Status
Available
Call number
Collection
Publication
Random House (2006), Edition: First Edition, Hardcover, 336 pages
Description
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 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-for example, 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. With remarkable precision, grace, and sympathy, Max-who himself suffers from an inherited neurological illness-explores maladies that have tormented humanity for centuries and gives reason to hope that someday cures will be found. And he eloquently demonstrates that in our relationship to nature and these ailments, we have been our own worst enemy.… (more)
User reviews
LibraryThing member arubabookwoman
In effect, this book is a medical detective story, tracing the medical quest to diagnose and treat cases of Fatal Familial Insomnia. One in two members of a family in Italy suffers from this disease (and it is traceable back through several generations), which strikes when victims is in their 50's,
The prion has been described as one of the strangest things in all biology. Insofar as is known, prions do not replicate themselves. It is not a virus or a bacteria, but is a protein, a non-living thing. In 1997, the prion was shown to be the infectious agent causing such diseases as Mad Cow disease, Creutzfeld-Jakob disease, kuru, a disease found only in some indigenous populations of New Guinea, and perhaps other diseases. Prion diseases appear to be the only diseases that can take one of three forms: genetic, infectious and accidental. We know that prions are found in all mammals, but we don't know their function in healthy mammals.
As one scientist says, "What kills viruses and bacteria barely affects them. Boiling will not disinfect them, nor will heat. You can't reliably 'kill' a prion with radiation. You can't pour formaldehyde on it to render it harmless--in fact formaldehyde makes prions tougher. Not all bleaches can kill prions and those that can need to be highly concentrated. Prions bond to metal. They can be spread, for instance, when doctors reuse the electrodes planted in patients' brains for EEGs or by dental equipment."
This was a fascinating scientific story about this strange protein of which I'd never heard (although obviously I'd heard of mad cow disease and Creutzfeld-Jakob). A couple of Nobel prize winners received their prizes based on their prion research, and I understand that there is much ongoing research, as there is much still to be discovered about it.
Recommended
3 stars
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and which is always fatal. Only about 40 families world-wide, all unrelated, suffer from this disease. Only in recent years was it discovered that Fatal Familial Insomnia is a prion disease. The prion has been described as one of the strangest things in all biology. Insofar as is known, prions do not replicate themselves. It is not a virus or a bacteria, but is a protein, a non-living thing. In 1997, the prion was shown to be the infectious agent causing such diseases as Mad Cow disease, Creutzfeld-Jakob disease, kuru, a disease found only in some indigenous populations of New Guinea, and perhaps other diseases. Prion diseases appear to be the only diseases that can take one of three forms: genetic, infectious and accidental. We know that prions are found in all mammals, but we don't know their function in healthy mammals.
As one scientist says, "What kills viruses and bacteria barely affects them. Boiling will not disinfect them, nor will heat. You can't reliably 'kill' a prion with radiation. You can't pour formaldehyde on it to render it harmless--in fact formaldehyde makes prions tougher. Not all bleaches can kill prions and those that can need to be highly concentrated. Prions bond to metal. They can be spread, for instance, when doctors reuse the electrodes planted in patients' brains for EEGs or by dental equipment."
This was a fascinating scientific story about this strange protein of which I'd never heard (although obviously I'd heard of mad cow disease and Creutzfeld-Jakob). A couple of Nobel prize winners received their prizes based on their prion research, and I understand that there is much ongoing research, as there is much still to be discovered about it.
Recommended
3 stars
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LibraryThing member psutto
Medical mystery, political commentary & scientific exploration of prions
This tells the story of a family that suffers from FFI (Fatal Familial Insomnia) which was a complete medical mystery until recently and how the diseases of Kuru (in cannibals tribes in Papua New Guinea), Creuzfeldt Jakob
Overall – A fascinating medical detective story
This tells the story of a family that suffers from FFI (Fatal Familial Insomnia) which was a complete medical mystery until recently and how the diseases of Kuru (in cannibals tribes in Papua New Guinea), Creuzfeldt Jakob
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Disease (CJD) and BSE helped identify why members of a certain Italian family were falling victim to a disease that baffled the medical community and was seen as a family curse. Victims inherit a tendency to manufacture prions in their own bodies. These accumulate and destroy the brain's sleep centres, resulting in sweaty, hollow-eyed demented death. In order to tell their story the author covers much of what is known about Prion diseases discovered through research into Scrapie, Kuru, CJD & BSE by its sometimes maverick researchers – for example Carlton Gajdusek laid the groundwork in working out what caused Kuru but was also an enthusiastic paedophile who graphically recorded his sexual exploits with young Fore (a Papua New Guinea tribe) people in his laboratory diaries. D. T. Max weaves all this information into a engrossing narrative that shows that although we now know much more about Prions there is still much to learn and it appears that we may not have learned our lessons from the BSE crisis (hence the political commentary). Protein mis-folding is a hot topic in medicine now with possible applications to a great many diseases including Alzheimer’s, Huntingdon’s and Parkinson’s.Overall – A fascinating medical detective story
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LibraryThing member jen.e.moore
An excellent history of prion diseases, framed by the story of an Italian family with a genetic prion disease, fatal familial insomnia, that kills by exhaustion. I'm equal parts fascinated by this disease's potential to illuminate the way sleep works, and horrified by the very thought of prion
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diseases. Show Less
LibraryThing member steadfastreader
Excellent overview of the history and current science regarding prion diseases such as Mad Cow, Kuru, and Fatal Familial Insomnia. Easy enough for the layman, yet comprehensive. Definitely fun.
