enough. The expert carefully removed a warped battery looking like it had an aneurysm ready to blow. He confirmed it wasn’t low thyroid after all, but a function of old age.

The new battery didn’t charge. Battery-man turned to electronic diagnostics for help, but alas, the phone flat-lined.

My phone was not repairable. At least my phone had died a natural death. My two previous cell phones had experienced terrible drowning deaths. One took a dive in an outhouse, the other glugged to the bottom of a delicious latte, taking my data with it.

My search for a new phone began that same afternoon. Unwilling to pay the price of the latest model, I looked for a refurbished phone — the search took me down a rabbit hole of hundreds of phone options and unfamiliar terminology. I needed a technology specialist’s help.

Using my landline, I called a techie relative and took his advice. I found a refurbished cellphone online. Two days later it arrived in a bubble-wrap envelope. I visited my Straight Talk service provider who transferred my data and sent me on my way feeling connected once again. Back home, I added new apps, including an exercise calculator to remind me to get off my chair and move around more. It’s like having a personal trainer in my pocket.

I returned to my writing, enjoying the new technology. Everything ran smoothly for several days, until another shocking malfunction occurred. It was 6:00 am. I had been working at my computer a little over an hour when my Fitbit reminded me to get up and and start counting steps. I walked to the kitchen, made a fresh pot of coffee and returned with a steamy cup of my favorite brew to continue editing my latest novel when both screens suddenly went black.

I followed a tangle of umbilical wires from monitors to computer. They were braided together with telephone, lamp and microphone wires, cords that had functioned for years. In the end, the main monitor was “undetectable.” Seconds earlier that same monitor was working fine. This looked like sudden death. Had the hard-drive totally failed? After wiggling a lot of cords, both monitor screens flickered and lit up, but without icons, mouse function or anyway to access my computer or the internet.

I went to my trusty laptop again, to troubleshoot the problem. After reading through many forums, I concluded my computer needed a new monitor driver.

After more reading and looking at a couple instructional YouTubes, I changed my diagnosis. The computer needed a new video card. I wasn’t exactly sure what a video card was, but I did find a large selection on Amazon. I had to do more research before placing an order, so watched a few more YouTube tutorials. I had trusted cute guys in bib overhauls to help me through fixing my dishwasher and tiling my laundry room but was not feeling confident they could guide me through a video card transplant.

I decided to continue untangling the rats’ nest of cords from behind and beneath my desk. The cords ran from monitors to computers, to a backup laptop, two external hard-drives, a landline, a video camera, and cell charger. I carefully labeled all the cords at each end and re-checked my connections. It took several hours of sitting on the floor with dust bunnies running by and dead flies surfacing from beneath the tangle of cords.

A final check suggested the cords were all connected properly until I moved the monitors to dust behind them. The main monitor cord looked like a highly diseased neuron. The sheath over the wire was severely damaged. Tiny missing chunks exposed bare wires. Defects along the wire looked like nibble marks. Hmmm.

Could that adorable mouse that visited me a few months earlier have been chewing on my main monitor cord? I had met him eye to eye when he was sitting on the top of a can of beans in my pantry munching on a trail-mix bar. It was probably tastier than the covering on the monitor cord.

His round pink ears looked far too large for his little head. Black eyes stared back at me looking as though he had to seriously consider whether to drop the delicious trail-mix bar and run away, or not. I couldn’t kill him. He was so adorable.

I told him to stay put but that I was issuing an eviction notice. I turned on the vacuum and sucked him through the hose into the transparent dust catcher. He landed on a cushion of dog hair and looked out at me from his plastic cage. I would have scolded him for chewing my computer cord had I known at that time what he’d been up to, but he was so little and frightened, I took him outside and let him go find a new home.

I decided my desk top computer needed a complete check-up and I wasn’t up to the task. The gnawed cord was not the problem, but I replaced the cord and dug out an old laptop to use as a secondary backup while the desktop went back to the hospital.

