Prion Pandemic in Wildlife

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

Michael Kuffel’s New Book

ANNOUNCING

A NEW BOOK BY MICHAEL J.M. KUFFEL

Hey all, I just published my solution to Forrest Fenn’s hidden treasure. Hope you like it!

In 2010, Forrest Fenn hid a treasure chest in the Rocky Mountains. I began investigating this unsolved mystery in 2018 by analyzing maps, books, and online references until I pieced it all together. June 2020, My mom and I geared up and hiked off trail into the wilderness to a hidden location where the treasure appears to have sat undisturbed for nearly ten years. This publication is a synopsis of key discoveries and photos from our trek. Forrest and the finder have yet to confirm the location, and perhaps they never will, so I invite you to consider this analysis a convincing proof verified in person.

Amazon link for purchase

About Michael J. M. Kuffel

Mike Kuffel is a former Enterprise Data Warehouse manager at University of Washington Medicine Information Technology Services, and a graduate of Cornell University. After over 20 years managing and maintaining complex technologies, he left the workforce in 2018 to spend time with his family and began investigating the unsolved mystery of Forrest Fenn’s hidden treasure. Years of technical analysis and troubleshooting proved valuable experience for finding anomalies and nuances in the myriad images and other content in Forrest Fenn’s books and online resources. After completing the quest for the chest in June 2020, his sanity has returned to “normal.”

Forrest Fenn’s Treasure Location Found?

Guest Blog by Mike Kuffel

As I scrolled through news feeds while riding the bus to work one day in early 2018, a headline caught my eye about a treasure worth millions hidden in the Rocky Mountains in 2010. I was skeptical, but read about Forrest Fenn hiding a chest full of treasure he had collected over the years that could only be found by solving a poem he wrote. My curiosity grew, so I did a little more digging, and found a few trustworthy sources, such as dalneitzel.com. A vast online world of treasure hunters shared ideas. Many followed their theories into the mountains and a few tragically died trying.

After hundreds of hours analyzing Forrest Fenn’s memoir, The Thrill of The Chase, and other resources, I finally pieced together the location of Forrest Fenn’s hidden treasure. In addition to his first memoir, I bought and read many of Forrest’s books, and looked for other publications that included information from Forrest. In addition, I spent much of my time scouring satellite maps for site names and plausible search areas that aligned with the poem.

I started recognizing mountain regions without checking names and landmarks. I read 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.

After diving down many rabbit holes, I eventually used the vague poem as guardrails that could only be clarified by aligning geographic references and GPS coordinates found in Forrest’s books and other content. There may be other ways, and no doubt I missed more hints than I found, but the references below led me and my mom to the end of Forrest’s rainbow (44° 51′ 57″ N, 110° 08′ 48″ W).

Forrest has yet to reveal the location, but I have extensive corroborating evidence that demonstrates the validity of this location, IMHO. I am contemplating the best way to share more detailed information that led me to this location.

Forrest Fenn’s Treasure Poem

1. “Begin it where warm waters halt”
   1. Start at Ice Box Canyon, NE corner of Yellowstone National Park
2. “And take it in the canyon down,”
   1. Drive down highway 212 along Soda Butte Creek
3. “Not far, but too far to walk.”
   1. Continue driving a few miles
4. “Put in below the home of Brown.”
   1. Park and cross Soda Butte Creek on foot at the Lamar Bridge
   2. This location was identified via geographic references, not by the meaning of Brown
   3. Home of Brown could refer to the Lamar Valley Ranger station once occupied by Ranger Brown, the Buffalo Ranch, Bison of Lamar, Brown Trout, Brown Bear, Moose, basically anything big and brown in the area.
5. “From there it’s no place for the meek,”
   1. Hike off trail into the wilderness angling (pun intended) toward the left end of the big cliffs near the bridge
6. “The end is ever drawing nigh;”
   1. Hike toward a narrow draw between the hills at the left end of the cliffs (the location was determined by analyzing drawings, photos, and paintings)
7. “There’ll be no paddle up your creek,”
   1. Hike uphill alongside the stream, avoiding steep switchback areas, forging through downed trees, dense forest, and rocky areas
8. “Just heavy loads and water high.”
   1. Look for the large boulders on the right upstream
9. “If you’ve been wise and found the blaze, Look quickly down, your quest to cease,”
   1. If you found GPS coordinates (44° 51′ 57″ N, 110° 08′ 48″ W), you will find a small shelf below a boulder with an overhanging ledge where the chest once sat.
10. “But tarry scant with marvel gaze, Just take the chest and go in peace.”
    1. Don’t stand there marveling, enjoy the thrill of finding the location the chest once occupied, then make a lot of noise before a bear sneaks up on you!
    2. Seriously, do not make this trek without being fully prepared to encounter wildlife.

