The Family that Couldn't Sleep by D.T. Max

The Family that Couldn't Sleep Reviews

• 
  Lynne King

  I reread this review today (1st March 2014) that I wrote last year because a friend, of a friend of mine, has died from Prion's disease and has lost two siblings in the past year. How dreadful...I must reread this book.
  
  * * * * * * * * *
  
  I have a problem and it concerns books. If I see a title that sparks my curiosity, I must have it. I can normally keep this under control but then an enemy was unleashed in the form of my Kindle Paperwhite in February 2013. As a consequence, one click on Amazon and yet another book is added.
  
  This book is a typical example of my “addiction” (which I hope is temporary), but the title cried out “buy me”, “buy me” and that’s what I did there and then. The three magical key words “sleep”, “medical” and “mystery” gave the seal of approval. I could feel that sense of anticipation and I knew that this was going to be an excellent read, which it proved to be.
  
  The author immediately whetted my appetite when I read that Stanley Prusiner received the Nobel Prize in Physiology or Medicine in October 1997 for his twenty-five years’ of study on prions. This dreadful “infectious agent responsible for bovine spongiform encephalopathy, or mad cow disease, Creutzfeldt-Jakob disease, and a disease in sheep called scrapie."
  
  The main reason for my intrigue was that prions are neither “a virus or a bacterium but a protein, a non-living thing” and also strangely enough, there’s no DNA. Prions cannot even be ”killed”, even though they are not alive. A bizarre statement.
  
  How could a non-living thing cause the havoc that it did in patients’ brains and still continue to do so? Well I soon found out when D.T. Max skillfully sets the scene for this incredible book with the first of various medical mysteries, that of a family in Veneto who in 2001 were thinking about what Prusiner had said. This Italian family has been suffering from a very rare disease called “Fatal familial insomnia (FFI)”, that is, not being able to sleep and finally dying from it. I initially thought that must be nonsense, because everyone sleeps for roughly a third of a day (that’s part of our makeup). However, FFI symptoms are grim, normally arrive in family members over fifty, sweating is profuse, troubles with sleeping, and finally they cannot sleep at all. The brain is in constant overdrive and they fall into a state of exhaustion that resembles a coma. Regrettably it’s downhill all the way until the eventual death, normally after about fifteen months.
  
  This family could trace the disease back to the mid-eighteenth century to a Venetian doctor as a document was found describing his horrifying, but very similar symptoms, resulting in his death.
  
  With further reading, I found out that when brains were examined after death, dreadful discoveries were made:
  
  “The brains were badly damaged – full of holes, astrocytes, and plaques, piles of tangled dead protein.”
  
  Comments from individuals like Pierluigi Gambetti, a director at the national prion surveillance center in Cleveland, one of the discoverers of the disease stated:
  
  “I used to think that Alzheimer’s was the worst diseases you could get? But to see a loved one disintegrate in front of your eyes – and for that person to know it is happening? Somehow, the fact that it is so rare makes it even worse, it seems to me. I think now even a car accident would be less cruel.”
  
  But to me, the most remarkable section of this book is when the author takes us to Papua New Guinea:
  
  “Young man, be careful! I ate your grandfather” – Elderly Fore to his nurse at an Okapa hospital in 1947.
  
  I had read about the practice of shrunken heads in Papua New Guinea, but it was rather unnerving to read that tribes out there were still cannibals in the 20th century.
  
  Here, in the late 1950’s, Western doctors had found that the Fore tribe (an extremely primitive tribe; hunter-gatherers but also great fighters – they just fought everyone and for the most minor reasons) were suffering from a rather strange ailment called “kuru”. This was another case where a non-living molecule could reproduce itself and cause an infection as if it were actually alive.
  
  How is that possible? It’s an absolute contradiction. But here again we have Carleton Gajdusek, another Nobel Prize winner (1976) for his work and what an interesting character he turns out to be; even spending time in jail in the States for his rather interesting “pleasures”. He liked young boys, “took an intense sexualized interest in the children he studied” but he said that this was only for medical research. The authorities in the States thought differently as they knew what a pedophile was.
  
  Nevertheless, Carleton Gajdusek did excellent work in Papua New Guinea and he deserved to be a Nobel Prize winner. However, he still couldn’t “crack kuro on his own”. Help was needed from epidemiologists and anthropologists, and in fact it was the latter who found out what the problem was there. All I can say here is that cannibalism is connected but in all fairness to the Fore, “they never ate their own children or grandchildren, though: they saw that as incest which was taboo”.
  
  I kept on wondering why the author had written this book and it transpires that he suffers from an inherited neurological illness. Not life-threatening such as FFI, but his muscles are slowly wasting away and he has to wear leg braces.
  
  After finishing this book, all I could think about was what a fantastic social document it is; plus poignant, moving, amusing but also uplifting in that it’s an excellent medical mystery, so do try it! I’m sure that you won’t be disappointed.
  
