Review by Martha Quillen
Chronic Wasting Disease – April 2007 – Colorado Central Magazine
The Family That Couldn’t Sleep – A Medical Mystery
by D.T. Max
Published in 2006 by Random House
ISBN: 1-4000-6245-4
HERE’S A BOOK about an Italian family that suffers from a mysterious illness, which apparently strikes only them, and nobody else, anywhere, which makes it seem like an unusual book for Colorado Central’s “From, For and About” section. But there’s a lot of interesting material in this book, which will, in all likelihood, alternately provide Colorado readers with shivers, worry, and paranoia along with some comfort and reassurance.
The Family That Couldn’t Sleep is about a Venetian family afflicted with Fatal Familial Insomnia (FFI). FFI causes sweating, shivering, chronic sleeplessness, hallucination, coma and death, and has been around for centuries. But until recently, it was too rare to receive much medical attention.
Now, however, some very prestigious researchers have added FFI to their field of study — because FFI is thought to be a prion disorder. And after mad cow disease spread through Britain’s dairy herds in epidemic proportion infecting an estimated 800,000 cattle and 160 humans (as of 2006), such disorders have gotten more attention.
Before reading this book, I read a dozen or so reviews about it, and everyone seemed to agree that the book was interesting and pretty good, but a few reviewers felt that it was too sensationalistic and alarming. I, on the other hand, figured it would be hard not to scare people with the details of such disorders, after having read a lot about them in an attempt to understand the dangers (or lack thereof) presented by Chronic Wasting Disease, a related disease affecting Colorado’s elk and deer herds.
I first developed an interest in prion disorders because local news stories and press releases didn’t make it clear whether eating infected meat could be dangerous, or whether CWD could potentially threaten Colorado’s cattle herds. The truth is, nobody knows. But descriptions of these brain-destroying disorders are certainly enough to put anyone off of his feed. Statistically, however, such disorders are rare, and far less likely to kill you than the far more common bacterial infections Americans routinely suffer due to contaminated meat and/or vegetables.
And Max’s book says just that.
Presumed prion disorders (experts are not totally in agreement on whether prions, which are proteins run amok rather than viruses, are the causative agent) tend to be scarier than other illnesses, because they’re not only incurable, they’re also amazingly indestructible. When English and French researchers tried to create a scrapie vaccine by killing the infectious agent, they discovered that infected tissue couldn’t be disinfected with formaldehyde. Then researchers found out that these infectious agents which devastate the brains of their victims couldn’t be killed with irradiation. And after years of research D. R. Wilson, of Scotland’s Moredun Institute, concluded that the scrapie-causing agent could survive “dessication, dosing with chloroform, phenol, and formalin; ultraviolet light; and cooking at 100 degrees centigrade for thirty minutes.”
Max writes, “The scrapie researcher Alan Dickinson told me he remembered Wilson at the end of his career as ‘very, very, very quiet. Of course, that was after his breakdown.’ In the end, Wilson published only one paper, recounting his attempts to destroy the strange virus.”
THESE DISEASES are clearly strange and frightening all on their own. But prion disorders don’t present the biggest threat in Max’s tale of disease and epidemics; people do. In tracking 300 years of brain-wasting disease, Max convincingly shows that the humans in charge of alleviating them repeatedly make the same mistakes, by worrying more about public relations than risks and consistently putting the problems which the disease may cause second to the economic and political repercussions of acknowledging those problems.
“The mad cow story is not a simple one,” Max writes. “It can even be seen as the greatest epidemiological triumph in a century. But while it’s true that British government scientists located the source of the mad cow infection quickly, roughly eight years passed after that discovery before an effective barrier was set up to protect humans from mad cow. It’s as if John Snow had successfully traced cholera to the Broad Street pump only to have the government tell Londoners to go on drawing their water from it while committee after committee studied the problem.”
And Max shows that U.S. officials are just as inclined as the British to postpone action. “In the mid-1990s, more to reassure foreign markets than to calm any anxious Americans, the USDA announced that it would begin testing cattle for mad cow disease.” But. . . .
“The USDA didn’t say how many cows it would test. Roughly 35 million cattle are slaughtered each year in the United States, and, it turned out, the USDA planned to test only about one in a thousand (by comparison, European countries consistently test almost 25 percent of their cows.)”
In the end, Max concludes that mad cow was not particularly communicable to humans and thus the people of Great Britain were very lucky. And we can only hope that people everywhere will continue to be lucky, because the foremost message in this book is that bureaucrats have been mucking things up when faced with problems with their food supply since at least 1772.
AS AN AUTHOR, D.T. Max has considerable talent in bringing this subject alive and in making genetic research both fascinating and understandable. But for the first 100 pages or so, The Family That Couldn’t Sleep bumbles around quite a bit. In relating the tale of Carleton Gajdusek, a Nobel Prize winning biologist who studied kuru, a prion disorder which struck cannibals in Papua New Guinea, Max writes:
He [Joseph Smadel, Gajdusek’s chief supporter at the National Institute of Health in the U.S.] knew that Gajdusek needed the field, where he might make significant discoveries and find romance. He was aware, too, that Gadjusek was as much an ethnographer as a scientist; it was a tendency Smadel was constantly trying to tamp down. “When you go over this,” Smadel wrote to him in 1956, enclosing one of the half dozen papers Gajdusek was working on at the time, “do not add the innumerable conversationally interesting points….Furthermore do not ask for us to include any travelogue pictures in this paper, so what if they live in mud houses or holes in the ground. Remember the old dictum in newspaper writing–if you are in doubt about the pertinence of a paragraph, leave it out.”
And that’s advice that D.T. Max should have taken to heart. Instead, Max seems determined to make enduring characters out of every person who every suffered from FFI; every doctor who ever studied it; every farmer who encountered dizzy sheep in the early days of scrapie infection, or crazed cows in the early days of mad cow disease; and all of the mad cow victims, and their families; and the government officials, and meat inspectors, and veterinary experts….
And developing so many characters is, of course, too tall an order for one book.
But in making the attempt, Max shares some truly fascinating facts and tales — about how scrapie infections were first transported from Spain to Great Britain via Merino Sheep, then spread rapidly and widely due to new breeding ideas; about how prion research evolved; about how the mad cow epidemic gained sway in England; about scrapie, kuru, CJD, and Chronic Wasting Disease; and about the sad state of food safety in the U.S. today (which includes frightening stats about our government’s failure to provide reliable standards and enough factory oversight to reduce contamination by common but deadly bacteria).
The Family That Couldn’t Sleep is a worthy tale, timely, thought-provoking, and never boring. But sometimes you wish that Max would get on with it, rather than side-tracking into descriptions of Venice, New Guinea, and individual researchers.
