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

0:01 Hi, this is Kelly and this is Jenna. We're the hosts of ODFM. That's One from Murder. Each week we discuss a true crime murder case and intertwine our unique sense of dark humor. Each episode relates to a word starting with the letter D. The stories we tell are serious and true, our opinions are not, but be warned we don't hold back on the details of the case or our inappropriate comments and colorful language. Join us every Monday on your favorite podcast provider for a new episode of ODFM. You're on a full ride scholarship to Hell. Hey, I'm Gina and I'm Amber, and we are here to bring you the Weird True Crime podcast, where we cover true crime cases that we'll leave you asking yourself, did that really happen.

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1:53 Here we are in season six. This season is dedicated to wildlife disease and concert. I've been trying to keep the seasons at about eight episodes, but this one may run a little longer. There's a lot to talk about when we talk about wildlife disease and conservation and the way they come together. I'll be introducing some things that we haven't talked about previously. Many of the diseases of wildlife are obscured, and though the symptoms may sound like a virus or a bacterial infection, they could be something completely different. And this brings us to today's episode, chronic wasting disease in servids. It's caused by an organism that we haven't talked about before. It's a prion. I'm the first to admit that I am terrified of prions. They're unpredictable, they're incurable, and they're fatal. The worst thing about them is that they take years to manifest, so you don't know you have one until it's too late to do much about at it sound terrifying. Keep listening. We'll begin, of course, with ideology and pathology. To start at the beginning, we have to figure out what a prion is. Prions only consist of proteins. There is no enclosed nucleic acid. They share superficial properties of viruses, such as size and infectiousness, but that's as close to a virus as they get. Prions

3:28 have unique properties that are capable of causing inherited, spontaneous or infectious disease. The prion protein PRP exists in two confirmations, the normal harmless or cellular form PrPC and the pathogenic form prp SC. This form was named after scrapey, which is an infectious disease of sheet I'll get to scrape in a little bit when I talk about the history of chronic wasting disease, and you know there's going to be an episode on it in the future when I talk about diseases of livestock. Trust me, the connection between CWED and scrapey will be apparent before you hit stop on your podcast player. The mechanism of disease for prions is very different from bacteria or viruses. It's more like peer pressure than an all out invasion, rogue proteins will bind to their normal relatives and induce them to refold into the pathogenic confirmation. The proteins that were misfolded to begin with will eventually subvert all of the normal proteins. Once this is done, neural degeneration and death occur. The gene that encodes the prion protein, the pr NP, can mutate, and this may result in prions with a very high likelihood of misfolding. This is the cause of hereditary prion disease. Normal prions

4:59 are known to misfold occasionally, and this is called spontaneous prion disease. Pathogenic misfolded proteins form insoluble aggregates known as amyloids. These are fibrils consisting of protein with a high beta sheet content. These beta sheets are short and form stacks that run sideways relative to the long axis of the fibers. If misfolded prions are transmitted to another susceptible host, the result is infectious prion disease. This is also called transmissible spongiform encephalopathy or TS. This infection can be spread from one cell to another and one animal to another by entry of the PRP SC prion. These prions can also jump from one species to another. Did I mention that prions scare the crap atomy? I'm mentioning that now, right now, there is no effective therapy to combat prion infection. Okay, so now you have the down and dirty on prions. Let's get to chronic wasting disease.

6:16 CWD is highly contagious. As the infected animals shed infectious prions in feces, saliva, and urine, other servids come along and pick it up. It's one of the most easily spread prion infections. Again, there is no treatment or cure for prion infections, but of course research is ongoing. All servid species are susceptible to CWD, and this disease has spread across wild North American servid populations for decades. So what is a servid? Servid species in North America include whitetailed deer, mule deer or blacktailed deer, l moose, caribou, and reindeer. Chronic wasting disease is a long term illness. Animals may remain healthy for a year or more, but they're still shedding the disease and other servits are picking it up. As the disease progresses, animals show signs of weight loss, decreased social interaction with the herd, loss of awareness, altered posture, head tremors, and lack of coordination. CWD affects the brain nerves, lymph nodes, spleen, and neuroendocrin tissues. Diagnosis requires laboratory testing of the lymph nodes and brain stem, and these samples must be collected postmortem. Live animal techniques do exist, but they're difficult to perform and they