• 
  Kate

  I learned a lot about prion diseases from this book, but it suffers from some major issues:
  
  1. It is poorly organized. The chapters alternate between telling the story of the "family that couldn't sleep"--an Italian family suffering from Fatal Familial Insomnia, or FFI--and covering the history of prion diseases & research. That would be fine on its own if there was still some kind of timeline holding everything together, but there isn't: one chapter will discuss prion research from 1970-2004, and then the next chapter will return to research in 1980 (and then to a disease not mentioned for 5 chapters), and so forth. It was impossible to get my bearings in this book. Furthermore, at no point does DT Max set aside time to go through a simple explanation of prions, prion diseases, and some of their basic differences: all these facts appear willy-nilly, buried in the text, without a glossary for assistance. Some terms aren't made clear until near the end.
  
  Also, the Italian family with FFI is discussed at length, and my copy of the book does not have a family tree. With this many relatives and a heritable disease involved, a family tree is a necessity.
  
  2. Lack of scientific rigor. The last few chapters inexplicably step away from science and turn to online message boards (no, REALLY, message boards) and amateur speculations. The book is subtitled "A Medical Mystery," not "A Medical Mystery plus stuff I read from these people online oh and I wonder about vaccines sometimes."
  
  3. One of the key researchers of prion disease--and a Nobel winner, to boot--is a pedophile. An unrepentant one, at that. Max, to his credit, does not sweep this under the rug, but his attempts to be serious-but-not-TOO-serious fall flat. It's gross.
  
  In sum: I did, as I noted, learn from this book, but it has serious issues (WHITHER THE EDITOR? WHITHER?) and that prevents me from giving it any more than the "it was okay" two stars. If you're not highly science-literate AND very interested in the topic, just skip this one.
• 
  Evie

  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!
  
• 
  Cindy

  Prion diseases are freaky! That little bits of proteins could mis-fold, and that topological change could decimate a brain is just bizarre. One of the facts I was most surprised by is that prion diseases have three methods of infection: genetic, direct contact (i.e. eating or touching infected tissue), and spontaneous (i.e. a protein accidentally misfolds in the body). No other disease vector can spread via all three methods like prions. They are freaky disease superstars!
  
  The Italian family in the title is beset with FFI, fatal familial insomnia, an inherited/genetic prion disease. It's sufferers tend to develop symptoms in middle-age (usually), and die fairly quickly. It's grim: their pupils turn into pin-pricks, they start sweating profusely, and they become unable to achieve any type of restful sleep. Eventually they lose all control, go into mad fits, finally fall into a coma and die. All the while their mind is intact.
  
  The family's biography is only a frame for the rest of the book. In order to explain FFI and how difficult it was to diagnose as a prion disease, you have to understand the history of prion diseases, and the history of the field. Max delves into scrapie, kuru, GSS, CJD, BSE, and other known prion diseases. In some ways, the story of the researchers trying to pin-down this new class of illness was more fascinating than the family that couldn't sleep. There are huge egos, government cover-ups, and other non-science dramas that affect the lives of many people.
  
  I really, really enjoyed this book - Max sets up the story in an extremely engaging way. It reads like a medical thriller - like something out of that TV show Mystery Diagnosis, but on steroids. There are twists and turns to the diagnosis, and a whole lotta shock factor.
  
  And yet I had to dock it a star. I thought there were two questions not just left unanswered, but totally unaddressed. 1) How does a genetic version of a prion disease, like FFI, not cause symptoms until middle-age? Is the disease building up slowly over time, or does something later set it off? 2) How does a simple mis-folding of a protein lead to a swiss-cheese brain? How do you connect the dots from misfolding to erosion of brain tissue and development of plaques.
  
  There's a good chance that there aren't satisfactory answers to either of those questions, but I was hoping Max would at least acknowledge or address them. He certainly didn't shy away from other more technical discussions.
  
  I had just finished the chapters on Mad Cow/BSE/CJD and Max goes into detail about the state of affairs today. Spoiler: it's not good at all, particularly the government's reluctance in the US. British beef is safer than US beef. Scary. My husband and I had already planned on eating beef for dinner - we had some leftover steaks that needed finishing. It certainly gave me pause. I don't eat a whole lot of beef as it is, but I might try to cut back a little more. Prions are just that freaky.
• 
  Anita Dalton

  The family that could not sleep is a family in Italy that suffers from a disease called Fatal Familial Insomnia. There are several other families in the world affected by the condition, so it is extremely rare. It is a condition that strikes family members generally in late middle age and causes them to begin to lose physical control of their bodies as they stop sleeping. They sweat, they develop a very distinct pinprick appearance to the pupils in their eyes, they stop sleeping, and in end stages, have virtually no control over their bodies. In many cases, those who suffered from it were assumed to be either crazy or chronic alcoholics, and there is not a thing that can be done to help them. So few people suffer from the condition, and a cure would be so expensive to find that there is little incentive for drug companies even to research the condition.
  
  
  Read the rest of the review here.
• 
  Petra: hiatus, finding it hard to communicate

  This stunning book is about a very rare inherited neurological disease which strikes in middle age and one of whose main symptoms is the inability to sleep which quite quickly leads to death. The book is written in a very readable way and follows one family, the main family who suffer frm this terrible disease.
• 
  Richard

   
  Every time I donate blood (and I've donated well over ten gallons) I'm asked whether I've spent at least three months in the U.K. prior to 1996 (
  c.f.). This is because of what we all called "Mad Cow Disease" and what the medical folks now call Variant Creutzfeld-Jacob Disease (vCJD).
  
