Tuesday, December 4, 2007

The Family That Couldn't Sleep - D T Max

Until recently, a "prion" was a bird, a small petrel to be exact. But, in 1982, "prion" gained a new meaning - "proteinaceous infectious particle," rogue proteins that can cause devastating diseases in humans and animals. Prions are proteins that fold, setting off a cascade of folding proteins that destroy surrounding cells. They are not living and cannot be killed by antibiotics or high heat or by any other way we use to treat and contain viruses or bacteria. A book about prion diseases is not going to be a light read, but D.T. Max makes the science comprehensible and the researchers three dimensional, fleshing out their personalities, rivalries and genius.
Max brings the science down to the personal with his study of an Italian family that for generations has been afflicted with "FFI" or fatal familial insomnia. Seemingly at random, family members in each generation have stopped sleeping, begun sweating, become disoriented, agitated, profoundly demented and died. Prions ravage their brains leaving behind plaques and holes. This family's story runs throughout the entire book, giving a glimpse at prion-caused devastation.
Prions first grabbed public attention with mad cow disease, but they've been around for a while. Animal prion diseases include scrapie in sheep and chronic wasting disease (CWD) in wildlife like elk and deer. Humans suffer from BSE (Bovine spongiform encephalopathy - aka mad cow), GSS (Gerstmann-Sträussler syndrome), kuru, and Creutzfeldt-Jakob disease, but as Max implies, these conditions could just be the tip of the iceberg. As he points out, one of the characteristics of prion diseases is the presence of amyloid plaques in the brains of infected individuals - plaques very much like those found in Alzheimer's victims.
Comment: Max shows how modern agricultural practices (breeding and feed) have contributed to the spread of prion diseases in both animals and humans. (Cow "cake" - you don't want to know what's in it!) I give The Family That Couldn't Sleep 4 stars for content and clear, uncomplicated delivery.

1 comment:

Maggie said...

This book just sounds fascinating, Vidalia! I'll order it for the library. ;)