7:50 have a very low success rate. We don't really know what effect CWD has on the wild servit population in North America, as it can take several years for the disease to become evident. Research and modeling studies published in the last ten years have shown that declines in population are between ten and twenty one percent in several deer and elk populations. This includes herds in Wyoming, Colorado, and Wisconsin. Okay, this is one of those times when it's better to go back to the beginning and move forward. You have the basic mechanism of disease, so that's a good start. The classic origin story of chronic wasting disease is that it originated at my alma mater, Colorado State University. This story is now being disputed by researchers, but you'll have to wait for that part. Researchers were doing nutritional studies on sheep, goats, and deer.

8:54 Jene Schollenveld, a researcher at Colorado State, suspects that some of the sheep had scrapeeet and the deer in the experiment became infected. The prion then jumped after mutation and became what we call chronic wasting disease. The deer and sheep were kept in the same pen from nineteen sixty seven to nineteen seventy one. None of the animals in the experiment ever showed symptoms of prion disease, and when the experiment was over, the deer were simply released back into the wild. The sheep that he used came from a scrapy experiment, but none of them ever showed symptoms of having the disease. The first cases were reported after this experiment ended, in captive deer that were being held on the research farm in Fort Collins, Colorado. Wild populations didn't start showing symptoms until the late seventies and This was believed to be malnutrition. The first confirmed diagnosis wasn't made until nineteen eighty one. In an elk in Rocky Mountain Nation Park. In the mid eighties, researchers decided that they were going to get rid of CWD.

10:07 They gathered all the captives servits and they killed them. They cleaned the pens and turned the soil, and brought a helicopter in to drop chlorine onto the site. They didn't put animals back in those pens for a full year after that. Then they brought in healthy wild elk calves. Within two years, chronic wasting disease was back and the herd was decimated. Michael Miller and his colleagues had failed. In an interview with NPR, he said, quote, the idea at the time was that we would do what we needed to do here locally to keep it from spreading to the western slope. What we didn't realize is that it was actually more widespread. It was a really nice idea that was probably ten, fifteen, maybe twenty years too late. End quote. Mule deer were then diagnosed with the disease in both Colorado and Wyoming by the mid nineteen nineties, it had spread to captive herds in Saskatchewan, Canada. By the year two thousand, it had spread to Nebraska, Oklahoma, and wild herds in Saskatchewan. In two thousand and one, CWD was identified in whitetailed deer in South Dakota and in a captive herd in Nebraska.

11:31 By two thousand and twelve, it had spread to Minnesota, Wisconsin, New Mexico, Utah, Illinois, Kansas, Virginia, North Dakota, Iowa, Pennsylvania, and Texas. It was found in Ohio in twenty fourteen. In two thousand and fifteen, Michigan confirmed its first case. Researchers began sampling animals from five to fifty kilometers outside the facility, and most of the infections were occurring within the five kilometer radius. Chronic wasting disease has an extended incubation period. While the exact time of incubation is unknown, it's estimated that the priyon works its misfolding magic for about six to eight months before symptoms begin. Then the animal can live for up to two years before finally succumbing to the disease.