  This book is poorly titled. Certainly, the horrible fate of an Italian family brings immediate human pathos to the story of prion diseases, but the more pressing story for many of us will be the atrocious practices in our food supply that permits—or even encourages—diseases like vCJD. But the real story lies elsewhere.
  
  (It doesn't impress me that the Italian family is described as "noble", according to the blurbs. What on earth does that have to do with anything? Is the disease be more terrible because it is inflicted on "modern, cultured Italians, with stylish hair and eyewear"?)
  
  There are several parallel stories told here. The chapters dealing with the Italian family's curse are interesting, as are those describing the investigation into
  Kuru ("a transmissible spongiform encephalopathy associated with the cannibalistic funeral practices of the Fore people of New Guinea"), although the chapters discussing the petty squabbles and large egos of the leading researchers in the field were less than riveting. But the heart of the book is the drama of how foolish cultures (British and American, mostly) willingly tolerate a socioeconomic system that eviscerates governmental regulation and industrial oversight, and how that played out in the Mad Cow disease crisis of the late 80s to mid-90s. (America mostly sitting smug on the sidelines, yet meanwhile tolerating incredibly disgusting practices in meat production, as well as incubating the
  Chronic Wasting Disease that is still spreading).
  
  A European Union committee estimated that the English ate as many as 640 billion "doses" of Bovine Spongiform Encephalopathy during the mad-cow crisis. Luckily, it turns out the disease jumps the species barrier to humans very, very poorly—although there was a time when we didn't know that. British agricultural regulators are also responsible for the promotion of British agriculture (as so with the USDA), so they buried the story as long as they could. By the mid-90s, when the crisis reached its peak, one expert admitted that they could not "rule out 500,000 cases", and newspapers were wondering whether Great Britain would soon end up quarantined with half a million dying a year.
  
  The problem? A fairly rare disease was spread through milk herds of Britain through the practice of protein supplements fed to milk cows. Among the sources of that protein are the carcasses of cows too diseased to permit into the human food supply. (Another source is chicken feces, which is still quite legal in the United States, by the way). Almost all diseases are eliminated through the rendering process, but "prions" are incredibly durable proteins.
  
  But early on, British authorities were astonishingly stupid in their response to this disease. Americans had developed an antibody test that would almost instantly confirm the presence of a prion-based disease, but the British regulators choose to use a laborious and lengthy process of microscopic visual inspection rather than admit to trading partners that their food supply might have yet another problem. In fact, England's chief epidemiologist "wasn't a believer in prions". This, despite the fact that by this time two different Nobel Prizes had been awarded (to Americans!) for the discovery of this new disease vector.
  
  
  Britain—and the world—got lucky because BSE/vCJD isn't easily transmissible from cows to humans or between humans. Unlike scrapie in sheep, for instance, or perhaps Kuru (which might, perhaps, be infectious by merely handling the corpse of an infected person, instead of through cannibalism). The United States has inadvertently pushed scrapie into the wild population of deer, elk, etc., in the American wild, where it spreads with apparent ease.
  
  
  After finishing this book, it is a relief to ponder that all-in-all the number of people that die annually due to prion diseases is probably barely one hundred, and so this is less of a realistic threat to the reader than, say, bee stings or choking on a chicken bone. But prions are the worst instances of proteins-gone-bad, and misfolding proteins are also suspect in many other diseases (e.g., Huntington's and Alzheimer's), and so we'll probably be hearing more about prion-like diseases in the future.
  
  This was an engaging and well-written book; I highly recommend it for anyone that enjoys science and medical non-fiction.
   
• 
  Grumpus

  This is based upon the audio download from [
  www.audible.com:].
  
  Narrated by: Grover Gardner
  
  Like a smart consumer in the market for electronics, appliances or cars, I research my purchases by looking up recommendations on Consumer Reports. When I am looking for a good book to read, I turn to my trusted source for reviews—Goodreads. Based on member recommendations, I know going in that the book I choose will more than likely receive a higher than average rating from me. You guys have never let me down. . . well, until now. Don't worry, I won't hold it against you. :-)
  
  The title of this book and the medical mystery it implied intrigued me and I was excited to read it. It wasn’t long before I felt that the title of the book was a misnomer. The audio version on which I base this opinion, was just over eight hours long and 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 the title but I still felt mislead on this one from the beginning.
  
  
• 
  Gina

  I'll try not to give 5-star ratings willy-nilly, but this was a pretty amazing family biography, spanning centuries' worth of generations and shedding light on a variety of subjects through the lens of this bizarre and incredibly rare genetic disease. Agriculture, attitudes toward disease, the relationship between science and doctors, the mysterious biological function of sleep, international politics, economic competition and, of course, personal profiles of the afflicted. The drama of the current generation, who have a 50/50 chance of one day suddenly losing the capacity for sleep, ties it all together, and gives you a significant reason to keep reading and caring. I learned so much! Also, I gained an appreciation for the medical significance of the anomoly. A successful and engaging blend of family biography and the wide-reaching social history.
  