I plugged in my old laptop and found out it was just too old to run anymore. The XP operating system was no longer supported by Microsoft or anyone else. Besides, it was locked and required an administrator for access. I am its administrator, but do you think I could remember the password? Not on your life. It was inaccessible in my brain data bank.

I returned to my new laptop to contact the Best Buy Geek Squad to help me figure out how to get the desktop computer and monitors functioning again.  Suddenly, an alert appeared on the laptop screen telling me to immediately change my password. Someone in a nearby town was trying to log onto my computer.

I quickly changed my password and wrote it down in a secure location.

The Geek Squad gave me an appointment time and said to bring my computer and monitors in for a complete diagnostic exam. I unplugged all those cords I had just finished labeling and rearranging. I loaded up my computer and made an ambulance run to the Best Buy ER, staffed by amazing geeks with crazy hairdos, body art, and so many facial piercings I was concerned they might short out. Within a day, they discharged the computer to my care in fine shape.

I diligently save and back-up my work on an external hard drive. I also save my work in a cloud where mice don’t live.

My computers and monitors are up and running. I have returned to writing medical thrillers full-time. My books are available on Amazon. Check them out. All proceeds from the sales of my books will probably go to the repair or purchase of new technology so I can keep writing.

I love technology – when everything works.

Thanks for stopping by.

Author Betty Kuffel, MD

Feather women

Remember, nothing is black and white.


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Marketing Indie Books

booksIndie-authors publish their books on Amazon and many other sites without the help of agents. There are advantages such as receiving 70% royalty on the cover price of e-books. The huge disadvantage is many excellent books go unnoticed due to lack of adequate marketing. Writers are writers and often unskilled in selling themselves or their products.

Writing a book from beginning to end requires focus and endurance, often taking years if not decades. Finishing a manuscript is a great accomplishment but reaching the end of the first draft is the beginning of another huge time-consuming step. Numerous edits, rewrites, beta-reads and paying for professional editing finally brings the book to the point of publication. At that time, the author makes the decision to either indie-publish or begin writing query letters to agents if seeking traditional publishing.

First time authors find the years of writing a novel or non-fiction book to The End quickly realize those words really mean –  The Beginning of Marketing. Whether self-published or through a contract with a traditional publisher, advertising and marketing will take the author on a new journey. Unless you are a celebrity, large book tours are a thing of the past.  Marketing takes unexpected time and education unless you are already an expert in this field or can hire a publicist.

Some indie-authors are highly successful marketers, many on their internet sites providing webinars, others on self-designed and orchestrated book tours, or by participating in writer events. If you have a platform for sales, such as writing a self-help book in a field where you are an expert, you already have a following who will purchase your book.

If you have indie-published and are ready to market, there are hundreds of options that20190108_064453 can take a tremendous amount of time. I have watched many webinars, talked with successful marketers and bought a stack of books on the topic. There is no easy way.

Below are a list of URLs as a starting point. I have not found a site that has everything, but I have used theses and found them helpful.  – Blog with numerous articles of interest – Comprehensive information on publication, book cover design, book trailers and banners to use on Facebook and Twitter, and ads to purchase to feature your books.

There are numerous sites to feature free books: Kindle Nation Daily and Digital Book Today – two I have used.

Offering free books is a marketing technique to generate visibility to that title and other books on your sales page on Amazon. Writing reviews on Amazon on the books you read is helpful to the author. Some advertising sites such as BookBub require a certain number of reviews before they will run a book ad.

Thanks to everyone who downloaded my free books on Jan. 5 & 6

Authors of the Flathead will have a panel of authors present a Thursday evening class on this topic in the near future. Check out for our meeting schedule and topics.