12:26 Symptoms are often more subtle in elk than in deer, but they remain the same. The animals may walk repetitive courses, show in coordination, and have a wide based stance. They may also carry their heads low and stay very close to water sources. They will continue to eat, but as the disease progresses, they'll consume less and less food, and this leads to loss of body condition. Essentially, the animal starves to death. In the final state, ages excessive drinking, urination, salivation, and drooling are commonly observed. The animal may survive for a few days to a year once these symptoms appear, due to difficulty swallowing aspiration. Pneumonia may be the final cause of death in these animals. Hunters will generally have the animals that they kill tested for CWED. Samples are taken from the lymph nodes near the base of the skull and a specific portion of the brainstone. Hunters don't usually harvest sick animals, so when these animals test positive, they are found in the preclinical stage of infection. There have been instances when people have found dead servits and have called fish and wildlife to test them, and these animals normally show signs of infection and will test positive for CWD. The prion can also be detected in

13:58 the environment, but testing as difficult and it isn't as reliable as testing dead animals. Eradication of CWED seems unlikely since the pryon can survive in the environment for long periods of time. It thrives well in soil that contains clay, and plants may be able to bind the prions for uptake and distribution into leaves and stems. If this disease remains unmanaged, affected areas can expand geographically and prevalence will grow among the herds that free range those areas in Wisconsin and Wyoming where the prevalence is the highest. Roughly forty to fifty percent of servids harvested and tested were positive for CWD. Are you a true crime advocate? Are you passionate about uncovering the truth and bringing justice to victims? Do you love the paranormal and spooky tales? Then you don't want to miss the True Crime and Paranormal Podcast Festival in Austin, Texas. It'll be going on from August twenty fifth to the twenty seventh. In twenty twenty three, This festival features panel discussions, workshops, live podcasts, all focusing on ethics and advocacy in the true crime sphere. Get your tickets now at www dot Truecrime Podcast Festival

15:30 dot com and join us in Austin for an unforgettable experience. Don't miss out on the chance to connect with other advocates and take your passion for truecrime and paranormal to the next level. Use Code Consequences at checkout for fifteen percent off the ticket price. That's Truecrime Podcast Festival dot Com with promo Code Consequences at checkout for fifteen percent off the ticket price. Special guests will include Julie Murray, Terra Newell, and Call your Landry to name a few. I'll be there representing another podcast that I'm involved with, True Consequences. Come by and meet Eric Carter, Landein and I We would love to meet you. The known distribution of CWED in captive and free ranging serviants continues on an upward trajectory.

16:26 However it's underestimated, especially in wild herds. It's easier to identify CWD in captive herds through surveillance and epidemiologic investigations. As of July twenty nineteen, chronic wasting disease has been detected in one hundred and twelve herds of captive deer and elk in seventeen states. Canada has found one hundred and nine herds in four provinces. South Korea imported the disease in nineteen ninety seven, and it's been found in that country in captive elk, red deer, and sika deer. CWED has been found in wild servits in twenty four states across the US, and in Alberta and Saskatchewan in Canada. Norway has a history of chronic wasting disease in deer that could be tracked back to the early nineteen hundreds, but the first cases in caribou and reindeer were found in twenty sixteen. It's also been found in wild moose in Finland and Sweden. There has been considerable research done on animals that may come into contact with the carcasses of servits that die from CWED. Testing on wild carnivores and omnivores have yielded no positive animals.

17:52 This tells us that the prion hasn't jumped into those species. Cattle that were housed with infect and mule deer for ten years did not develop the disease. But then there are pigs. Pigs seem to be the deal breakers on a lot of things. An experiment on pigs was done to see if they could be inoculated orally or inoculated directly into the brain with material from affected deer. When the research was done, it was found that even though pigs have a high resistance to the priyon, it still may be possible for swine to serve as a reservoir for CWD under natural conditions. That said, there are other tss that can be transmitted from animals to humans, and because the pryon could mutate and jump, wildlife management professionals recommend that hunter's harvesting deer in affected areas, meat processors an taxidermists who handle servid carcasses takes some common sense measures to avoid exposure to the pryon and other pathogens that they may come in contact with. These precautions include do not shoot, handle, or consume any animal that is acting abnormally or appears to be sick. Contact the local Game and Fish department to report these animals. Wear latex or rubber gloves when field dressing

19:22 your harvest. Bone out the meat from your animal don't saw through bone, and avoid cutting through the brain or the spinal column. Minimize handling of the brain and spinal tissues. Wash your hands the cause of death one hundred seconds to midnight mantra, and wash your instruments thoroughly. After field dressing is completed. Instruments, cutting boards, and other items used for processing should be soaked for five minutes in a forty percent solution of sold bleach to inactivate the prions.