LibraryThing member dandelionroots
Intensely interesting. Follows the history of infectious and inherited prion diseases through their outbreaks in animals and their appearance in humans using the story of the Venetian family with fatal familial insomnia as a backdrop. Nature is terrifying, especially when humans inadvertently
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amplify its horrors. Show Less
LibraryThing member sallypursell
Riveting. Loved it.
LibraryThing member Niecierpek
A very well-written book on the latest research on prions and prion caused diseases (kuru, scrapie, mad cow disease etc), interspersed with a leit-motif of the history of a Venetian family suffering from the fatal familial insomnia- also a prion caused disease- robbing the victims first of sleep
A prion is a protein that is neither bacterial, fungal nor viral and contains no genetic material. It occurs normally in a harmless form, but by folding into an aberrant shape, the normal prion turns into a rogue agent. It then makes other normal prions to become aberrant prions and wreaks havoc in the brain of the victim leaving it with holes and plaques resembling those of Alzheimer’s patients.
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and then leading quickly to his or her death amidst a lot of suffering.A prion is a protein that is neither bacterial, fungal nor viral and contains no genetic material. It occurs normally in a harmless form, but by folding into an aberrant shape, the normal prion turns into a rogue agent. It then makes other normal prions to become aberrant prions and wreaks havoc in the brain of the victim leaving it with holes and plaques resembling those of Alzheimer’s patients.
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LibraryThing member ScoutJ
This was a fascinating look not only at the titular family, but of prion diseases as a whole. Max covered the family with Fatal Familial Insomnia, as well as many other forms of the disease group, up to and including, Bovine Spongeiform Encephalophopy, aka Mad Cow Disease.
LibraryThing member Vidalia
A fascinating look at prions - rogue proteins that fold, creating chain reactions of folded proteins in human cells. Prions are not "germs" - neither viruses nor bacteria. They are not living entities, and yet they cause diseases in humans - terrifying illnesses like "mad cow disease." Max makes
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complicated medical science a compelling, and comprehensible, read. Show Less
LibraryThing member iammoore
very much like the book on Henrietta Lack: personal story mixed with biology
LibraryThing member carolcarter
Despite its sad subject this book was very good. I found it difficult to put down. Mr. Max looks at prion diseases which are as varied as scrapie in sheep, mad cow disease, Creutzfeldt-Jacob disease and Fatal Familial Insomnia - hence the title. The book opens with the title family in Italy which
The science is very simply and concisely explained and the history of discovery is full of characters. A very enjoyable book.
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has been plagued with FFI for at least two hundred years. Max presents lots of science and the entire history of the discovery of prions and how they cause disease. There is., as yet, no cure for any of them. Scrapie and mad cow can be controlled and, I suppose, FFI could be bred out of existence but we are talking of humans. The remains of the Italian family have now all been tested and could know which of them will likely get the disease and/or pass it on. To a man/woman they have refused to know these results. If there were a cure they would likely feel differently. The science is very simply and concisely explained and the history of discovery is full of characters. A very enjoyable book.
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LibraryThing member daisyq
I found this to be a fascinating read about the discovery of prion diseases. It highlighted some interesting people who were central to research in this area.
I found the sections about the discovery and cover up of BSE/mad cow in the UK particularly interesting.
This was interspersed with the sad
I found the sections about the discovery and cover up of BSE/mad cow in the UK particularly interesting.
This was interspersed with the sad
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story of the family with fatal familial insomnia, and the way they were misdiagnosed over many years. Show Less
LibraryThing member delphica
Oh, this was intriguing. A very basic overview of prion diseases for the lay person. Full of amazing facts!