For anyone who has purchased The Thrill of The Chase, the best example of hints within art is the drawing of a bombing run. Hints include geographic references of the Lamar Valley, including the Soda Butte cone (bottom middle). The hills in the background have an arrow pointing down in a narrow canyon (top middle). Also, find GPS number 51 in the stones in the bombing area on the right. There are numerous 8s and/or 88 (8.8 minutes is 8 minutes 48 seconds). (p.99 The Thrill of the Chase chapter My War for Me)

In October 2019, I believed I had enough evidence to confirm the chest was within this field of boulders. However, we had just returned from Yellowstone that September and snow 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.

Ah well, we had always been more interested in solving the challenge than collecting the reward, so we kept our plans and continued our adventure to our own thrilling conclusion. Could we find where the chest had been?

We knew confirming the location would be harder without a treasure chest as confirmation. I wanted to correlate satellite shapes in the boulders, but none of them panned out in person. GPS signals drifted, making the task more difficult as well. We searched high and low in the rocks until my mom noticed a small flat shelf beneath an overhanging ledge that formed a triangle. 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.

We found where Forrest hid the treasure. The chase was over.

My mom and I made five trips to Yellowstone since May 2018, and almost bagged the prize. We enjoyed the “Thrill of the Chase” and crossed the finish line. We had great adventures and will always cherish the memories and photos of our adventures in Yellowstone. Thanks Mom!

Thank you for visiting!

Mike Kuffel (mikek888@yahoo.com)

FREE E-BOOK EVENT

FREE E-BOOK OFFER for 4 DAYS JANUARY 23-26

FATAL FEAST  &   DEADLY PYRE

Four days: Thursday January 23  through Sunday January 26

Follow this link to my Amazon Author Page: FREE BOOK LINK

Please share the event with friends and write a review. I hope you enjoy the books.

FATAL FEAST    A Biological Thriller

Fatal Feast is set in Montana. A prion pandemic threatens the world as brilliant young 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.

As the disease spreads in ranching and hunting country, authorities suppress public information to save the country from economic disaster. Callie’s promising treatment may be the only hope to prevent a world-wide pandemic. With forces against her mounting, can she save mankind and herself?

FREE BOOK LINK

DEADLY PYRE   A Medical Thriller

Deadly Pyre – Book One in a medical thriller series 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?

FREE BOOK LINK

 

Birthing a Book

Writing a book is a lot like pregnancy. You think about having a baby for a long time, often years before making the decision. You research many aspects and even take a class 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.

I just delivered my seventh book, Fatal Feast. Some people say after your first, book or baby, each one that follows is easier. I can speak to experience with birthing multiple books but only one child. I was driven to write my first three medically related science-based nonfiction books. The next four, medical thrillers, were easier. Instead of being totally fact-based and tedious, the novels were much more fun because you just make it up.

My interests in neurology and infectious diseases meshed with the outbreak of mad cow disease in Great Britain thirty years ago and evolved to the bio-thriller concept for Fatal Feast. The strange contagious protein killing cattle spread to hundreds of people who died after eating infected beef. Three decades is a very long gestation, so you can imagine, giving birth felt like a great achievement. With Chronic Wasting Disease, a frightening prion variant of mad cow disease spreading through wildlife and threatening humans, the Fatal Feast delivery is timely.

I am in a recovery phase, getting back in shape, and exercising more after book cover design and intensive weeks of editing to produce the final product. Now, I have more time to read and catch up with non-writing projects. Newborns sleep a lot but need attention and if you are going to show off your new child (or grandchild), marketing must become a prime “postpartum” focus.

Most writers are not skilled speakers, nor do they like to talk about themselves. As for me, I’d rather be writing than spending time showing off my offspring. However, like any project, marketing requires research and possibly stepping outside your comfort zone. One common method to provide books an avenue to expand visibility and generate reviews is to offer E-books free of charge for special events.