  Note: seems like every other word in this book is "sleep," so read it when you're not too tired, or you won't make it very far. Furthermore, you will feel like a jerk when this family's exhaustion somehow functions as your bedtime story/lullaby.
• 
  Петър Стойков

  Световната истерия около болестта Луда крава попремина, така че може би сега, на трезва глава, е времето да се запознаем с историята на прионните болести - история на, както казах, масова световна истерия, канибализъм и двама нобелови лауреати от които единият е педофил, а другият - безскрупулен психопат и лъжец.
  
  А самите приони са ебати врътката човек - за вирусите нали се спори дали са живи изобщо, толкова са елементарни... а прионите са по-елементарни и от тях.
• 
  Talulah Mankiller

  Max’s “hook” is the family history of an Italian clan that suffers from a rare prion disease called fatal familial insomnia: basically, it eats away part of your brain, burns out your adrenal gland, and eventually kills you because you can’t sleep. Several victims were actually observed by a world-renowned sleep clinic before their deaths, and even though the patients went into REM and everything, their sleep wasn’t…normal. REM is supposed to momentarily paralyze the sleeper, but the people with FFI were up, walking around, and occasionally bowing to the Queen of England (no, I didn’t make that last part up). Unsurprisingly, when the poor bastards finally “awakened,” they didn’t feel like they’d slept at all. Because they hadn’t.
  
  Say it with me: THAT SHIT BLOWS.
  
  Anyway, that’s the hook, and it’s a good one, but the truth is that Max actually wrote what is essentially a pop history of prion diseases in general: he covers mad cow, scrapie, and kuru, among others. Prions, in case you didn’t know (and I didn’t) are defective proteins that replicate themselves ad infinitum—introduce a prion into your system, and it’ll turn your good proteins bad. Which, in turn, will gunk up your brain, drive you crazy, and then kill you. Fun!
  
  Prions are actually a source of much contention and debate, because they go against our current understanding of infection: basically, we believe that in order for something to infect us, it has to be alive. Viruses technically aren’t, but even they have RNA/DNA, so they pass on a technicality. Prions, though, are just protein. They aren’t alive, not even on a technicality. There are still scientists who believe that the protein theory is wrong and that if we can just get the purification process right, we’ll find out that prions are nothing more than teensy, weensy little viruses.
  
  We live in hope.
  
  ANYWAY. Not surprisingly, given the fact that we still don’t agree on what even causes them, prion diseases are incurable; the ones that have reached epidemic levels, like scrapie and kuru, have gone away only because they burned through their original hosts and the practices that led to them were discontinued, so there are no fresh victims. This made The Family that Couldn’t Sleep a downer, because Max goes through all the horrible, HORRIBLE symptoms these poor people have to deal with before their early deaths, and then…yeah, there’s no cure. Not even the hint of a cure. Not even the whiff of a cure. And although there’s now a test that can predict who’s a carrier (that is, an eventual victim), and although most of the family has taken that test, everyone has refused to learn the results. Which I can totally understand, because that’s not something I could live with knowing, either, but at the same time? The chances of passing this on are 50/50, which means that the younger members of the family who choose to have children will give it to at least some of their babies.*
  
  BUMMER doesn’t even begin to describe it.
  
  The Family that Couldn’t Sleep is fascinating, albeit depressing, but it is hugely, HUGELY problematic. To begin with, Kuru is an illness that devastated certain tribes in Papua New Guinea in the decades immediately following WWII. The people trying to cure it? Were either colonial Australians (they got the territory as a “gift” from the defeated Germans) or Americans. In fact, the chief American investigator on the scene was always going on and on about how great and unspoiled and untouched the “wild” places were and blah blah blah, racist bullshit. Max makes some efforts to show that these attitudes were BAD WRONG BAD WRONG, but usually he’s just very quietly ironic, or he’s silent on the subject altogether.
  
  Horrifyingly enough, however, the racism isn’t the worst part. Guys, I know that we disagree on a lot of things as a society (or societies). I know that we have conversations about what constitutes racism and sexual violence and sexism and whatnot, but I thought we were ALL AGREED that having sex with under-aged boys was just plain wrong. There’s some debate about under-aged girls because we suck at being human beings, but we were ALL AGREED that LITTLE BOYS WERE OFF-LIMITS. And yet! One of the big early researchers in the field of prions was a man named Daniel Carleton Gajdusek. Daniel Carleton Gajdusek? Was a self-admitted pedophile who was later convicted of molesting one of the multiple boys he “adopted”; oh, and he just loved spending time in Papua New Guinea studying Kuru, because some of the tribes there had cultural traditions of pedophilia, and that shit was RIGHT UP HIS ALLEY.
  
  And Max doesn’t condemn him. In fact, the way that he talks about Gajdusek’s “sexuality” is just…ugh. It’s very noncommittal, and at times he almost treats it like it’s a joke. Hahahahaha! He touched a little boy’s weiner! How amusing! Max treats Gajdusek as kind of a loveable, sad little joke, and frankly? The man was a predator. He may have done great things,** but he’s still a goddamn predator. Be honest about that.
  
  Recommended for: Anyone who really, really enjoys reading about diseases and can stand all the FAIL.
  
