S6 E2: Don't Eat Those Brains! The Story of Chronic Wasting Disease

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Hi, this is Kelly and this
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Cause of Death one hundred seconds to
Midnight. I'm your host, Jackie Moranti.

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Here we are in season six.
This season is dedicated to wildlife disease

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and concert. I've been trying to
keep the seasons at about eight episodes,

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but this one may run a little
longer. There's a lot to talk about

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when we talk about wildlife disease and
conservation and the way they come together.

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I'll be introducing some things that we
haven't talked about previously. Many of the

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diseases of wildlife are obscured, and
though the symptoms may sound like a virus

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or a bacterial infection, they could
be something completely different. And this brings

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us to today's episode, chronic wasting
disease in servids. It's caused by an

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organism that we haven't talked about before. It's a prion. I'm the first

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to admit that I am terrified of
prions. They're unpredictable, they're incurable,

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and they're fatal. The worst thing
about them is that they take years to

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manifest, so you don't know you
have one until it's too late to do

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much about at it sound terrifying.
Keep listening. We'll begin, of course,

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with ideology and pathology. To start
at the beginning, we have to

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figure out what a prion is.
Prions only consist of proteins. There is

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no enclosed nucleic acid. They share
superficial properties of viruses, such as size

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and infectiousness, but that's as close
to a virus as they get. Prions

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have unique properties that are capable of
causing inherited, spontaneous or infectious disease.

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The prion protein PRP exists in two
confirmations, the normal harmless or cellular form

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PrPC and the pathogenic form prp SC. This form was named after scrapey,

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which is an infectious disease of sheet
I'll get to scrape in a little bit

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when I talk about the history of
chronic wasting disease, and you know there's

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going to be an episode on it
in the future when I talk about diseases

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of livestock. Trust me, the
connection between CWED and scrapey will be apparent

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before you hit stop on your podcast
player. The mechanism of disease for prions

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is very different from bacteria or viruses. It's more like peer pressure than an

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all out invasion, rogue proteins will
bind to their normal relatives and induce them

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to refold into the pathogenic confirmation.
The proteins that were misfolded to begin with

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will eventually subvert all of the normal
proteins. Once this is done, neural

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degeneration and death occur. The gene
that encodes the prion protein, the pr

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NP, can mutate, and this
may result in prions with a very high

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likelihood of misfolding. This is the
cause of hereditary prion disease. Normal prions

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are known to misfold occasionally, and
this is called spontaneous prion disease. Pathogenic

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misfolded proteins form insoluble aggregates known as
amyloids. These are fibrils consisting of protein

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with a high beta sheet content.
These beta sheets are short and form stacks

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that run sideways relative to the long
axis of the fibers. If misfolded prions

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are transmitted to another susceptible host,
the result is infectious prion disease. This

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is also called transmissible spongiform encephalopathy or
TS. This infection can be spread from

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one cell to another and one animal
to another by entry of the PRP SC

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prion. These prions can also jump
from one species to another. Did I

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mention that prions scare the crap atomy? I'm mentioning that now, right now,

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there is no effective therapy to combat
prion infection. Okay, so now

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you have the down and dirty on
prions. Let's get to chronic wasting disease.

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CWD is highly contagious. As the
infected animals shed infectious prions in feces,

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saliva, and urine, other servids
come along and pick it up.

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It's one of the most easily spread
prion infections. Again, there is no

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treatment or cure for prion infections,
but of course research is ongoing. All

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servid species are susceptible to CWD,
and this disease has spread across wild North

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American servid populations for decades. So
what is a servid? Servid species in

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North America include whitetailed deer, mule
deer or blacktailed deer, l moose,

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caribou, and reindeer. Chronic wasting
disease is a long term illness. Animals

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may remain healthy for a year or
more, but they're still shedding the disease

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and other servits are picking it up. As the disease progresses, animals show

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signs of weight loss, decreased social
interaction with the herd, loss of awareness,

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altered posture, head tremors, and
lack of coordination. CWD affects the

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brain nerves, lymph nodes, spleen, and neuroendocrin tissues. Diagnosis requires laboratory

