The Family That Couldn't Sleep: A Medical Mystery

Nursing Book Club

The Family That Couldn't Sleep: A Medical Mystery

Prions are a point of contention between scientists, but some believe they're responsible for an array of unexplained illnesses, including fatal insomnia.

By D.T. Max; Random House, 2006
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Reviewed By Elizabeth Hanink, RN, BSN

The Family That Couldn't Sleep“He dropped dead from exhaustion.” “I didn’t sleep a wink last night.” Slight exaggerations, wouldn’t you say? Not in the case of some old Italian families. For the past two centuries, various members have been dying from exhaustion and lack of sleep. It is a curse that relatives have hidden from each other and outsiders, while they stood helplessly by and watched loved ones succumb to dementia, ataxia and, finally, death. This grim affliction, fatal familial insomnia (FFI), is one of a group of neurological maladies that have this in common: all of them, scientists believe, are caused by prions.

Prions, short for proteinaceous infectious particle, are misshapen proteins which cause healthy proteins to misfold, fatally clumping together in the brain. Prions cause diseases beyond sleep disorders: Kuru and mad cow disease with its human variant, Creutzfeldt-Jakob, are almost certainly among them. It is possible that what is often diagnosed as Alzheimer’s is also prion-related. All are fatal diseases, not easy to watch, not easy to live with, and because so few people suffer from them now, not easy to study because securing research funding is difficult. (Kuru, it happens, only affects the Fore, aborigines of New Guinea, who have practiced cannibalism.)

That’s not the only problem. Those who do work with these diseases, some of them brilliant and original thinkers, have their own demons. Some have Nobel Prizes, yet do not use their colleague’s terminology or acknowledge each other’s contributions. The cross-disciplinary rivalries and competitions that transcend international borders are endless and block progress in the field. So has institutional indifference. The author of The Family That Couldn’t Sleep, D. T. Max, suffers from an unusual neurological disorder himself. He captures all this contention and more.

Mr. Max is a talented science writer who can make the elusive nature of prions understandable, all while drawing the reader into the medical anthropology of obscure tribes and anonymous Venetian families. Taking no sides in the controversies, he reveals that some very respectable researchers do not believe in prions, or that one disease agent can be genetic, infectious and accidental, all at the same time. His ability to illustrate with words the linkage between scrappie in sheep, mad cow and Creutzfeldt-Jakob makes these otherwise complicated relationships clear. And he acknowledges the pioneer stage at which research now exists. Bureaucratic boondoggles in medicine, government and academia would be comic, were they not so sad.

Mr. Max also asks good questions, not all of them answered. Has our government protected the American public sufficiently from mad cow? Should it do more? Should we do more to protect ourselves? And what about the infectious nature of prions? Are we thinking about it? Is the long-term incubation of these illnesses lulling us into a false sense of security?

Where do nurses fit in? We aren’t, after all, cannibals. Nor are we likely members of the 40 families worldwide who are afflicted by FFI. But we do eat beef; we do care for patients with prion diseases, known and unknown; we do handle reusable medical instruments. If prions are the causative agent of multiple diseases, and there is considerable opinion that they are, we should know about them and understand them.

Elizabeth Hanink, RN, PHN, BSN, is a freelance writer in Los Angeles. She has 30 years of nursing experience.

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