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deadly feasts

14 april 2016

Deadly Feasts is a terrific book, but its timeliness, back in 1997/98, has meant that it holds up less well than Richard Rhodes' truly great scientific/political histories Making of the Atomic Bomb and Dark Sun.

The scientific history in Deadly Feasts is compelling. Starting with the mid-20th-century outbreak of a fatal disease called Kuru, among the Fore people of New Guinea, Rhodes opens up a concise yet complex narrative of scientific understanding of TSEs, "transmissible spongiform encephalopathies," diseases that riddle the brain from within and cannot be reversed. These diseases share features with Alzheimer's and with Parkinson's, but are far scarier in that, as their name suggests, you can catch them, not simply develop them with age. TSEs do not spread in the air or by touch or sexually, but they can spread when people or animals eat compromised material – as the Fore used to, in cannibalism that honored, as it consumed, the dead of their own families.

Scrapie, a disease of sheep, is an animal TSE, but humans appear immune to it. Creutzfeldt-Jakob, a disease that spontaneously affects about one in a million people every year, was the best-known human TSE (that is, not at all well-known) before American physician Carleton Gajdusek and other researchers brought studies of kuru to Western attention. And if you were even minimally clued-in during the 1990s, you know about "variant" Creutzfeldt-Jakob disease and its relation to BSE, a disease of cattle more commonly called "mad cow." When variant-Creutzfeldt-Jakob cases started to appear in England at a far higher rate than one in a million, coincident with an outbreak of BSE among English cattle, the stage was set for fears of a great epidemic.

In fact, writing in 1997, Rhodes imagines worst-case scenarios where a substantial portion of medical efforts in the UK would be diverted to caring for moribund variant-Creutzfeldt-Jakob patients by the years 2015-16. Since this isn't happening, we seem to have dodged an epidemiological bullet. Governments responded to scientific concerns with inertia and half-measures, but they did respond, and potential major outbreaks were curtailed. Still, the long incubation period for these TSEs means that we are not out of the woods yet. I spent so much time in England between 1981 and 1996 that I still can't donate blood in the United States. I did love my steak-and-kidney pie. If my brain turns to mush soon … well, who could tell, the punchline goes.

Rhodes' step-by-step explication of the research that led to connections among a bewildering variety of human and animal diseases is first-rate. As the story nears 1997, of course, the revelations come thicker and faster and mean less and less in the long run. From reading a little on the Internet after finishing Deadly Feasts, all I can really tell is that the mechanisms that cause TSEs and lead to their transmission are still not very well understood. (Or maybe Wikipedia just isn't as well-written as a Richard Rhodes book.)

The anti-hero of Rhodes' book is Nobel laureate Stanley Prusiner, who coined the term "prion" for the infectious agent behind TSEs. When Prusiner did so in 1982 he was basically inferring a new kind of infectious agent from the lack of observations of anything else; Rhodes is somewhat appalled by Prusiner's arrogance in doing so, and some dissenters still think that "prion" is another term for "who the heck knows." But Prusiner's theory has stood the test of time. The basic idea is that mammals produce a kind of protein that, properly folded, seems to do very little to help or harm an individual. When a prion, a misfolded version of that protein, enters their system, it causes these proteins to misfold in turn, eventually destroying that animal's brain.

Gajdusek (himself a Nobel prizewinner) seems to have been impatient with the prion theory, or perhaps simply not to like Prusiner. Gajdusek wondered if TSEs were caused by a kind of chemical crystallization. Normal germs seemed not to be involved: they could never be seen, filters didn't seem to catch them, and most ominously, the TSE agent couldn't be killed by most methods of sterilization, short of soaking in bleach (useful for floors but less so for hamburger). In other words, the TSE agent seemed not to be alive – it had no DNA or RNA, unlike every other disease-causing organism on earth. Prions were halfway between germs and poisons, in a category of their own. Whether that category is "real" or not seems not to matter much anymore, because prions certainly behave for all purposes as if they are real.

Creutzfeldt-Jakob disease arises spontaneously every now and then, but it is not very infectious, and that's probably why I'm sitting here long after my mince-eating decade-and-a-half. As diseases go, it's hideous, and Rhodes gets a lot of mileage out of sheer terror. Your body dissolves into uncontrollable tremors and your mind succumbs to weird dementias – and that's if you're lucky. In the form of kuru, you get all the physical agony but stay lucid throughout, eventually dying of dehydration and starvation because you can't swallow. And at that, those descriptions of death aren't the most horrific parts of Deadly Feasts. The worst for my money is a description of a cornea transplant (131-33) that is bad enough to think about as fairly routine surgery – but in this case resulted in the recipient contracting C-J disease because the donor had died of it.

Rhodes, Richard. Deadly Feasts: The "prion" controversy and the public's health. 1997. New York: Simon & Schuster, 1998. RA 644 .P93R46