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testing of the lymph nodes and brain
stem, and these samples must be collected

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postmortem. Live animal techniques do exist, but they're difficult to perform and they

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have a very low success rate.
We don't really know what effect CWD has

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on the wild servit population in North
America, as it can take several years

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for the disease to become evident.
Research and modeling studies published in the last

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ten years have shown that declines in
population are between ten and twenty one percent

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in several deer and elk populations.
This includes herds in Wyoming, Colorado,

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and Wisconsin. Okay, this is
one of those times when it's better to

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go back to the beginning and move
forward. You have the basic mechanism of

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disease, so that's a good start. The classic origin story of chronic wasting

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disease is that it originated at my
alma mater, Colorado State University. This

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story is now being disputed by researchers, but you'll have to wait for that

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part. Researchers were doing nutritional studies
on sheep, goats, and deer.

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Jene Schollenveld, a researcher at Colorado
State, suspects that some of the sheep

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had scrapeeet and the deer in the
experiment became infected. The prion then jumped

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after mutation and became what we call
chronic wasting disease. The deer and sheep

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were kept in the same pen from
nineteen sixty seven to nineteen seventy one.

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None of the animals in the experiment
ever showed symptoms of prion disease, and

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when the experiment was over, the
deer were simply released back into the wild.

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The sheep that he used came from
a scrapy experiment, but none of

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them ever showed symptoms of having the
disease. The first cases were reported after

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this experiment ended, in captive deer
that were being held on the research farm

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in Fort Collins, Colorado. Wild
populations didn't start showing symptoms until the late

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seventies and This was believed to be
malnutrition. The first confirmed diagnosis wasn't made

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until nineteen eighty one. In an
elk in Rocky Mountain Nation Park. In

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the mid eighties, researchers decided that
they were going to get rid of CWD.

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They gathered all the captives servits and
they killed them. They cleaned the

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pens and turned the soil, and
brought a helicopter in to drop chlorine onto

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the site. They didn't put animals
back in those pens for a full year

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after that. Then they brought in
healthy wild elk calves. Within two years,

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chronic wasting disease was back and the
herd was decimated. Michael Miller and

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his colleagues had failed. In an
interview with NPR, he said, quote,

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the idea at the time was that
we would do what we needed to

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do here locally to keep it from
spreading to the western slope. What we

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didn't realize is that it was actually
more widespread. It was a really nice

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idea that was probably ten, fifteen, maybe twenty years too late. End

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quote. Mule deer were then diagnosed
with the disease in both Colorado and Wyoming

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by the mid nineteen nineties, it
had spread to captive herds in Saskatchewan,

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Canada. By the year two thousand, it had spread to Nebraska, Oklahoma,

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and wild herds in Saskatchewan. In
two thousand and one, CWD was

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identified in whitetailed deer in South Dakota
and in a captive herd in Nebraska.

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By two thousand and twelve, it
had spread to Minnesota, Wisconsin, New

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Mexico, Utah, Illinois, Kansas, Virginia, North Dakota, Iowa,

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Pennsylvania, and Texas. It was
found in Ohio in twenty fourteen. In

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two thousand and fifteen, Michigan confirmed
its first case. Researchers began sampling animals

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from five to fifty kilometers outside the
facility, and most of the infections were

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occurring within the five kilometer radius.
Chronic wasting disease has an extended incubation period.

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While the exact time of incubation is
unknown, it's estimated that the priyon

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works its misfolding magic for about six
to eight months before symptoms begin. Then

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the animal can live for up to
two years before finally succumbing to the disease.

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Symptoms are often more subtle in elk
than in deer, but they remain

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the same. The animals may walk
repetitive courses, show in coordination, and

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have a wide based stance. They
may also carry their heads low and stay

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very close to water sources. They
will continue to eat, but as the

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disease progresses, they'll consume less and
less food, and this leads to loss

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of body condition. Essentially, the
animal starves to death. In the final

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state, ages excessive drinking, urination, salivation, and drooling are commonly observed.