Happy New Year and Happy Writing,


Posted in Appendices for Download | 3 Comments


On Amazon: Jan. 5 & 6








deadly pyre betty kuffel 3d cover mockupDeadly Pyre is the first in my Kelly McKay Medical Thriller Series. Experiences as an ER physician and pilot energize my writing. Search and rescue, sled dog racing in Alaska, and surviving a plane crash in the snowy mountains of Idaho inspire stories including this novel set in Seattle.

Please download a free copy during this weekend sale. Share the free offer with your friends. I’d appreciate a review on the Amazon site.

Review from Amazon:

A Crash Cart Ride through the ER March 27, 2018

Lives saved and lost in waves of trauma, exhaustion, buried emotions, drug addiction, competition, and murder. Deadly is suitably named!

Fascinating medical descriptions transported me into the mind of a talented doctor trying to survive her last months of residency. For anyone who wonders about the courageous souls breaking their backs in the cutthroat medical field, this is for you!






Heart disease is the number one killer of men and women. With the current epidemic of obesity, evidence of heart disease is now seen in children. After years of caring for people with these diseases, I felt compelled to write this guide to help people understand the science behind heart health and make informed choices.



Your Heart will be available free on Amazon all weekend. I would love to have you share the free offer with your family and friends. Please write a review on Amazon.


Review from Amazon:

I plan to purchase books for each of my sons and hope they read every word to learn how important it is to eat healthy, exercise regularly and keep their blood pressure and cholesterol levels within normal range. Maybe then, they will be able to offset any bad family genes they may have inherited from me. Dr. Kuffel’s encouragement to try the Five/Two method of eating as a way to reduce weight has been unbelievably easy to follow and effective. Friends of mine have also used the Five/Two plan and dropped several pounds within weeks. It is reassuring to know the information provided in Your Heart is science-based, well researched and written by a very experienced doctor. It is an excellent handbook for anyone wanting to know how to change their lifestyle to improve their health.

Thanks for stopping by.

I hope you enjoy the books.



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Extinction – A Biological Thriller


Buck.5One of my current projects nearing completion is a bio-thriller addressing the real threat of spreading prion disease. There have been numerous beginnings and titles, but the ending has never changed – death. After years of research, multiple critiques, two final beta reads and a new title, I have the book nearly ready for agent queries.

Since the outbreak of mad cow disease in Great Britain in the 1990s, awareness of this contagious fatal disease has improved. More people recognize the term prion, (pree – on) but few experience the proper level of alarm at this strange contagious protein found in meat. The abnormal infectious protein acts like an untreatable virus or bacteria targeting the nervous system that inevitably kills.

Scientists have studied prions for decades and are closer to early diagnosis in cattle andAngus bull wildlife. Prevention is key to survival. Spread among animals, and to humans must be stopped. Infection means death. It takes 1-2 years for symptoms in animals to become apparent so early detection to cull infected animals before they enter the food supply is essential. You could be eating contaminated beef, venison, elk, and moose without recognizing it is infected.

Cooking the meat well-done doesn’t damage prion infectivity. The protein is resistant to common methods of sterilization and is stable in the environment, contaminating ground for decades. Do I have your attention?

I’ll be busy sending out queries soon. Stay tuned.

Thanks for stopping by.



Posted in Beef, Biological Thriller, Deer, Medical Thriller, Prion Disease | Leave a comment

Free Medical Thriller e-Books

Two New Medical Thrillers FREE on Amazon September 28, 29 and 30

Deadly Pyre – A Kelly McKay Medical Thriller – Book One is set in Seattle



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?


Alaska Flight – Romantic Medical Thriller

Flight nurse Liz Elliot vows never to fly again.

Liz quits her job after a medical helicopter piloted by her lover crashes in the desert, killing him and two close friends.

Alone in Alaska and no longer flying, she feels safe and ready to begin a new journey. During her interview at the Alaska Native Hospital, a male nurse burst in and quits, leaving flying bush doctor Paul Lasher without a nurse. He needs Liz’s help for an emergency preterm deliver in the bush. He must leave immediately to reach the woman in a remote village in time.

