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)
This has become one of my favorite non-fiction books on audible. If you enjoyed "Splendid Solution", "The Great Influenza" or "Germs" you will love this one. It may not have the humor or entertainment value of "The Omnivore's Delimma" but the author does a fine job of weaving personal stories with science to create a fascinating story. This is a great introduction to Mad Cow and other prion diseases, and also provides a distrubing account of how governments bumble their way through such outbreaks.
Grover Gardner also performs another excellent reading. In my opinion, Gardner is by far the top narrator for any material that has any scientific or technical content. His voice moves gracefully over the text - always with the right nuance and pronunciation - allowing the listener to become quite captivated by the story.
Wonderfully written account of prions: the diseases associated with them; the nobel laureates who study them; people affected; and how the problem began, was discovered, and has spread. Like a PBS special and a novel rolled into one, you learn quite a bit while being thoroughly entertained. A page turner.
I know others have put down this book because it isn't just about the life of the family that couldn't sleep. True, it is a book about prions, and tells of all of the research in many fields and into many disorders. But, scientific though it is, it really is facinating and quite easy to follow along with and understand. The reader does have a bit of an annoying habit of ending every sentence the same way with a strange inflection, but you'll get used to it, or at least learn to ignore it.
among the top books i have listened to on audible
well researched, up to date, and narratives that bring you to understand the lives affected by those afflicted with prion disease.
a fatal insomnia
a unique historical perspective on a rare inherited genetic disorder that strikes its victims with symptoms of a lethal insomnia in their most productive years. Also, tied into this narrative of the familial disease, is its link to the prion disease of mad cow, or CJD. The research documented by D.T. Max, brings to light cases of individuals affected by CJD that has occurred in the U.S. and the U.K. which can only be explained prions from contaminated beef. That the threat still exists, and human cases are being suppressed by the USDA, should make anyone concerned about the potential impact of circulating prions in our beef supply.
It is surprising how fascinating a medical mystery can be. I was hanging on every word. I was terrified, but still could not stop listening.
I picked this right after starting the short-story anthology Zombies vs. Unicorns (zombie fiction sometimes blames prions) on Kindle. Fun connection. As to this book, it was a great story, interesting science, kept a good pace, and gave me pause (yet again) about eating factory-farm meat...
College English professor who loves classic literature, psychology, neurology and hates pop trash like Twilight and Fifty Shades of Grey.
look into the frightening world or prion diseases.
Well paced and scientific. A good deal of jargon and acronyms but that was what I was expecting. More like a history of prion disease with lots of case studies and history.
Yes. The story is well researched and written in an engrossing style. Medical mysteries and detective work are as fascinating as any other sort of story, real or fictional. The author, D. T. Max, suffers from one form of misfolded proteins that impair nerve transmission to his muscles. In researching his own little understood condition, Max interviewed members of an Italian family prone to develop fatal insomnia, an even more rare, inherited misfolded protein illness affecting very few families worldwide. Max also studied assorted prion diseases, usually contagious, sometimes possibly not, that have become known to the public in recent decades. Prions are misfolded proteins, similar to the causative problem in the author's syndrome. Prions cause, among other fatal illnesses, the so-called Mad Cow disease (bovine spongiform encephalopathy) and Creutzfeldt-Jakob disease (CJD.) This book reveals the difficulty researchers encountered in discerning the causes of these illnesses, the various governments that dealt poorly with reducing spread of contagion by prions, and the totally inadequate efforts that are being made towards treatment and cures.
This is not a book centered on characters, though many fascinating and very special people are described.
Again, not pertinent. Gardner's narration was well paced and suited the material.
Yes, even though it is long and there is a great deal of data to absorb. I found the subject matter fascinating.
Anyone who likes medical mysteries would enjoy this book. But it is more than a story about discovery. Max's book makes it clear that we all face some degree of risk of contracting a fatal prion-based illness, thanks to the tendency of governments to allow exposure of the population to continue, out of fear of the negative economic impact that might occur or simply out of sheer hubris and denial. Equally alarming is the paucity of research into prevention, treatment and cures, since the number of affected people doesn't appear high enough to provide pharmaceutical companies with the incentive of making a profit. I foresee a time when there will be plenty of sufferers to prompt investment in finding ways to prevent and treat protein misfolding illnesses. That need not happen, if the curative effort is pursued sooner rather than later. I have taken care of two patients who suffered from CJD. Their ends were horrible and tragic. I hope for all our sakes that some intrepid researchers continue the fight against prions and other misfolded protein syndromes, so that our children don't have to deal with the heartbreak of such illness in their lives.
Prions. Before reading The Family That Couldn't Sleep, I had no idea what those were. Since finishing this book, I've developed an equal sense of respect and fear of them. "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." How's that for a mouthful?
At the center of this book is a Venetian family with a deadly legacy of Fatal Familial Insomnia dating back to the 1700s. FFI is a disease that strikes its victims in middle age, and causes complete insomnia, exhaustion, and eventual death within a matter of months. Max, himself a victim of a degenerative neurological disorder, expounds on the history of prions, theories on their origins, and the culminative affects on peoples and lands throughout the world. Cast your mind back to the Mad Cow Disease scare in Europe, or even the first cases of scrapie among sheep in Europe in the 18th century; these can be linked back to very bad little prions.
I really enjoyed the break down of scientific terms and I especially loved the history part. I find that I almost always enjoy the style and flow of books that are written by journalists, which is probably why it put me in mind of Brain on Fire by Susannah Cahalan and Lost in Shangri-la by Mitchell Zuckoff. A great read whether you're scientifically inclined, or just along for the adventure ride! Another plus: I now kinda understand the scientific references Amy Farrah Fowler, a fictional neurobiologist on the show The Big Bang Theory, periodically makes to her research work. Winning!
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