What these strange conditions, including fatal familial insomnia, kuru, scrapie, and mad cow disease, share is their cause: prions. Prions are ordinary proteins that sometimes "go wrong", resulting in neurological illnesses that are always fatal. Even more mysterious and frightening, prions are almost impossible to destroy because they are not alive and have no DNA. And the diseases they bring are now spreading around the world.
In The Family That Couldn't Sleep, essayist and journalist D. T. Max tells the spellbinding story of the prion's hidden past and deadly future. Through exclusive interviews and original archival research, Max explains this story's connection to human greed and ambition, from the Prussian chemist Justus von Liebig, who made cattle meatier by feeding them the flesh of other cows, to New Guinean natives whose custom of eating the brains of the dead nearly wiped them out.
The biologists who have investigated these afflictions are just as extraordinary. They include Daniel Carleton Gajdusek, a self-described "pedagogic pedophiliac pediatrician" who cracked kuru and won the Nobel Prize, and another Nobel winner, Stanley Prusiner, a driven, feared self-promoter who identified the key protein that revolutionized prion study.
©2006 D.T. Max; (P)2006 Tantor Media, Inc.
"Very timely and compellingly written." (Booklist)
The title of this book and the medical mystery it implied intrigued me and I was excited to listen to it. It wasn’t long before I felt that the title of the book was a misnomer. I would be surprised if there was one hour of cumulative time on the discussion of the Italian family who “couldn’t sleep”. A more apt title would be something like, Prion Disease: A History of Discovery in Animals and Humans. But then, who's going to read that?
The book dealt more with the study of prions—the smallest known infectious agent which is a naturally occurring protein molecule that lacks nucleic acid. It is these prions that are at the root of this family’s illness called Fatal Family Insomnia (FFI). It starts with sweating and constricted pupils the size of pin points and ultimately prevents family members with FFI from sleeping, leading to death. Members of this family have a 50/50 chance of passing this hereditary illness on to their offspring.
The majority of the book discusses the history and pathology of such prion-related diseases such as mad cow disease in cows, scrapies in sheep, kuru in humans (cannibal-related), Creutzfeldt-Jakob syndrome and Alzheimer’s and NOT the subject of the book as titled.
Everyone knows how horrible Alzheimer’s is with the loss of mind that accompanies it. Just imagine how awful it would be to have Alzheimer’s but you still know what is happening to you. That is what it is like for this family. FFI is horrendous and rare (only 40 families in the world have it) and it is this rarity that prevents the needed money being allocated to it for researching its cure.
It’s a sad family story, but again, and disappointingly so, the "family" is minor player in the saga. It was an okay book if you’re looking for a medical mystery but it was not what I had signed on for. The author had me at title but I still felt mislead on this one from the beginning.
D. T. Max has written a biography of sorts about a family plagued by insominia. The malady remained a mysteria from the 1760s when it was first reported to the 1990s when it was recognized as a genetic disorder. Max, a gifted science writer, empathetically illuminates the story of the afflicted family by linking it to the English mad-cow epidemic, medical and biological theory, and related issues.
Ultimately, I look for books that inform, are well written and well read. This book fills the bill on all three counts. I picked it up for the discipline of learning about topics outside my field of reference. I was not disappointed.
This is an excellent book for those individuals who have diseases for which scientists have not yet found a cure such as Parkinson's disease and any other one caused by the deterioration of proteins. The book is very well-written for the lay person and it is quite entertaining as well.
Thorough and informative, interesting despite the somewhat random topic. Covers the entire gamut of "prion" self-replicating proteins and the damage they have wrought upon both man and beast, and the brains of those unfortunate enough to become afflicted. Narrator is somewhat nasal but carries the narrative well.
The title is a bit misleading. The book is informative, but more about Mad Cow Disease then the actual condition and lives of the family that is afflicted with this disease.
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