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)
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!
If you have any interest in science please; please enjoy this book-
The favorite is the saddest-
The inflection in his voice was perfect for setting the tone and carrying it-
This book is interesting and a bit frightening with the history of animal to human disease transmission
I loved reading 'The Disappearing Spoon' and 'The Poisoner's Handbook' and this book feels a little bit like that-
I love AUDIBLE! I never get mad at traffic jams and can listen to many different books, despite of my short time.
I learned a lot of prion from this book. The story is good and the writer tries to entangle the other kinds of prion disease. It's not just about the family that couldn't sleep. I hope that there will be a cure for prions soon.
Wow, I had no idea how much prions affected our world! From cannibals, to mad cows, to inherited conditions. Mad cow hit the head lines and we all learned some, but this book exposes and explains the stories behind the headlines, and why these diseases are so scary. Nothing graphic, good language, highly recommend this book to all interested in disease, science, and a good listen.
There is a family of disorders that eat away the brains of their victims, whether they are humans or livestock. I know, sounds depressing, but the search for the cause of these disorders is utterly fascinating. There are enough personal stories to make it feel a bit like a novel at times, but it is actually a true scientific detective story. There are brilliant and odd scientists, conflicted and disorganized governments, and some deeply sad and touching personal stories. There are also enough ironic and even humorous moments to keep things from ever feeling too heavy. I particularly liked hearing that a researcher had for years kept a flock of infected sheep on the roof of a London hospital. I've been passionate about reading up on these prion diseases for years, and I still found lots in this book that was new to me. I've shared this book with people who were unfamiliar with prions, and they have found it captivating and easy to understand.
This was a great book. It had very good information and was well written. Fascinating biological information was expressed thoughtfully. I have a degree in biology and still felt that the information was in depth, but would be appropriate for any listener.
The reader did an amazing job with this book! It almost felt like a novel a times, partly due to how well the reader did his job. The stories are incredibly compelling and the science is completely accessible and fully explained and very interesting. I cannot imagine a way to die that is more terrible than FFI - the prion related disease that robs victims of their ability to ever fall into sleep. And in the exposition of this disease and its history, is woven the development, history, and scientific exploration of all prion diseases. On top of that, there is a personal feel, as though you are reading the diary or memoir, since the author suffers his own afliction which, for the author, was the motivation for looking into these orphan diseases. If you are not into science/medical stories, this may not appeal... but if you have any interest, this is very, very well done.
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.
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