PRIONS. WHAT THE...?

When I get tired of worrying about money (which appears to be one of those endless loops anyway, mostly because my basic assumptions may be wrong), I worry about the burgeoning problem of bodily disorders based on the assumption that the world is a continuous sheet of code in time, place and substance. (I also think about theology based on this assumption, but it doesn’t worry me -- in fact, it’s a source of reassurance, a kind of repeated assurance that what is now will always exist and has always existed in some form.)

To come back down to earth (my ex-husband and my best former boss both used to say, “Get your feet on the ground!”), Petunia my neighbor came over to see whether I could find out anything on the computer about what is killing her close friend. At first they said she had a blood clot in her brain and inserted a stent to hold open an artery. Now they are saying she has a disease that starts with a “P.” Petunia, who has macular degeneration so can’t write things down, had learned to spell the word which is short anyway. Prion disease.

Petunia’s husband read her the long and rather technical download I gave her from Wikipedia. They tried to puzzle through it as best they could but she still had questions, which I was pretty hard pressed to address. Anyway, at this point no one could answer them for her. The first time I Googled “prion,” I was still back in Portland in the Nineties and there was almost nothing known about them. This was when the first publicity about “mad cow disease” began to hit.

To put it short and crude, prion disease seems to be a mysterious mis-folding of proteins into something that has stopped being a viable part of the constantly transforming feedback loops of a biological being because it is stuck. It is “polymerazed” into something so stable that burning, radioactivity, standard chemicals, just don’t faze it. In terms of my little wood-burning stove in the garage, it makes “klinkers,” stuff that’s left over after the burning has happened. (I guess actually it’s a coal-burner’s term. In childhood I woke every morning to the sound of our coal furnace having its grate shaken to knock the klinkers down out of the way of a new fire.) Hard ash.

And it’s a hardass kind of problem: mad cow, scrapie in sheep, maybe Alzheimer’s, chronic wasting disease in deer and elk, and other mysterious conditions that show no infectious agent, no organ failure, just a stuck brain. Kuru, the cannibals’ disease from eating human brains, is one of the more colorful versions and challenges the idea that it’s a “modern” disease that comes from our changed environment -- since those tribes were talked out of cannibalism and given can openers.

As it turns out, Petunia’s friend and her family had prided themselves on eating lots of venison and elk, partly because they liked hunting, partly because it helps with the money, and partly because they believed it was healthier. People don’t eat the meat, have symptoms right away, and then fall over like the victims of poison mushrooms or death camas. Rather the malfunctions of little cells accumulate until the whole system hits critical mass and everyone notices that something is very wrong. A healthy immune system cannot get rid of prions. Cooking, very high temperatures, bleach -- all the standard household sanitizing measures -- do not work. It’s even hard to test for the stuff.

In the laboratory there are measures that can finally destabilize the interlocked molecules of the protein and disassemble it. But no one so far can figure out how it works. It doesn’t seem to be “alive” in the way that creatures we know use their nucleus information to manage what a cell does -- a tiny entity that’s much more active than we ever imagined until we developed instruments that could watch it block entrance of some things, open gates to other things, change docking ports on its surface, rearrange structures of storage, metabolism and transformation in internal sub-cell structures.

At the same time as I’m looking up “prion,” I’m getting material via a sort of chain-letter network from people who monitor protected high-tech medical journals and newsletters and translate results for the rest of us. Most recently posts have suggested that bisphenol-A interacts with leptin, causing the metabolic dysfunction that we call Diabetes II. (This stuff is in the linings of cans, so watch out, you folks with can openers.) Another suggests something similar pertaining to the chemicals used in children’s nightclothes to make them flame resistant. There is a host of manmade chemicals associated with polymeres and carbons (which is the base of organic life on this planet) as well as many “tweaked” hormones used as pesticides, herbicides, growth promoters and the like. That is, we have produced many molecules by now that are distributed everywhere in our environment -- like in the breast milk and livers of polar bears -- and that we cannot avoid ingesting. They are just a little bit off, not quite normal.

Many of these are prohibited in other countries, but not here because our political system protects big spenders. (Am I paranoid? Well, I watched “The Constant Gardener” last week, and that would make anyone paranoid. And it didn’t even involve the USA.) Anyway, it’s a fool’s assumption that national borders mean much to chemicals which travel on the wind, in the water, in products -- everywhere. So it’s not ridiculous to assume that Petunia’s friend is dying at the hands of some politician tolerating a desk jockey pushing bench chemists to produce new products while not funding extensive testing or government supervision so as not to interfere with CEO income. And there’s no way to simply abstain.

In China or Russia or other manufacturing-intensive economies, life expectancy is dwindling but incomes are growing. I seem to have this near-unconscious and not particularly rational conviction that to be rich is to be “sick” or otherwise doomed in some way, that it undermines judgment and morality. When the prions finally get to reproductive success (and you know the stories about frogs with weird anomalies as well as I do) then the problem will be solved because there won’t be any more people making new skewed substances. Anything that evolves a way to get rid of prions in their systems will survive and repopulate the planet.

Of course, we can jump the evolution queue by inventing things that un-do prions, breaking up the klinkers. But what it it turns out to dissolve proteins in general? Or some unsuspected keystone protein that brings the whole biological carbon-based unit to dissolution? Maybe I’ll go back to worrying about money. But, you know, it seems to be connected.