20:03 However, the items must be completely clean with absolutely no tissue or organic manner present for this to be effective. Avoid consuming the brain, spinal cord, eyes, spleen, tonsils, and lymph nodes of harvested animals. Note that normal field dressing and boning out of the carcass will remove most, if not all, of these parts, and cutting away all the fatty tissue will remove the remaining lymph nodes. If you have an animal that has tested positive for CWD, don't eat it. Just don't. Honestly, we don't know enough about prions to be sure that cooking the animal will thoroughly kill them off.

20:51 If you have your animal process commercially, request that your animal be processed individually. Ask them not to mix and match with someone else's harvest. This disease will be difficult to control. First of all, we're dealing with millions of wild servids. There are thirty six million wild deer in the United States alone. That's a lot of animals to control and monitor. Then we have the biggest problem. There is no cure and no vaccine today. Vaccines have only prolonged the incubation period. They haven't provided immunity. There is no treatment available. Though several anti virals and anti malarial drugs have been tested, these drugs have had little to no effect on the disease. Yes, this will be problematic. Long incubation periods, extremely subtle signs of early clinical disease, absence of a reliable and practical anti mortem diagnostic test, environmental contamination with an extremely resistant infectious agent, and incomplete understanding of the transmission combined to put an insurmountable wallup when it comes to eradication or even the control of CWD currently in wild populations. Primary prevention strategies include regulation of herd movement and carcasses. These

22:27 are the highest risk factors for introducing chronic wasting disease into new areas. In captive facilities, management options are limited to quarantine or elimination of infected animals. It remains to be seen whether or not contaminated environments can ever be completely disinfected. Current recommendations for captive facilities include leaving those pens unused for an extended period of time before introducing healthy animals back into the environment. Free Ranging reservoirs near infected captive facilities could also severely impair attempts at eradication from both populations of animals.

23:15 In twenty fourteen, the USDA implemented a CWD herd certification program in cooperation with state agencies regulating captive servids. Participation in the program is mandatory for interstate shipping of any commercial captive animals. Participation is voluntary for shooting enclosures, hobby farms, and other facilities where animals will not be transported across state lines. The herd Certification program established minimal requirements, such as testing of all on site deaths over twelve months of age for a five year period. This is necessary to a choir and maintain herd certification. Unfortunately, certificationism bulletproof an interstate infection from certified herds. Has been documented. Some states have taken it upon themselves to utter requirements for certification of captive herds for interstate shipping. In areas where CWED may not yet be endemic, eradication of CWD is possible, but early detection is key to the goal. Eradication attempts are now underway in an isolated mountainous region in Norway where CWED was detected in wild reindeer in twenty sixteen.

24:42 The prevalence in the herd was approximately one percent, and Norwegian authorities believed that CWED was introduced recently, was detected early, and eradication was feasible. The entire herd of approximately two thousand animals was destroyed between twenty sixteen and twenty eighteen, and wild reindeer will not be introduced into the area for several years. The mountainous terrain will keep the other herds from immigrating into the area, so when reindeer are reintroduced, it will be with the help of humans sustain Selective culling of deer in one of the CWD affected areas of northern Illinois has successfully limited the prevalence and slowed the expansion of the disease. States conduct long term, active surveillance programs to monitor CWD distribution and prevalence in endemic areas, to determine the extent of the affected area, and to evaluate the effects of disease management efforts. In some areas, supplemental feed minerals or baiting may be banned or regulated to limit the range expansion of the disease and decreased transmission. If we're being honest, selective culling was practiced throughout the original endemic areas of Colorado and Wyoming for many years, and that approach proved insufficient to reduce the prevalence