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The animal may survive for a few
days to a year once these symptoms

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appear, due to difficulty swallowing aspiration. Pneumonia may be the final cause of

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death in these animals. Hunters will
generally have the animals that they kill tested

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for CWED. Samples are taken from
the lymph nodes near the base of the

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skull and a specific portion of the
brainstone. Hunters don't usually harvest sick animals,

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so when these animals test positive,
they are found in the preclinical stage

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of infection. There have been instances
when people have found dead servits and have

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called fish and wildlife to test them, and these animals normally show signs of

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infection and will test positive for CWD. The prion can also be detected in

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the environment, but testing as difficult
and it isn't as reliable as testing dead

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animals. Eradication of CWED seems unlikely
since the pryon can survive in the environment

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for long periods of time. It
thrives well in soil that contains clay,

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and plants may be able to bind
the prions for uptake and distribution into leaves

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and stems. If this disease remains
unmanaged, affected areas can expand geographically and

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prevalence will grow among the herds that
free range those areas in Wisconsin and Wyoming

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where the prevalence is the highest.
Roughly forty to fifty percent of servids harvested

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and tested were positive for CWD.
Are you a true crime advocate? Are

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checkout for fifteen percent off the ticket
price. Special guests will include Julie Murray,

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Terra Newell, and Call your Landry
to name a few. I'll be

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there representing another podcast that I'm involved
with, True Consequences. Come by and

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00:16:11.360 --> 00:16:18.679
meet Eric Carter, Landein and I
We would love to meet you. The

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known distribution of CWED in captive and
free ranging serviants continues on an upward trajectory.

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However it's underestimated, especially in wild
herds. It's easier to identify CWD

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in captive herds through surveillance and epidemiologic
investigations. As of July twenty nineteen,

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chronic wasting disease has been detected in
one hundred and twelve herds of captive deer

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and elk in seventeen states. Canada
has found one hundred and nine herds in

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four provinces. South Korea imported the
disease in nineteen ninety seven, and it's

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been found in that country in captive
elk, red deer, and sika deer.

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CWED has been found in wild servits
in twenty four states across the US,

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and in Alberta and Saskatchewan in Canada. Norway has a history of chronic

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wasting disease in deer that could be
tracked back to the early nineteen hundreds,

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but the first cases in caribou and
reindeer were found in twenty sixteen. It's

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also been found in wild moose in
Finland and Sweden. There has been considerable

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research done on animals that may come
into contact with the carcasses of servits that

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die from CWED. Testing on wild
carnivores and omnivores have yielded no positive animals.

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This tells us that the prion hasn't
jumped into those species. Cattle that

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were housed with infect and mule deer
for ten years did not develop the disease.

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But then there are pigs. Pigs
seem to be the deal breakers on

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a lot of things. An experiment
on pigs was done to see if they

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could be inoculated orally or inoculated directly
into the brain with material from affected deer.

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When the research was done, it
was found that even though pigs have

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a high resistance to the priyon,
it still may be possible for swine to

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serve as a reservoir for CWD under
natural conditions. That said, there are

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other tss that can be transmitted from
animals to humans, and because the pryon

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could mutate and jump, wildlife management
professionals recommend that hunter's harvesting deer in affected

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areas, meat processors an taxidermists who
handle servid carcasses takes some common sense measures

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to avoid exposure to the pryon and
other pathogens that they may come in contact

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with. These precautions include do not
shoot, handle, or consume any animal

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that is acting abnormally or appears to
be sick. Contact the local Game and

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Fish department to report these animals.
Wear latex or rubber gloves when field dressing

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your harvest. Bone out the meat
from your animal don't saw through bone,

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and avoid cutting through the brain or
the spinal column. Minimize handling of the

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brain and spinal tissues. Wash your
hands the cause of death one hundred seconds

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to midnight mantra, and wash your
instruments thoroughly. After field dressing is completed.

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Instruments, cutting boards, and other
items used for processing should be soaked

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for five minutes in a forty percent
solution of sold bleach to inactivate the prions.

