Title: The Family That Couldn’t Sleep
Author: D.T. Max
Date Completed: 2-6
The sub-title to D.T. Max’s first book The Family That Couldn’t Sleep is A Medical Mystery – and it’s every bit that. The family of the title suffers from Fatal Familial Insomnia, a disease passed with deadly results from generation to generation in one Italian family. For decades doctors were unable to identify the disease, which is understandable since FFI is both rare in the extreme and upsets conventional scientific understanding about the nature of disease itself. FFI is one form of the human equivalent of bovine spongiform encephalopathy, more commonly known as mad cow disease.
FFI, BSE, kuru (another related disease that afflicts humans) and scrapie (which afflicts sheep) are all caused by prions – a nasty, infectious protein that is almost impossible to eradicate -- it is resistant to both heat and radiation, for example. And its results are devastating. “The symptoms of FFI are remarkable and grim,” writes Max. The sufferer first begins to sweat profusely, his pupils shrink to pinpricks and he begins to hold his head in an odd, stiff way. Women suddenly enter menopause. Men become impotent. “The sufferer begins to have trouble sleeping and tries compensating with a nap in the afternoon, but to no avail. His blood pressure and pulse have become elevated and his body is in overdrive . . . Their exhaustion is immense, beyond comprehension. Once the sufferer can no longer sleep, a downward progress ensues, as he loses his ability to walk or balance. Perhaps most tragic, the ability to think remains intact . . .”
Death, as the name so obviously suggests, is the inevitable outcome of FFI, as it is with kuru, mad cow and every other prion-based disease. Ultimately, the actions of the prions leave the sufferers’ brain – whether human, cow or sheep – resembling so much Swiss cheese. Much of the mystery has to do with the nature of prions, which are proteins. Proteins are accumulations of ordinary molecules, but are not alive. Typically, science held that infections could be created only by living agents, such as a virus. Prions put the medical and scientific community into a spin.
Besides charting the efforts of the Italian family to identify the disease that has decimated generations after generation (and its struggle to come to terms with the sheer horror of what confronts them), Max traces the investigation into the nature of prions and furnishes a frightening account of the failure of the British government to act in a timely fashion when Mad Cow Disease first surfaced in that country. The Family That Couldn’t Sleep is a riveting, sobering chronicle. Max has written one of today’s genuine horror stories.
Books now read in ’07: 12
Title: Falling Through the Earth
Author: Danielle Trusson
Date Completed: 2-9
Some 58,000 American soldiers died in
Daniel Trussoni is not a sympathetic figure. He is a violent, brooding alcoholic, who cheats on his wife and withholds affection from his children. He is the sort of man who mistakes obstinacy for principle and rationalization for reason. It is remarkable that Danielle Trussoni survived her upbringing and it is a testament to her spirit that she emerged as a bright, creative adult, who becomes both a wife and mother. Falling Through the Earth is an elegant, elegiac book. Its truth will never touch Dan Trussoni, but it is certain that this memoir goes a long way to bring healing and comfort to his gifted daughter.