  *Individual members of the family have taken steps to avoid passing the gene on; one woman waited to have children until her own mother was past the age where the disease was a possibility (it strikes by or before middle age). Once her mother had made it through the dangerous age without showing symptoms, the woman had children. Another woman, whose father had already died of the disease, chose to adopt; that was probably a good decision, because she later succumbed as well.
  
  **And whether he accomplished very much at all is EXTREMELY debatable. Another thing I got from The Family that Couldn’t Sleep? It’s easier to get a Nobel Prize than you’d think. Gajdusek got one, and I still can’t really figure out why. He wasn’t even the one who established that what was killing kuru patients wasn’t a virus; as far as I know, he just ran around collecting brains and bodily fluids and shooting up chimpanzees and waiting to see if they got sick. I don’t get how that is so stupendous.
• 
  Coral

  Boy, this was SCIENCEY. As someone who is not a scientist, this wasn't totally user friendly. Interesting and sad though. Some real life terror.
• 
  Kristin

  Sadly, I waited a week to write my review, when I should have written it immediately upon completing the book when I was full of emotional and intellectual reactions to the ideas contained in the book. With that said, I HIGHLY recommend it, as it's a fascinating read, and despite the topic being rather academic, it reads almost like a novel. Very well written.
  
  The book is basically about protein diseases (almost all of which are brain-based, if not all?). It focuses on one where the prion protein malfunctions, attaches to other prion proteins, making them malfunction, and you end up with people being unable to sleep (where they eventually die from the lack of sleep). The author does focus a lot on the one particular disease (FFI), but he uses it to explore larger medical implications for treating, and diagnosing, all disease. Really on the level of the discovery of bacteria and viruses. I'm aware, like everyone, of viruses and bacteria and their role in disease. But I have never heard about the role proteins can play (i.e. prion protein diseases can be hereditary, infectious and spontaneous -- examples are scrapie in sheep, and mad cow disease more recently in Europe and the US).
  
  On a very personal level, as someone with a brain disease (hereditary, chronic migraines), and with recent research in the field of how addressing proteins could prove a treatment for migraine prevention, it's absolutely fascinating to read the book. The author, himself, has a neurological disease, and this personal connection to the content of the book really speaks to readers with neurological diseases. For example, he notes, "We are by nature sick animals and the fabulously long, healthy lives many of us are living today are unnatural." For someone with an incurable, disabling disease (meaning you often feel like a huge financial and physical burden on all of those around you), living in the West, largely surrounded by healthy people, this historical, and even modern worldwide, context brings enormous comfort.
  
  The author also, at the end of the book, describes what it's like to personally live with a neurological disease, and be treated by doctors for it. "Some doctors say that neurology attracts the unusually shy or nerdy, medical students more comfortable with interpreting the results of a patient's EMG or MRI than talking to him or her about what he or she is experiencing. But I believe the reason no one looks you in the eye during a neurology exam [he refers to when the neuro does the physical exams, having you walk a straight line, does reflex checks, etc.] is shame - the neurologists's shame. The fact is that the neurologist can diagnose you but he can't CURE you. For him, it is still 1860. Almost 150 years later, he still has nothing more to offer than the accuracy of the clinical gaze." He also notes, "When I leave, the neurologist suggests I come back in a year or two. Sometimes I do. Sometimes I don't. Sometimes I go to another doctor instead, to see if something more can be found out." These descriptions get right to the heart of how it feels, as a patient, to be treated for a neurological disease.
  
  Regardless of my personal reactions to the content of the book, relating to my own medical health, the book does a brilliant job describing medical facts, medical history, the science (physics and chemistry) behind disease, all making what could be incredibly difficult-to-understand material accessible to the common reader. If you're not reading this book, you're missing out on being aware of what is likely the CENTER OF DISEASE RESEARCH of our times.
• 
  AdiTurbo

  Excellent non-fiction that reads like a thriller and gives you a good review of the history of prion research, the development and spreading of prion diseases in animals and humans and the scientific aspects of the discovery and treatment of prions. It's fascinating, super-scary and even moving. Daniel Max the writer is emotionally invested in the issue and it shows. The writing is far from dry, and is full of compassion towards the people who have suffered from these diseases and their relatives who have to live with the grief and the fear of developing the disease themselves. A great read.
• 
  Alemanita

  Muy, muy interesante, aunque ahora creo que voy a morir de una enfermedad priónica.
• 
  Rossdavidh

  Subtitle: A Medical Mystery. The family of the book's title has a curse worthy of a novel from Poe or Lovecraft. Around middle age or later, apparently healthy men and women (going back at least six generations) will first notice that their pupils are constricted to tiny points, and that they have trouble sleeping. As the disease, for which no treatment is known, progresses, they will become ever more desperate for sleep, and unsurprisingly (perhaps in part as a result) less and less sane. The disease takes months to kill its victims, but is 100% fatal, and the family members who stay by their side see them disintegrate (mentally, and to a certain degree physically) before their eyes, but with agonizing slowness.
  
  Welcome to the world of prion diseases. Sometimes an inherited condition, sometimes introduced by environmental factors, what prions have in common is that they are not alive, have no DNA, are inheritable or even infectious, and are poorly (if at all) understood even today.
  
