The apocalypse is here for North American cervids. It arrived no fewer than five decades ago and its progress has been… slow. Make no mistake, the zombie deer are here. As their range extends throughout North America, it’s become increasingly likely that you’ve heard something about them. It’s 2019, though, and evidence of impending doom falls at our door every day. Are “zombie deer” really a threat?
To understand our risk, we should first understand the affliction itself. What some media sources are calling the “zombie deer disease” isn’t particularly unique to deer or the current year. Sure, the disease itself, Chronic Wasting Disease is limited to deer and their close relatives in the wild (so far), but its kin are not. Chronic Wasting Disease is a prion disorder, a complex and necessarily terrifying concept.
Prion disorders aren’t transmitted through viruses or bacteria, and they can’t be reliably targeted and removed like some cancers. Prion disorders start at the cellular level with, would you believe it, prions. Again, these aren’t out-of-body invaders. They’re more insidious than that, brewed from within. Prions are proteins, the hard workers of the cellular system. They’re a lot more than that, but to explain better, I’d have to learn what they actually do, and that’d be time consuming and hard. I’m passing the savings onto you, really. A prion is a misfolded protein, one whose structure differs from what it should be. You could say it’s “a little different” from the other kids, but you shouldn’t, because it’s not a kid, it’s a prion, and it’s here to fuck up your day.
A misfolded protein isn’t just a little different. Proteins are folded the way they are to complete specific tasks, and if they’re folded incorrectly, they can’t complete that task. One rogue protein might not ruin your day, your body has plenty more. How many more? Science says a lot. The (additional) problem with prions, the bad boys terrorists of the cellular system, is that they replicate themselves. In replicating, prions don’t simply multiply, but instead replace functional prions with their own clones. God creating Adam in his own quirky image.
This is where destruction starts: at conception, essentially. Once the prions appear and start multiplying, their path is, for the most part, unobstructable. Think of slowly overwriting a hard drive with corrupted files, if you’re a nerd. Except, dependent on your definition of speed (nerd), it’s not a slow overwriting process. It’s pretty quick. Once prion disorder symptoms start showing up, we’re talking months on the low end to a couple years on the optimistic. By the time the muscular difficulties, speech trouble, and rapidly-progressing dementia set in, it’s a fast trot to the end.
So prion disorders suck. Pour one out for the deer of America, right? Weeeeeeell… it’s a little deeper than that. Chronic Wasting Disease is, in the wild, so far limited to deer. In laboratory conditions maintained by the scientific sadists of the world, it’s been successfully transferred to all sorts of species, including great apes (note: that’s us, sorta). Even if the deer can’t turn us into servants of the night, though, the situation is still more complex. CWD is only for deer (ish), but prion disorders are a little more liberal in their distribution. If you’ve ever heard of folks going mad over Mad Cow Disease, it’s for good reason. Mad Cow Disease, or, Bovine Spongiform Encephalopathy, if you want to sound like a “real” medical condition with “real” problems, is a prion disorder found most commonly in cattle. Unlike CWD (so far) though, BSE/MCD/AOL has made the jump to humans who eat infected beef.
In humans, Bovine Spongiform Encephalopathy manifests as Variant Creutzfeldt-Jakobs disease, (as opposed to normal Creutzfelft-Jakobs disease, its more vanilla, inherited cousin). Prions can exist anywhere in the body, but they’re especially plentiful in the brain and spinal cord. So while eating contaminated beef is a big nasty no-no, ingesting infected brain matter is a death sentence. Actually. Most people who develop vCJD don’t live much longer than a year past diagnosis. For normal CJD, you’d be lucky to make it that far. As the prions replicate and rewrite the hard-wiring of your brain, they’ll slowly (or quickly, comparatively) eat away at the rest of you until, together, you reach the definite conclusion predestined for the start. There is no cure for prion disorders. There is no protective vaccine. Once you have one, there is no way to slow the disorder’s progress. There is no way to mitigate its symptoms or effects. Doctors’ best advice? Enjoy life while you can.
The CJD family is the most frequent cabal of prion disorders in humans, but it’s definitely not the only one. There’s also Fatal Insomnia, a disease that lives up to its terrifying name (imagine being unable to sleep one night, then the next night, then every night until you die) and Kuru, a morbidly fascinating case endemic to a limited tribal group in Papua New Guinea. The disease itself is marked by a sort of uniform shaking and physical withering until there’s little left to wither. It’s not too unbelievably different from its sister maladies, but by the time scientists were able to diagnose a neurological basis for the problem, locals had known it for decades, with the first case potentially going as far back as the 1910s. Some had deduced that witchcraft was to blame.
Perhaps the strangest factor in the quest to identify the man/cadre of witches behind the curtain of Kuru was that the disease seemed to impact women and children exclusively. It wouldn’t be until decades after the disease’s first appearance when scientists finally discovered its true cause was witches. What a spooky ending.
The real answer is arguably more interesting. The Fore people, the folks at the center of the New Guinean Kuru outbreak, practice (or have practiced — I don’t know what’s trending right now) funerary cannibalism, meaning that members of the tribe ritually feast on their dead. Western moral concerns (of which I can only think of “the taste”) aside, the obvious problem should already be on (and ideally not in) your mind: our friends the prions. Additionally, while all members of the tribe take part in the cannibalistic rites, tissues from the brain and spinal cord are traditionally given to the women and children, who thereafter demonstrate the weight-loss benefits of the All-Brain Diet.
So what’s all this horror for? Is this the apocalypse for humans? Best guess right now is “probably not”, though anything’s possible. At this rate, though, we’re more likely to die en masse to super flu than we are to spongiform encephalopathy, especially given the known transmission methods. In fact, and hate me for this if you will, the apocalypse probably isn’t here for deer either. Chronic Wasting Disease is spreading, but it’s been spreading for a while. The increase in alarm levels across the internet probably owe more to the phrase “zombie deer” than the affliction itself. Indeed, in humans, prion disorders are rare. The National Institutes of Health project an incidence rate of about one person per million per year, or around 350 annually. And, sure, that number’s considerable, but it’s less the thrilling and unique conditions laid out here and more a few other disorders with three-letter names that attack older folks and coincide with dementia. As if things weren’t bad enough.
So what can you do to avoid contracting a prion disorder? The biggest thing is staying away from infected meat, particularly brain and spinal cord tissue. No burger is worth immediate neurodegeneration, much less a brain burger. Let’s stick to conventional cuts, y’all. No one wants to hear their friend was killed by something that “tastes like chicken”. Keep in mind, too, that prions are proteins, not lifeforms, so cooking your vertebrae before serving with assorted sauces won’t do anything to kill the disease. Beyond that, though, there’s not much you can do. Find out if you have a family predisposition to prion disorders, or don’t. Again, like other neurodegenerative conditions, most inherent prion diseases don’t attack until the later stages of life. For some, that’s late enough to render decades of worry wasted time. As with all terrifying maladies, though, maybe the message here is to enjoy the life and health you have while you still have it.
Anyway, wasn’t this about deer?
Happy Halloween, y’all.
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(Originally published on bengrapevine.com on October 29, 2019, months before the non-zombie pandemic)