The disease that no one and everyone has heard of

It's a funny thing being in a different part of the country. Wildlife stuff that has been widely publicized in the West has barely even made the news here. When people ask me what I am doing here and I tell them I am working on chronic wasting disease, the first question I get is, "What's that?" My usual response is, "You've heard of mad cow disease, right? That's what it is, except in deer." The next question is usually, "Can humans get it?" We don't know the answer to that yet. As yet there is no scientific evidence that it can jump the species (or in this case, genus) barrier, but since there is no evidence either way the World Health Organization recommends that no one consume meat from deer or elk that test positive for the disease.
CWD is caused by a little piece of material known as a proteinaceous infectious particle, or prion. Prions cannot be denatured (unfolded) or lysed (broken up) by anything in the body, so it runs around making tiny holes in the brain. It is part of a group of diseases known as transmissible spongiform encephalopathies (TSEs). We humans have our own forms (Creutzfedt-Jakob disease and kuru), cows get bovine spongiform encephalopathy, sheep get scrapie, and deer, elk, and moose get chronic wasting disease. There are a couple of different ways these are said to proliferate, but to discuss the how, when where, and why of all of them can (and has) fill several books. Suffice it to say there is still a lot we don't know about TSEs, and I'll just focus on CWD and how to avoid it.
The physical symptoms of CWD are an awful lot like several different types of deer diseases, so most wildlife agencies stress that only someone who has been through CWD training should make the call on what ails a deer. Typical symptoms are frequent urination and drinking, excessive salivation, aimless wandering, staring, wide stance and lowered ears, and no flight response at the approach of humans. (Please keep in mind that this is not an all-inclusive list.) All deer that are suspected of having CWD must be killed. Why? Because to test for the disease we have to have the lymph nodes and maybe even the brain in order to make a diagnosis. It might seem inhumane, but I really don't think it is. For one thing, by the time symptoms show up the deer will live only a few days or weeks longer. Besides, considering what is happening I'd rather shoot the deer; it's going to die anyway, and as I mentioned before prions are nearly impossible to kill. Would you rather have the diseased carcass scavenged and scattered to the four winds? That's a pretty darn good proliferation mechanism!
The group that has been affected the most by emerging research (this disease was first discovered in the late 1970s) is hunters. Right now hunters don't have to give their deers' heads up for testing (they want those antlers!), but they should. It's pretty frightening to think that you could end up in a hospital bed dying (and yes, you will die) from deer meat you consumed two years ago--that's about how long it takes for the prion to do enough damage for symptoms to show up. However, there are some pretty simple things hunters can do to avoid such a fate. First and most obvious, let the wildlife department test your deer. It's not as though you can't keep the meat, which is really the point of hunting to begin with. State agencies now recommend that you wear gloves and maybe even a protective smock while field dressing and later butchering the animal. After that, disinfect your instruments in a fifty percent bleach solution. Be careful not to let anything soak in the bleach--it can damage the blades! Not many people are going to eat the deer's brain, but if you've been in that habit I would stop now! You should request results from your deer's tests. A lot of places won't give them to you unless you specifically ask. It seems a waste to get rid of the meat if the test results are positive, but I for one would be very leery of eating meat that might be contaminated with prions. Really, what's the worse fate?
It's perfectly natural, but a little strange that people on the East Coast aren't more aware of this disease. Out here agencies have managed for deer for so many years that the populations have become overabundant and caused other problems (which I will address elsewhere). A lot of people in this area hunt for deer. CWD is mostly confined to northern Colorado, southeastern Wyoming, and the Nebraska panhandle, but a case showed up in Slanesville, West Virginia a few years ago. An isolated case, but as my boss explained, "It's not a question of if it will get here, but when." It's time we start educating people before we find out that in some cases CWD can jump the species barrier like it did with BSE (mad cow disease).