  But D.T. Max, the author, is just getting started. Woven into this tale are government incompetence on a scale large enough to get people killed, deception, backstabbing, pedophilia, and cannibalism. Incredibly, instead of being sensationalist, all of this lurid material is crucially relevant to the tale. Which, by the way, is told by an author who has a similar condition.
  
  D.T. Max's condition is nonfatal, but it shares several features with mad cow disease. scrapie, kuru, and the "fatal familial insomnia" that has a grip on the family of the book's title. First, it is related to how proteins fold. We have a tendency to think of molecules as being defined by the number and kind of atoms they have. But, just as DNA would not work if it were not in it's particular double helix shape, many proteins have to have the right three-dimensional folding pattern or they won't work properly.
  
  Secondly, it is not terribly well understood. Just as it took medical science longer to understand viruses than to understand bacterial infection, because the virus is DNA without a cell to speak of, so protein-misfolding diseases are not just new kinds of diseases. They're a different class of disease entirely, and this makes much of what medical doctors know about how to treat disease useless in this case.
  
  Explaining all of this takes a while, and could easily have been tedious. Max does a good job of interspersing the technical stuff with tales of the human side of the topic, hopping back and forth between the family of victims in Italy, and the exploits of researchers in places like New Guinea, where cannibalism led to prion disease called kuru. The struggle to understand how this disease worked took a generation, and (frustratingly) came too late to do any good, as the population had ceased the ritual cannibalism which was the source of the disease for cultural and religious reasons long before the link between it and kuru was discovered.
  
  The history of government attempts to cope with, and in some cases to suppress knowledge of, scrapie (in sheep) and mad cow disease, is frustrating as any tale of government incompetence. Max makes that point that, if mad cow had been even slightly easier to catch than it was, the number of dead could have been orders of magnitude higher, given the widespread consumption of beef in England and elsewhere, the near universal use of the techniques (such as feeding sheep or cows to cows) which caused the disease, and the slowness of the U.K. government to admit that there was a serious problem.
  
  The biggest names in the field are drawn to life fairly vividly, as well. One is a great field researcher, who could get more information from the Fore tribe of New Guinea than any other westerner, but who ended up in jail for pedophilia (over 50 preteen natives from various Pacific Islands ended up living at his house in the U.S., though the great majority never accused him of anything improper). Another American essentially forced out any younger researcher who wanted to do prion research, and is credited with essentially monopolizing research money in the field. Both were conspicuously ignored by British researchers trying to understand mad cow disease when it was clear to outsiders that they needed help, apparently because of a desire not to have non-U.K. researchers learning too much about the seriousness of the situation (and spilling the beans to the press, hurting British beef sales).
  
  More than anything else, this book is the story of people dealing with the unknown. In part this is because much to do with these diseases is STILL unknown, though clearly we understand a lot more than we did a generation ago. Tales of scientific and medical progress often focus on the ones who sorted it out and made it clear to the rest of us, and this can give the false impression that that is how things normally work in research. In fact, just as often or more, there is more unknown than known, and when that which is unknown is killing people, it increases the frustration.
  
  It takes a special kind of integrity to say, "we don't know", when the question relates to why people are dying, because that inevitably means we don't know how to cure or prevent it. The family of the book title refused to talk of the way their older members died, for generations, because no treatment was known and therefore admitting what was happening was too terrifying. However, as Poe and Lovecraft knew, what is unknown and hinted at is more terrifying than what is plainly seen. This is a story of people who resolved to look squarely at the unknown and lethal, regardless of the frustration and risk. If you don't know how the disease works, how to you protect yourself from being infected in the course of your research? It is dark material, brightly told, and a great read from beginning to end.
• 
  Rose

  I enjoyed this book, but there's one part of it I don't understand. If you can point out what I'm missing please do so - it's been a while since I studied genetics.
  
  The book says that the gene encoding the "prion gene" involved in CJD/kuru/FFI has two alleles. One codes for a methionine at a particular site and the other codes for a valine in the same position. If you have two copies of the same allele, one maternal and one paternal, you are homozygous. If you have one of each kind, you are heterozygous.
  
  Apparently, "homozygotes were at higher risk of prion disease than heterozygotes [this appears to go for both val/val and met/met] and there were more heterozygotes in the British population than chance would dictate." This led the researchers involved to suggest that "some evolutionary force might have favoured heterozygotes over homozygotes".
  
  Backing up this suggestion would is the fact that "heterozygotes were overrepresented worldwide, in every race and every ethnicity". (Though Max also states at a different point in the book that "Japan is terrified of BSE because its population's genetic makeup is heavily homozygous," which doesn't seem to agree with the previous statement.) This lead to the conclusion that "their common ancestors must have faced a situation that winnowed out the homozygotes".
  
  If both parents are heterozygous (both val/met), according to classical genetics, 25% of the offspring will be val/val, 25% met/met and 50% val/met - since it appears that both variants of heterozygosity (val/val and met/met) result in vulnerability, that means half of the offspring will be homozygous and therefore vulnerable, and half heterozygous - exactly equal proportions.
  