So, with this birth announcement I am also announcing a four-day free event for two of my medical thrillers, the new baby, Fatal Feast, set in Montana, and my first ER based medical thriller Deadly Pyre set in Seattle.

The event begins tomorrow January 23^rd and extends through January 26^th. Please share the event with friends.

My next blog will post with the information and hot links just after midnight tonight.

Thanks for stopping by to meet the babies.

Betty

 

Chronic Wasting Disease in Wildlife

CWD Concerns

I have followed prion research since the outbreak of mad cow disease in Great Britain three decades ago. With a mad cow variant prion causing the Chronic Wasting Disease epidemic spreading in wildlife, this blog is an overview of a disease I believe carries great risk to man and beast. My new biological thriller, Fatal Feast, is set in Montana, featuring pandemic spread of prion disease.

Amazon

Hunting in Montana is a way of life. In some families, killing your first deer is a coming of age event. Mounting a trophy on the wall, filling the freezer with venison or elk each fall, and hunting into old age are common. But is it still safe to eat venison, elk, moose and other wild game? Maybe, maybe not.

Across the United States and in Canada the public have been alerted to the epidemic spread of a fatal neurological disease in wildlife. They are cautioned not to eat infected meat, to handle meat with caution, and instructions to limit spread from infected carcasses.

However, the infectious agent is invisible and will contaminate surfaces and tools in contact with blood and other body fluids. All contaminated surfaces should be washed with soap and water, then soaked for five minutes with 40% bleach. Infected tissue must be incinerated to stop infectivity. Prions are not killed by usual sterilization techniques. In fact, people have died after being infected by disease transferred to them from “sterilized” surgical instruments

It is true no humans have been identified with CWD from eating prion-infected meat, but research has shown, both spider monkeys and macaques are susceptible and die from eating infected venison. With these non-human primates having genetic makeup so close to humans, the likelihood of CWD disease jumping the species barrier and infecting humans is possible.  CWD is a variant of mad cow disease that killed humans in Great Britain in the 1990s. Humans died after consuming infected beef.

The first CWD positive deer was identified in Montana in 2017. Since that time, the infected animals have been identified on both sides of the Rocky Mountains. Twenty-seven states and three Canadian provinces now have spreading CWD. Alaskan caribou have the genetic makeup risking spread of the disease to them. In Norway, 2,000 reindeer were exterminated in an attempt to stop spread in an expansive herd. In Montana and elsewhere, kill zones, like the one in Libby, are organized to euthanize large herds of animals at risk for spread.

Prions are stable in soil for years and are taken up in growing plants that may be able to transmit the disease. Carcasses left in the forest contaminate the ground for years, sometimes decades, depending on the type of soil. In the wild, prions are spread naturally to scavengers such as mice. Crows are known carriers. Studies show transmission in herds from licking behavior and ground contamination easily spread disease.

Ill animals are easy to identify when the disease has progressed. They appear weak, confused, thin, unafraid of humans, uncoordinated and drooling. Early in the disease process as prions spread through the body, the animal appears normal but is contagious. Friends of mine driving along the west Hungry Horse Dam road stopped their vehicle to watch an ailing doe with twin yearlings. The young ones were lively and beautiful. The doe appeared unstable, thin, and stumbled up to their car and pressed her nose against the side window, peering in at them with vacant eyes. No positive CWD deer have been identified in that hunting zone, but it appears CWD has arrived there, too.

After the unusual protein, a prion, acting like an unstoppable super-bug, enters the body, disease begins in the small intestine concentrating in lymph tissue. Gradually, over about a year and a half in deer, prions spread along nerves to the brain before symptoms are visible.

Clumps of the abnormal protein disrupt nerve function and destroy brain tissue. Microscopic examination of brain tissue shows resulting cell damage and holes making it look like a sea sponge, thus the name spongiform. Mad cow disease is called bovine spongiform encephalopathy. The generic term for an array of prion disorders in animals and humans is transmissible spongiform encephalopathy (TSE). There are many forms. Some human forms are inherited.

CWD information is available on state hunting websites.

http://fwp.mt.gov/fishAndWildlife/diseasesAndResearch/diseases/chronicWastingDisease/management.html

A Lucky Dog Turns Seventeen

Valkyrie a Wonder Dog

Many of you may know Valkyrie. She has been a wonderful part of our lives for so many years. I want to share her story with you as she reaches a landmark of survival for a large dog. Tomorrow, Thanksgiving Day, is her 17th birthday. She is moving slower than her puppy days but still filled with life and is loved dearly.