Not knowing her past is about to catch up to her, Liz accepts the challenge, flying to the wilderness emergency with an attractive stranger. She wonders if she’s lost her mind, as she is about to lose her heart and her life.


I hope you enjoy them. Please share the offer with your friends and write a review on Amazon.






Posted in A new book, Medical Thriller | 1 Comment

Fatal Feast – a biological thriller

Prion Disease

  Chronic Wasting Disease = Mad Cow Disease = Human Disease

If you are a meat eater, is it time to worry? Maybe.

Eating meat can kill you. Remember the Mad Cow disease outbreak in Great Britain in the 1990s? Eating beef from mad cows caused the disease in man. The same disease is spreading through wildlife across the United States and Canada.

When diseased beef is consumed by humans they develop the always fatal variant Creutzfeldt-Jakob disease (vCJD) and die within months. This disease is spreading through wildlife in the United States and elsewhere. In wildlife, it is called chronic wasting disease. There have been no verified cases of transmission to humans from eating infected deer and elk meat, but based on transmissibility of mad cow disease to humans, it is possible.

When prions enter the body the infectious protein spreads in fluids, organs and muscles, accumulating in the nervous system causing progressive disability and death within months.

Prion disease has been around for decades, maybe longer. My first real interest in this strange contagious disease began with studying the neurologic disease in the Fore people of Papua New Guinea who developed Kuru spread by death ritual cannibalism. Few people worry about prion disease because they are not cannibals. But the meat you eat may be contaminated.

Thanks for stopping by.


Posted in A new book, Appendices for Download, Biological Thriller, Deer, Medical Thriller | Tagged , , , , | Leave a comment


Your Heart – Prevent & Reverse Heart Disease in Women, Men & Children


FREE E-BOOK         FEBRUARY 13, 14 & 15

 Heart Disease – Then and Now

The United States designated February 1964 as the first American Heart Month to raise awareness of the number one killer. More than fifty years later, in spite of increased awareness resulting in lifestyle changes, reduction in smoking, and increased exercise, all modifications that reduce risk, it remains number one killer in men and women around the world.

In addition, an obesity epidemic has pushed millions of people into Type 2 diabetes, that markedly raises heart disease risks. Early heart disease changes are now seen in elementary age obese children.

Cardiovascular disease includes heart disease and stroke. It kills more people than any other health problem.

In 1960 when my father died of his second heart attack at age 52 (his first occurred at age 46), we knew a positive family history for heart disease, eating fatty meat, and smoking were bad. Heart attack treatment back then consisted of bedrest and oxygen. There were no medications to lower cholesterol, and heart medications were few.

Science has given us the biology behind this devastating disease. We know what happens at the molecular level and how to modify contributing factors. We know high blood fats, low activity levels, obesity, and tobacco use all contribute to early death from heart disease.

You ask, what about heart disease that runs in families?  You can counteract “good genes” by making bad life choices: overeat, use tobacco, and don’t exercise. Or, you can offset “bad genes” by making healthy choices.

Dramatic interventions to dilate and stent diseased heart arteries, or surgically bypass vessels that are too diseased to stent, have saved lives. But there are many options for people to take control and make healthy changes to lower risk before something happens.

With the right knowledge and actions, the number one cause of death in women and men is preventable. If you are healthy and want to stay that way, or if you are overweight, have diabetes or cardiovascular disease, you can live longer by modifying your risks.

After taking care of hundreds of people in crisis from heart disease over years of work in emergency rooms, I wrote a heart health handbook. In just two-hundred pages, Your Heart will give you the science behind the disease, some actions to improve your health and common interventions that could save your life or the life of a loved one. Many dietary options are available including the Mediterranean 5/2 diet that can help you lose weight for life.

Happy Valentine’s Day.

Take care of your heart.

Betty Kuffel

For more information:


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