  So how can you winnow out the homozygotes and leave your population full of heterozygotes? Whenever you have two heterozygous parents, half of the offspring will be homozygous. You might see a pattern in which homozygotes for a particular allele are underrepresented in a population because they have an increased risk of foetal nonviability, but there was no suggestion in the book that this might apply in the case of the prion gene. It seems like the homozygotes are generally healthy unless they are exposed to a prion disease or also inherit a prion-creating mutation, as in FFI.
  
  Like I said, it's been several years since I studied this, so maybe I'm off base completely. I'd appreciate any comments that could clear things up.
  
  Also, Max states that "In World War I, Italy had entered the wrong war on the wrong side at the wrong time." OK, so Italy didn't do well out of the Great War...but it was still on the winning Allied side, right?
• 
  Jamie Collins

  An interesting book about the discovery of the cause of prion diseases, such as “mad cow” disease and the fatal familial insomnia which afflicts the titular Family. The book meanders around quite a bit, and ends with a chapter about the author’s own neurodegenerative disorder.
  
  We learn the history of the Italian family whose members are at risk of dying a horrible death in middle age - long after they have already passed the genetic defect on to some of their children. It’s a little disturbing to read that in the 1990’s they preferred to be kept ignorant of the results of genetic testing.
  
  The author profiles two Nobel Prize winning scientists who were early investigators of these brain-destroying diseases. It’s slightly bizarre to read about Carlton Gajdusek, an unrepentant pedophile who worked to find the cause of kuru in Papua New Guinea, which was possibly spread by cannibalism. Then there’s Stanley Prusiner, who came up with the catchy name “prion” to describe the infectious agent - and who was anxious to hoard research credit for himself, and warned his graduate students not to go into competition against him.
  
  Prions are pretty scary. You can’t make infected meat safe by cooking it or irradiating it. You can’t remove prions from surgical tools using normal sterilization methods. Eventually the author gets around to describing, at a very elementary level, the mechanics of prion infections (still debated by some scientists) and how the disease can be both inherited and infectious(!). He discusses the mad cow epidemic in Britain, which was badly mishandled by the government and could have been much worse if the population of Britain had a different genetic makeup - he says that the general population of Japan, for instance, would be much more susceptible to mad cow disease.
• 
  Barrita

  Wow.
  
  Ya había estudiado los priones antes y por supuesto el Insomnio Familiar Fatal es algo que se estudia siempre en Psicofisiología cuando se tratan temas de sueño. Me habría gustado tener este libro entonces, porque de forma sencilla describe lo básico que hay que saber sobre el tema.
  
  Habla de lo que son los priones, las historia de su estudio y las enfermedades que se pueden producir por ellos, tanto en animales como en personas. Todo llevado con el hilo conductor de la famosa familia italiana que ha sufrido Insomnio Familiar Fatal por generaciones.
  
  Mi única queja es que, quizá por una forma descuidada de escribirlo, el autor parece implicar en ciertos puntos que las enfermedades prionicas son culpa de los seres humanos y su curiosidad, como si la selección artificial fuera la cruel respuesta a meter mano en la naturaleza. La cosa es que la selección natural es igual de cruel, pero mas lenta. Los resultados inesperados que se han encontrado al practicar la selección artificial en todas sus formas solo son una muestra de que hay muchos factores en juego en la formación de un ser vivo. Colgar culpas me parece que está de mas.
  
  Fuera de eso el libro es bastante bueno, aunque se me aterrorizó a mi que ya conocía un poco de priones, no sé que hará por alguien que no los conozca. No están vivos, solo son proteínas deformes. No son afectados por radiación, frío, calor ni productos químicos, asi que no hay forma segura de eliminarlos. Ni el tiempo los afecta: hay contagio incluso después de años de que los priones estuvieras aislados. Las enfermedades prionicas se presentan en las tres formas en que puede darse una enfermedad: por factores genéticos, por infección y de forma accidental o esporádica. Y básicamente se comen los cerebros.
  
  No sé que están haciendo los escritores de terror escribiendo sobre virus y bacterias.
• 
  Emma

  I first studied prion disease in a virology class during my last year of university. I must have enjoyed them because it was one of the only topics that kept my attention and prevented me from daydreaming like I usually did during that class. Because of this, I was familiar with the disease FFI (fatal familial insomnia). I find it so interesting that there are people who literally can't sleep. I know insomnia affects millions and decreases quality and quantity of sleep, but sufferers of FFI literally can't sleep because infectious proteins destroy their thalamus, a part of the brain that is responsible for circadian rhythms and other primal functions (body temperature, thirst, etc.).
  I liked how the author spoke to the reader as they already had some knowledge of virology and pathology - I didn't feel like the language had been dumbed down, but it was also easy enough to understand and follow.
  
  The molecular part of the prions is interesting, but I enjoyed the epidemiology parts the best - following the pedigree of the Italian family plagued with FFI, mapping the CJD and BSE outbreaks across Britain, and discovering the mystery behind Kuru.
  
  One of my favourite parts was reading about the contention between Gajdusek who studied Kuru on site in Papua New Guinea and Stanley Prusiner who studied prions back in the States. I actually found it quite funny that they hated each other and sabotaged each others' work. It brings more interest to the (sometimes) dry world of science work.
  
