Then something very strange happened (even stranger than cows eating sheep): the cows started dying, struck down insane with swiss-cheese-holes in their brains, something the veterinarians called Bovine Spongiform Encephalopathy (BSE), commonly known as Mad Cow Disease (MCD). To compress the timeline a little, then something very scary happened: the British people started dying, struck down insane with swiss-cheese-holes in their brains, something the doctors called variant Creutzfeldt-Jakob disease or vCJD. A typically British discussion ensued about whether or not scrapie and BSE and vCJD could possibly be related. Duh.
In a fascinating scientific detective story I’ve been following for years which could not possibly be adequately covered in a single SFT paragraph, in fact worthy of a two-part C.S.I., it turns out that sheep scrapie is even more mysterious than anybody ever believed. It’s not caused by a bacterium or virus or any other previously known infectious agent. Nope, it’s caused by a prion – a new name given to a type of warped protein that amazingly has the ability to warp other proteins into its deadly brain-wrecking shape. To make a long story short the sheep prion for scrapie when eaten by cows causes BSE, and when eaten in turn by humans causes vCJD. Prions have stretched cherished scientific beliefs in a lot of ways because they are NOT inactivated by normal heat sterilization procedures like backyard grilling and they take a VERY long time to warp enough of their sister proteins to yield their deadly symptoms. Getting to the bottom of the weird experimental data such characteristics of prions generate in a lab required a lot of the same brash, dogged determination shown by American James Watson discovering DNA, exhibited this time around by American Dr. Stanley B. Prusiner who, shall we say, ruffled a lot of feathers on his way to a Nobel Prize.
While they were clapping politely for Dr. Prusiner in Stockholm, all hell was breaking loose in England. First the British government denied there was any problem at all, and even televised the British Minister of Agriculture feeding his daughter a backyard-grilled hamburger. (D’oh!) But Brits kept dying of vCJD and eventually an international embargo on British beef was levied around the world. OK, said the British government, maybe there IS a problem after all, so let’s kill all our cattle herds and burn them in pits. This breathtaking political turnaround resulted in the biggest barbeque in history (even by Texas standards) with 1,300,000+ British cattle put in a trench and put to the torch. There was no small government scrutiny of the whole mess.
Once prion transmission of scrapie/BSE/vCJD became accepted, the question on everybody’s mind in England was ultimately just how many vCJD deaths were going to occur, and when. There was a feeling in everybody’s gut that it could be really bad. There wasn’t a single case of BSE in British cattle before 1986, when they (unknowingly) began eating ground-up sheep. In the 15 years it took to recognize that an epidemic was underway and get it under control in the cattle, almost 200,000 of them had died of BSE. There was fear of a similar stunning levels of human casualties once the human infectees who had eaten ground-up cow began to exhibit their disease. The Lancet report released this week is an official overview of the vCJD deaths so far. The British vCJD death toll was 28 in 2000; 20 in 2001 and 17 in 2002. “That … is encouraging. However, to conclude that the epidemic is in permanent decline would be premature,” said Robert Will, a researcher at the CJD Surveillance Unit. New forecasts predict that as few as 10 additional Britons and as many as 7,000 could get the illness by 2080. This is bad, but previous predictions from only a couple of years ago ranged as high as 135,000+.