April 2019 marked the 16^th anniversary of Valkyrie surviving a plane crash in the mountains of Idaho and being found after disappearing in snowy mountain lion country for ten days. On an outing with friends in two other light planes, carburetor icing stopped 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.

Within seconds, we struck tall pines at 100 mph ripping off both wings, spinning the plane like a violent crashing carnival ride from hell, impacting nearly inverted on a snowy slope. To our surprise, we were alive, but for how long. Tom was trapped in the wreckage with his upper body resting on the snow. I was awake, talking, moving everything, but a lower leg bone stabbed through my favorite jeans. Not good. As an ER doctor, I knew what to do, but that’s another story.

This is Valkyrie’s story.

Valkyrie, an experienced flying 1-year-old German Shepherd mix puppy survived impact uninjured and hopped out via the broken canopy. She appeared confused and sat a few feet away up on the mountainside looking down at the crashed plane. Val remained close as I used the pant leg as a sling to lift my dangling leg and slide out onto the snow through the broken plexiglass.

First thoughts were thankfulness we had survived but concern about rescue grew. We knew our friends would report us missing and mount a search. Tom tested our ELT; the electronic locator transmitter was working so it could help searchers find us. Using my cellphone that often didn’t work well even in the city, on one bar, after many tries dialing 9-1-1, a voice answered. I didn’t know if my rapid-fire description of our crash with two human survivors and a dog reached the operator before I lost contact and was unable to connect again. It was noon and hours to go before darkness. Valkyrie disappeared at times circling the crash site from a distance, between resting within view above us as if on guard.

To cheer me, Tom chose Valkyrie at a few weeks of age, from a litter of pups born to a German Shepherd mama left at the Humane Society. I was in mourning after the recent death of Valentine, our beloved German Shepherd. Valkyrie was our sixth dog 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.

Offering her a treat for good behavior or performing tricks interested her little. She tested our wits and patience. With help from professional trainer Kristen who suggested little stinky Vienna sausages as treats, Valkyrie improved. But, at the time of the crash, she was still excitable, not food driven and wouldn’t come when called. I worried about her getting lost.

Lying on the windy snowy mountainside trying to shield ourselves took a toll as we waited, hoping for rescue before dark or hypothermia set in. Six hours after the crash, we heard snow machine engines above us and soon, voices 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.)

Rescuers chain-sawed Tom out of the wreckage, stabilized him on a board and hoisted him up in a wire basket with the help of a motorized assist to snow machines on a trail. We both received stellar care and stabilization. Valkyrie was frightened and came to me shivering as I lay on a sled behind a snow machine. I tied the string from my jacket hood to her collar and told the rescuers I wouldn’t leave without her.

They said I wasn’t thinking straight due to hypothermia and would care for her. The noise of a nearby snow machine starting frightened her and she jerked from my grasp, disappearing in the forest. They assured me they’d find her. I had to leave and was soon airlifted by helicopter to St. Pat’s hospital in Missoula.

The next few days were a blur of decisions and activities. Tom in ICU, both of us with numerous surgeries under the care of a skilled trauma team, but heart broken. Valkyrie was missing.

Barb, a close friend and a friend of dog trainer Kristen mounted a search for Valkyrie, notified the newspaper and set out to search for her. The front page news article drew volunteer dog searchers from miles around, many of them carrying cans of Vienna sausages after hearing it was one of few foods that enticed Valkyrie. Barb arranged to have someone come to the hospital and do audio tapes of our voices calling. “Here, Val. Come. Let’s go for a ride. Here, Val. Here little girl.” You get the idea. They played the tape in their cars driving in the vicinity of the crash.

No dog. A few hopeful sightings. Days went by. After a week, Barb and Kristen had to return home and gave the tape to searchers from the local Humane Society, Vara McGarrell and friends took up the search. On the tenth day after Valkyrie’s disappearance, they spotted her about a mile from the crash site near a highway rest stop. Vara’s “bait dog” Chigger was playing with a tennis ball in the parking lot. Valkyrie’s love for balls and other dogs drew her from hiding. Thin and weak, she made her way to visit Chigger.

Vara rushed to her car and turned the tape on high volume, attracting Valkyrie to our voices. She came to the car but was too weak to get in. Vara picked her up, placed her inside and closed the door. Safe. Trapped and very skinny, Valkyrie crept to the front seat and looked under the dash, listening to our voices.