  The book gets 5/5 stars because it was incredibly interesting and enthralling, and taught me a lot more about prions. I wish I read it this time last year because maybe I would have paid more attention in virology class.
• 
  Chloe

  I will not forget this title soon. The horror of the familial genetic sadness of this disease is momentous. As we have begun to decipher and advance in genetic medical research it becomes clear to me that genetic disorders define us as humans to huge degree. If we can learn more - we will create more treatments and cures.
• 
  Lisa

  This was really interesting. It was all in layman's terms and easy to follow. I feel like I learned a lot from this book. It balanced medical theory, fact, and case examples well. I found the FFI information really fascinating. CJD made me a little nervous, I'm afraid to say...
• 
  Emma

  I took a break from nonfiction for a while but WE ARE BACK BABYYYYYYY!! This book has revived my love for nonfiction, it gave me everything I love in the genre, which is taking one specific topic and then showing how it’s related to many other bizarre and fascinating things.
  Overall this book is about prion diseases, diseases caused by a misfolded protein. This topic is not boring, because the history of investigating this type of disease has led various researchers through islander peoples who maybe were cannibals but maybe weren’t, illegally smuggling Merino sheep for their superior wool, mad cow disease, a researcher who was an out and proud pedophile who got taken down by the FBI and then likes prison more than his normal life, scientific theft, a horse racetrack disease outbreak, a detailed chronology about how England bred better sheep for meat, and other little threads around the world. I eat this stuff. The more obscure and irrelevant to me, the better. I just love hoarding random little facts and stories.
  My main complaint is that the use Italian family from the title of the book was strange. The beginning was great, setting the family up as a framing device to show how this kind of disease impacts real life and how the search to identify the disease began. The family’s story was returned to throughout the book, but the way their story was wrapped up was strange and anticlimactic. There is no cure and there’s not much they can do about their situation, but their story felt randomly abandoned near the end rather than given a well-written reflection and conclusion.
• 
  Melissa

  I'm not sure whether this book makes me afraid of not being able to sleep or of eating beef, but it definitely makes me afraid of something. A comprehensive look at prion diseases, it starts with the Italian family who the book is centered on. A family that is tragically afflicted with FFI (Fatal Familial Insomnia). Essentially, this poor family has folks that start to be unable to sleep once they hit around fifty or so, and this progressively deteriorates their health until they die (except for a few unlucky who die sooner with more erratic behaviors).
  
  Max looks into the science and various researchers who not only looked at this family's case, but also similar diseases that were later found to be a part of the same type of issue. Mad Cow Disease, creutzfeldt-jakob disease, and others. One of the shared concepts for many of these prion disease (for those that aren't inherited at any rate) are that cannabilism, regardless of intentioned or not, is probably not very good for your health. And this holds across species.
  
  This is a science-laden book. There are a lot of medical terms and concepts that are not always spelled out for a casual reader. As such, it can be hard to follow at times. I also, while appreciating the wide array of prion science available, wish there had been more about the family itself. The premise and title of the book indicated it, but it really was more about prions in general.
  
  Interesting, but also terrifying, this is not a book to read if you already try to self-diagnose yourself quite a bit. Also not a good book to read if you enjoy eating beef.
  
  Review by M. Reynard 2024
• 
  Gilda Felt

  I vividly remember when the mad cow scare started. No one knew what it was or where it came from. I think most figured it was like AIDS, an old disease that had recently jumped from another species. But then more was brought out about kuru and scrapie, and it became apparent that, whatever it was, it had been around for a long time.
  
  That history is explored here, starting with fatal familial insomnia, which I had never heard of, to the chronic wasting disease which is decimating deer herds, which, because of where I live, I’ve heard plenty about. It’s a fascinating, though terrifying history of what would be come to be known as prions, proteins that “fold” themselves into these devastating diseases. It’s a hard book to read, yet a hard book to put down.
  
  
• 
  Lorie

  Such an odd book. It was a little messy in the way the information was organized, but I found it to be absolutely fascinating. I learned a lot, that’s for sure. If I could just find someone that would love to discuss rare and absolutely terrifying illnesses over a cup of tea I’d be in good company.
• 
  David

  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 reasonably 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). 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.
• 
  Gabriel Avocado

  interesting read. its actually a misleading title: this is more about a history of prion diseases than specifically about that family suffering from an incurable insomnia. i love pop history books but i wish they wouldnt gloss over a lot of the awful racist imperialism in medical history. this book also downplays heavily the fact that one of the men critical to the discovery of prions was a pedophile and kidnapped (because thats what it was) preteen polynesian boys to rape in the US, where they were treated with the utmost contempt and hatred. but i guess he was a good guy because he did science stuff.
  
  things like that really anger me because i dont ask for perfection in doctors since theyre humans too, but its as if his victims, indigenous children, were more to blame than him. after all, what have natives, particularly native children, ever done for european society? and this book really tries to act like australias imperialist venture into papa new guinea was anything but an open land grab. they helped spread the racist myth of the cannibal native, something the book itself admits but is too cowardly to condemn.
  
  all in all, it was an interesting history that i could appreciate but the racist and rape apologia was a lot. how can we write about medical history if we dont also examine, criticize, learn from, and condemn the negative aspects of past medical professionals, INCLUDING the racism inherent in western scientific practices? guess im just being nitpicky!