|
|
| The Family That Couldn't Sleep: A Medical Mystery |
|
|---|
|
|
|
| Insomnia Books from Amazon |
 |
|
The Family That Couldn't Sleep: A Medical Mystery
List Price: $15.95
Amazon Price: $9.01
Average Customer Rating:  (24 reviews)
Editorial Review: For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of sheep rub their fleeces raw before collapsing. In England, cows attack their owners in the milking parlors, while in the American West, thousands of deer starve to death in fields full of grass.
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 sprea...
Customer Reviews:
0 of 0 found this review helpful:
 Great read, 2008-06-16
This book is a great book on the history of prions. Max easily illustrates how prions are connected to other important diseases such as alzheimers and diabetes. He flawlessly goes from past to present, connecting the two times with the venetian family who has a defective prion gene. It is really amazing that prions don't affect more people. It is also a wake up call for the beef industry in America.
0 of 0 found this review helpful:
will keep you awake, 2008-03-06
This is a fascinating medical 'thriller', only it's real! it was nearly impossible to stop listening to it and i think anyone who likes medical thrillers or anything related to the medical field, would love this.
The book focuses on prions and their role in disease, especially 'mad cow disease'.
1 of 1 found this review helpful:
A story well told -- and, unfortunately, it's a true one, 2008-01-13
This book does a lot to clear up the story of prions, what they are, what they do, how their threat is real. The Italian family who gives the story its title is but one instance of prions affecting human and animal life. The research is impeccable, and particularly interesting is the process by which medical and veterinary sciences came together to begin unraveling the prion mystery. Because, to be accurate, documentation on how livestock has been affected by prion disease had been, until recently, far more complete and detailed than human prion disease.
The author tells the story unemotionally, which is good, but the reading is far from arid or too technical. The human factor -- how scientists competed for the credit, sometimes damaging other professionals' reputations and careers -- makes it even more interesting. All this makes "The Family That Couldn't Sleep" a fundamental work for anyone who wants to understand these proteins better, and also for people curious about the inner workings of scientific research.
0 of 0 found this review helpful:
It's about time!, 2008-01-10
This is a very scary book. The Family that Couldn't Sleep by D. T. Maxd was a very thought provoking study of some of the neurodegenerative diseases that have eluded our understanding. Most of those that the author mentions are truly horrific to the individual who suffers them and to their families. I started my nursing practice on a neurology ward where I encountered many of the maladies the author describes. What was particularly disturbing to me was that years later many of these insidious diseases are as little understood as they were when I first encountered them. The sufferer of ALS, amyotrophic lateral sclerosis--often referred to as "Lou Gehrig's Disease" after the baseball player who died from it--still finds medical science unable to offer much more than they did when it was first described. Huntington's Disease still devastates families that carry the genetic misprint. While the treatment of myasthenia gravis has progressed to some degree, that of Alzheimer's disease (the old organic brain syndrome or pre-senile dementia) and Creutzfeld-Jacab Disease (formerly referred to as Jacob-Creutzfeld's) are still in their infancy. The similarity between the latter disorder and Kuru has been known for years, but understanding and treatment elude us. According to the author, even the prion concept has its detractors. If nothing else the author was certainly able to capture the devastation that such disorders cause their sufferers and their families. In my early practice I met a man who came in with mild neurological symptoms; he received a diagnosis of Huntington's, and within months he became a changed person because of the unrelenting course of his disease. He ultimately ended up in a nursing home, more or less "insane." Worse yet was the fact that both of his children had a 50-50 chance of having the disorder or of passing the disposition on to their own children. The heartbreak of his wife in witnessing his decline and than recognizing the symptoms anew in her son was awful.
By bringing these disorders and the agonies of the sufferers to public attention Max may well spur more intensive research into these many disorders. And it's about time.
4 of 5 found this review helpful:
 Rogue proteins may keep you up at night., 2008-01-08
You may find yourself staying up all night to finish this fascinating book. Just be glad you don't share the wrong genes with the family of the title.
This account of prion-based spongiform encephelopathic diseases covers a lot of ground: the Italian family of the title suffering from FFI (fatal familial insomnia), the mysterious epidemic of kuru among the Fore tribe of New Guinea, eventually linked to the practice of eating their dead ancestors' brains, the rare genetically transmitted Creuzfeldt-Jacob disease (CJD), various animal spongiform encephelopathies, from scrapie in sheep to mad cow disease to chronic wasting disease in deer. All of these diseases share a common feature - they are transmitted by an infectious agent of a kind thought until recently by scientists to be impossible, and the incubation time from infection to manifestation of disease symptoms is remarkably long. The culprits are *prions*, which are a type of rogue protein. The idea that a protein could act as an infectious agent flew completely in the face of scientific received wisdom to date when first introduced and the science underlying this class of degenerative brain diseases is both complex and controversial.
The author's exposition is clear, but ultimately I think he does not do complete justice to the material (which is really fascinating). It may be that his scope is too ambitious - with so much ground to cover, the exposition occasionally lapses into sketchiness. To be fair, there can be no single "right" level of detail that would suit all readers, and D.T. Max generally shows good judgement about what to include to keep the exposition intelligible while moving his story along.
That said, the material related to kuru, cannibalism among the Fore, and the linkage to scrapie, CJD, and mad cow disease has already been presented in the 1998 book by Richard Rhodes, "Deadly Feasts: Tracking The Secrets Of A Terrifying New Plague". I preferred the Rhodes account - his exposition of the science was clearer, and I thought he told a better, tighter story.
However, there's not that much to choose between the two, and Max's book does have the extra material about FFI, which is interesting in its own right. Max does make one misjudgement, in my opinion, which is to include an account of his own illness (he has been diagnosed with Charcot-Marie-Tooth disease which, although it is a neurodegenerative muscular disorder, is neither prion-related nor an amyloid plaque disease). Inclusion of this essentially irrelevant material is a distraction, which just muddies the exposition.
One final criticism is that Max includes an unquestioning discussion of putative geographical "clusters" of CJD cases, based solely on their identification by patients' family members, whom he refers to as "Creutzfeldt Jakobins" (a hideous, tin-ear coinage, which he seems to think is clever). These so-called clusters are almost certainly spurious, based on an incorrect application of the relevant probability models and Max's failure to identify the error detracts from his objectivity as a science writer and contributes to a presentation of disease spread scenarios which are unduly alarmist. The discussion of possible treatment options in the final chapter also struck me as weak, an over-interpretation of what are essentially just anecdotal data. One sees this kind of over-interpretation all the time in the popular press, but I would have expected better from a science writer as experienced as D.T. Max.
However, these are minor criticisms of this well-written account of a fascinating subject.
Availability: Usually ships in 24 hours
Buy from Amazon
|
| |
|
|
|
| |
|
|
|
|
|
|