Happy crying rescuers popped open a can of Vienna sausages offering the starving dog some food. A trip to the veterinarian found no unexpected problems, but when Valkyrie’s close friend Barbara arrived, the dog went into such a wail of relief and excitement, the vet techs moved them into a private room until Barb could calm her down.

By that time, I was out of the hospital and on crutches, but Tom remained in ICU. Friends carried the weak dog to visit Tom for a joyful reunion. Vara has become a close friend and parties with us each year at our Crash Survival Celebration.

What do I attest Valkyrie’s long life to? Choosing the right dog and human parents, a strong will, a cheerful attitude, lots of exercise, good food but not too much, and many friends. Just like people, dogs thrive with social involvement and love.

Happy 17^th Birthday to Valkyrie.

 

Thank you to the search and rescue team members from Salmon and Gibbonsville, ID, and to everyone from the Humane Society and surrounding area who helped look for Val, especially her friends Vara, Barb and Kristen.

 

Fatal Feast – A Biological Thriller

A New Publication

Announcing the publication of Fatal Feast on August 30th, a biological thriller set in Montana. I began researching the topic 30 years ago with the outbreak of mad cow disease in Great Britain. Prion disease is an infectious protein currently epidemic in wildlife in Montana, in twenty-five U.S. states and in Canada. This book will be of particular interest to beef eaters and hunters.

Brilliant young researcher Dr. Callie Archer vows to find a cure for an aggressive prion variant of mad cow disease that killed her father. Like unstoppable super-bugs, the deadly proteins infect livestock and wild game threatening world food supplies. Unknowing humans who eat infected meat become paranoid, violent and die horrible deaths.

Federal authorities isolate Dr. Archer’s primate research project at an NIH high-risk laboratory in the mountains of Montana for protection from radical animal rights activists. While she risks her life to stop the catastrophic disease that could prove fatal to millions, a sexist director, sabotaging cohort, and a handsome rancher obstruct her progress.

Dr. Archer closes in on a cure, but murderous activists penetrate her lab, steal infected animals, and nearly kill her. As the disease spreads in ranching and hunting country, authorities suppress public information to save the country from economic disaster.

Callie’s promising treatment may be the only hope to prevent a world-wide pandemic. With forces against her mounting, can she save mankind and herself?

Fatal Feast is available as an e-book and paperback. I would love to have you write an honest review on Amazon.

Thanks for stopping by.

 

Check out my two April posts on prion disease in humans.

Purchase on Amazon

A Tribute to Mom

 

Eight large bookcases in my home support heavy medical texts, tomes of biology and chemistry, writing reference books and evidence of broad reading interests through the years. Some might call me a book hoarder. While sorting and rearranging books, trying my best to part with some for a garage sale, I ran across Modern Medical Counselor. Seeing Mom’s “doctor book” interrupted my progress and prompted this blog.

I sat down to read parts of the book that guided her while raising four daughters. I was impressed with the solid basic health information I found in the book published in 1943. Anatomy, bandaging methods, germ theory, sanitation, pregnancy and mental health issues were meshed with information on normal bodily processes, common illnesses and proper exercise. She consulted the text for natural remedies and medical information to help make decisions before calling our family doctor who often made home visits.

Mom was an avid reader, homemaker, seamstress, baker, canner and self-taught nutritionist. A Betty Crocker cookbook and collection of recipes from her mother and the other farm women in the small community of her childhood provided amazing meals. Dad’s stable income from railroading provided a solid income, but Mom’s high school education and reading gave her added skills to keep us well-fed and healthy.

In the back of the book, she recorded health histories and immunization records on all of us. A photo of Mom over my desk reminds me each day of her love and caring over a 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.

At eighty-nine, her mind and outlook were bright, and her spirit strong. She had much more to live for but doubt she left anything undone. She loved her family dearly and with the help of my sister Bev, they published and distributed a book of favorite recipes requested by her family. After her death, we found beautifully wrapped gifts for her two beloved unborn grandchildren she would never know.

In the end, she died as she lived, surrounded by her family in her home on the shores of a peaceful Minnesota lake. Best wishes on Mother’s Day.

 

Happy Mother’s day to my sisters.

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.

Overview

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.

Diagnosis

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 and 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:

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Disease-Fact-Sheet

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