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However, the items must be completely
clean with absolutely no tissue or organic

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manner present for this to be effective. Avoid consuming the brain, spinal cord,

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eyes, spleen, tonsils, and
lymph nodes of harvested animals. Note

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that normal field dressing and boning out
of the carcass will remove most, if

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not all, of these parts,
and cutting away all the fatty tissue will

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remove the remaining lymph nodes. If
you have an animal that has tested positive

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for CWD, don't eat it.
Just don't. Honestly, we don't know

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enough about prions to be sure that
cooking the animal will thoroughly kill them off.

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If you have your animal process commercially, request that your animal be processed

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individually. Ask them not to mix
and match with someone else's harvest. This

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disease will be difficult to control.
First of all, we're dealing with millions

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of wild servids. There are thirty
six million wild deer in the United States

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alone. That's a lot of animals
to control and monitor. Then we have

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the biggest problem. There is no
cure and no vaccine today. Vaccines have

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only prolonged the incubation period. They
haven't provided immunity. There is no treatment

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available. Though several anti virals and
anti malarial drugs have been tested, these

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drugs have had little to no effect
on the disease. Yes, this will

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be problematic. Long incubation periods,
extremely subtle signs of early clinical disease,

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absence of a reliable and practical anti
mortem diagnostic test, environmental contamination with an

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extremely resistant infectious agent, and incomplete
understanding of the transmission combined to put an

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insurmountable wallup when it comes to eradication
or even the control of CWD currently in

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wild populations. Primary prevention strategies include
regulation of herd movement and carcasses. These

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are the highest risk factors for introducing
chronic wasting disease into new areas. In

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captive facilities, management options are limited
to quarantine or elimination of infected animals.

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It remains to be seen whether or
not contaminated environments can ever be completely disinfected.

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Current recommendations for captive facilities include leaving
those pens unused for an extended period

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of time before introducing healthy animals back
into the environment. Free Ranging reservoirs near

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infected captive facilities could also severely impair
attempts at eradication from both populations of animals.

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In twenty fourteen, the USDA implemented
a CWD herd certification program in cooperation

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with state agencies regulating captive servids.
Participation in the program is mandatory for interstate

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shipping of any commercial captive animals.
Participation is voluntary for shooting enclosures, hobby

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farms, and other facilities where animals
will not be transported across state lines.

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The herd Certification program established minimal requirements, such as testing of all on site

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deaths over twelve months of age for
a five year period. This is necessary

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to a choir and maintain herd certification. Unfortunately, certificationism bulletproof an interstate infection

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from certified herds. Has been documented. Some states have taken it upon themselves

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to utter requirements for certification of captive
herds for interstate shipping. In areas where

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CWED may not yet be endemic,
eradication of CWD is possible, but early

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detection is key to the goal.
Eradication attempts are now underway in an isolated

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mountainous region in Norway where CWED was
detected in wild reindeer in twenty sixteen.

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The prevalence in the herd was approximately
one percent, and Norwegian authorities believed that

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CWED was introduced recently, was detected
early, and eradication was feasible. The

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entire herd of approximately two thousand animals
was destroyed between twenty sixteen and twenty eighteen,

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and wild reindeer will not be introduced
into the area for several years.

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The mountainous terrain will keep the other
herds from immigrating into the area, so

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when reindeer are reintroduced, it will
be with the help of humans sustain Selective

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culling of deer in one of the
CWD affected areas of northern Illinois has successfully

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limited the prevalence and slowed the expansion
of the disease. States conduct long term,

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active surveillance programs to monitor CWD distribution
and prevalence in endemic areas, to

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determine the extent of the affected area, and to evaluate the effects of disease

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management efforts. In some areas,
supplemental feed minerals or baiting may be banned

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or regulated to limit the range expansion
of the disease and decreased transmission. If

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we're being honest, selective culling was
practiced throughout the original endemic areas of Colorado

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and Wyoming for many years, and
that approach proved insufficient to reduce the prevalence

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of the disease on a herd level. In those areas which have a high

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number of infected animals, an experimental
enzyme that was developed at the University of

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Nebraska Lincoln and Creighton University is commercially
available. Pri enzyme would be effective in

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degrading CWED prions in the soil,
and this could help limit the indirect disease