26:15 of the disease on a herd level. In those areas which have a high number of infected animals, an experimental enzyme that was developed at the University of Nebraska Lincoln and Creighton University is commercially available. Pri enzyme would be effective in degrading CWED prions in the soil, and this could help limit the indirect disease transmission to deer end elk. In twenty nineteen, Congress expressed a renewed interest in CWED. Legislation was developed in the House of Representatives and the Senate to fund man management and research for CWED. Possible topics included evaluating the use of dogs to detect CWD, exploring targeted sex and age class, removal of deer to reduce the spread and prevalence of CWD, and characterizing and mapping CWD pryon strains across the US. Mark Zabel, who was one of my professors at CSU and has studied prions for years, has mewsed that fire could be the solution to prion disease in the soil. I've included some of doctor Zabel's papers in the show notes. If you're interested in pryon's go check them out.

27:47 Doctor Zabel is now thinking that the disease didn't begin in the Mountain West, he told MPR. Quote most of the outbreaks in the US can be traced back to movement of animals on the game farms from the front rank age to places like Saskatchewan in Canada, to the Midwest and Wisconsin to South Dakota repopulation in Arkansas. But then there are some that have no known connection end quote. The prime example was the outbreak in twenty sixteen in Norway, then Finland and Sweden. Those animals had absolutely no connection with any of the herds in the US or Canada. This brings us to the hypothesis there may be many different strains of CWED, and those strains could be emerging at different times worldwide.

28:41 Zabel and his colleague Caitlin Wagner, a PhD candidate in microbiology at CSU, are looking into the possibility by comparing prions from infected deer in Texas with infected elk in Colorado. They're also comparing deer and elk from seven other dates. Wagner explained to NPR, quote, if they are different, this would suggest that we have different strain proper, which is evidence we're building our case that we may have multiple strains of CWD circulating in the US end quote. The first step is to see if a chemical called guandine destroys both samples with relative ease. We must remember that the shape of a prion dictates everything about it, including the way it interacts with animal cells and the ease with which chemicals can unfold the protein. In the experiment that was observed by NPR, the prions from the Texas deer were a lot harder to destroy than the Colorado elk strongly suggesting that Zabel and Wagner were looking at two different strains of the disease. This research is still in its infancy, so many more experi aramons must be done, but it's one step closer to evaluating the disease and explaining some of the gaps in the original story. Doctor Zabel ended the interview with a grave warning quote, if it's still evolving, it may still evolve into

30:18 a form that could potentially eventually affect humans. End quote. That's some scary food for thought. I'm not sure that chronic wasting disease will ever be under control, not without some very drastic measures. If we let it take its course, the disease will spread and possibly jump to domestic animals then people. If we eradicate entire herds, that could be horrible for the environment. Hunters would have a fit, and so would many animal welfare groups. It's a very sticky situation and there are no easy answers. I do encourage you to read the show notes if you're interested in chronic wasting disease. There are papers in there from several researchers, government documents from the Department of Interior, news articles and so much more. Much of this is repetitive, but it's nice to see that several groups are in league to eradicate this disease that's so harmful to serve its. That wraps up another episode of Cause of Death one hundred seconds to midnight. Please feel free to contact me on the socials links to all the places I can be found, or in the show notes. There is also a link to my Patreon page where you can sign up for ad free episodes, bonus content, and other perks for as little as a dollar

31:52 a month. Don't forget Apples subscriptions where you can also get AD free and bonus content. Stop by the websites for my awesome sponsors and use bonus codes for some great deals on some fantastic products. I asked my followers on social media to pick the episode for next time, so I'll see you in two weeks when I'll talk about chemical spills and the effects they have on wildlife. Everyone from railroads to trucking companies to oil companies is going to get scrutinized with that one. You will not want to miss it until then. Don't eat any deer brains