It starts off looking at a family afflicted by an inherited form of a prion disease, and then explores other incidents of prion disease outbreaks in animals and in humans.
As a hypochondriac,
It starts off looking at a family afflicted by an inherited form of a prion disease, and then explores other incidents of prion disease outbreaks in animals and in humans.
As a hypochondriac,
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it was okay in that I only thought I had prion disease for a relatively short portion of the book, and it wore off quickly when I was done. :) Show Less
LibraryThing member marshapetry
Really enjoyed, nicely told, and pulled together a lot of information. Kept me interested the whole time. Got a bit scared of "prions" and eating meat but I'll just have to get over it :)
LibraryThing member SylviaC
Interesting subject, and mostly well-presented. He does tend to over-dramatize, especially in the sections where he doesn't have a lot of solid facts to work with.
LibraryThing member debs4jc
In sharing the story of an Italian family beset by a strange medical illness that causes insomnia and eventually death, the author also explains the history of that rare class of illness known as prion diseases. Much is still unknown about them, but he goes through the history of how what we do
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know was discovered. Prion diseases, like scrapie in sheep and mad cow disease in humans and cattle, are caused by a malformed protein. The author explains things pretty well this book does get dry in places. If you are interested in medical stuff or have a personal interest in prion diseases then this book will interest you. Otherwise it might not, but I did find the stuff about mad cow disease a bit frightening, as still so much is unknown about how it is spread, who might be exposed, and there is no cure. Show Less
LibraryThing member cmbohn
themes: disease, heredity, science, sleep, farming/agriculture
So you've been having trouble sleeping? I bet your trouble is nothing compared to what this Italian family has gone through. They are cursed with the gene that causes Fatal Familial Insomnia, which is pretty much just what it sounds
But that's just the beginning of this book, although the author comes back to the family over and over. The real subject of the book is prion disease, that weird little twist that lets renegade proteins act like a virus and attack the body in ways that science can't yet combat.
The most familiar of these, and the only one that I recognized at the outset as a prion disease, is Mad Cow Disease, or bovine spongiform encephalopathy or BSE. That "spongiform" part is because it basically turns the brain into a spongy, gooey, hole-riddled mass. (Sorry if you were eating. Hope it wasn't beef.) And because the disease is caused by a deformed protein, cooking it will not not kill the disease. Neither will radiation, alcohol, antibiotics. In fact, almost nothing will kill it. It's seriously bad news.
I really liked this book. It reads more like a mystery. He does tend to throw around some medical jargon, and then later he'll oversimplify to avoid the jargon, so it's not perfect. But it was a read that will keep you up at night.
4 stars
So you've been having trouble sleeping? I bet your trouble is nothing compared to what this Italian family has gone through. They are cursed with the gene that causes Fatal Familial Insomnia, which is pretty much just what it sounds
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like. You inherit it, and you can't sleep, and you die. A few other things happen too, but that's the killer. The brain, and the rest of the body too, needs sleep. But that's just the beginning of this book, although the author comes back to the family over and over. The real subject of the book is prion disease, that weird little twist that lets renegade proteins act like a virus and attack the body in ways that science can't yet combat.
The most familiar of these, and the only one that I recognized at the outset as a prion disease, is Mad Cow Disease, or bovine spongiform encephalopathy or BSE. That "spongiform" part is because it basically turns the brain into a spongy, gooey, hole-riddled mass. (Sorry if you were eating. Hope it wasn't beef.) And because the disease is caused by a deformed protein, cooking it will not not kill the disease. Neither will radiation, alcohol, antibiotics. In fact, almost nothing will kill it. It's seriously bad news.
I really liked this book. It reads more like a mystery. He does tend to throw around some medical jargon, and then later he'll oversimplify to avoid the jargon, so it's not perfect. But it was a read that will keep you up at night.
4 stars
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LibraryThing member Rob.Larson
I picked up this book because I have insomnia. It is very eye opening (no pun intended), with tons of facts about the meat industry, which make you want to go vegetarian! I was also surprised to learn about how animals have been bred for hunting purposes (deer, elk). What a mess people have created
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by being greedy! Excellent author, though the material is quite technical in parts. Also, I was shocked to learn that animals (cats, dogs, et al) "humanely" put down are part of products rendered for animal feed. Amazing. Show Less
LibraryThing member abuannie
Fascinating linkage betwen prion and misfolded protein diseases, very easy and interesting read - interspersing prion science with thehistory of one Italian family's Fatal familial insomnia.
Awards
Salon Book Award (2006)
Physical description
336 p.; 9.2 inches
ISBN
1400062454 / 9781400062454
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