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transmission to deer end elk. In
twenty nineteen, Congress expressed a renewed interest

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in CWED. Legislation was developed in
the House of Representatives and the Senate to

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fund man management and research for CWED. Possible topics included evaluating the use of

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dogs to detect CWD, exploring targeted
sex and age class, removal of deer

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to reduce the spread and prevalence of
CWD, and characterizing and mapping CWD pryon

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strains across the US. Mark Zabel, who was one of my professors at

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CSU and has studied prions for years, has mewsed that fire could be the

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solution to prion disease in the soil. I've included some of doctor Zabel's papers

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in the show notes. If you're
interested in pryon's go check them out.

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Doctor Zabel is now thinking that the
disease didn't begin in the Mountain West,

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he told MPR. Quote most of
the outbreaks in the US can be traced

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back to movement of animals on the
game farms from the front rank age to

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places like Saskatchewan in Canada, to
the Midwest and Wisconsin to South Dakota repopulation

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in Arkansas. But then there are
some that have no known connection end quote.

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The prime example was the outbreak in
twenty sixteen in Norway, then Finland

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and Sweden. Those animals had absolutely
no connection with any of the herds in

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the US or Canada. This brings
us to the hypothesis there may be many

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different strains of CWED, and those
strains could be emerging at different times worldwide.

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Zabel and his colleague Caitlin Wagner,
a PhD candidate in microbiology at CSU,

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are looking into the possibility by comparing
prions from infected deer in Texas with

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infected elk in Colorado. They're also
comparing deer and elk from seven other dates.

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Wagner explained to NPR, quote,
if they are different, this would

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suggest that we have different strain proper, which is evidence we're building our case

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that we may have multiple strains of
CWD circulating in the US end quote.

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The first step is to see if
a chemical called guandine destroys both samples with

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relative ease. We must remember that
the shape of a prion dictates everything about

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it, including the way it interacts
with animal cells and the ease with which

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chemicals can unfold the protein. In
the experiment that was observed by NPR,

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the prions from the Texas deer were
a lot harder to destroy than the Colorado

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elk strongly suggesting that Zabel and Wagner
were looking at two different strains of the

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disease. This research is still in
its infancy, so many more experi aramons

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must be done, but it's one
step closer to evaluating the disease and explaining

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some of the gaps in the original
story. Doctor Zabel ended the interview with

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a grave warning quote, if it's
still evolving, it may still evolve into

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a form that could potentially eventually affect
humans. End quote. That's some scary

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food for thought. I'm not sure
that chronic wasting disease will ever be under

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control, not without some very drastic
measures. If we let it take its

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course, the disease will spread and
possibly jump to domestic animals then people.

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If we eradicate entire herds, that
could be horrible for the environment. Hunters

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would have a fit, and so
would many animal welfare groups. It's a

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very sticky situation and there are no
easy answers. I do encourage you to

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read the show notes if you're interested
in chronic wasting disease. There are papers

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in there from several researchers, government
documents from the Department of Interior, news

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articles and so much more. Much
of this is repetitive, but it's nice

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to see that several groups are in
league to eradicate this disease that's so harmful

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to serve its. That wraps up
another episode of Cause of Death one hundred

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seconds to midnight. Please feel free
to contact me on the socials links to

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00:31:40.359 --> 00:31:42.720
all the places I can be found, or in the show notes. There

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00:31:42.799 --> 00:31:47.880
is also a link to my Patreon
page where you can sign up for ad

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00:31:47.920 --> 00:31:52.759
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bonus content. Stop by the websites
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for some great deals on some fantastic
products. I asked my followers on social

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00:32:10.640 --> 00:32:15.000
media to pick the episode for next
time, so I'll see you in two

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00:32:15.000 --> 00:32:21.240
weeks when I'll talk about chemical spills
and the effects they have on wildlife.

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00:32:22.880 --> 00:32:29.440
Everyone from railroads to trucking companies to
oil companies is going to get scrutinized with

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00:32:29.480 --> 00:32:34.759
that one. You will not want
to miss it until then. Don't eat

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00:32:34.799 --> 00:34:39.960
any deer brains