Steve: Corey, our guest today is an old friend of yours, D.T. or Dan Max. I’ve heard you talk in the past about a nonfiction science book he wrote called The Family That Couldn’t Sleep, which is about a family with a genetic condition that makes them vulnerable to a kind of prionic disease that makes them insomniacs and eventually kills them. I think for our readers who haven’t read the book, maybe you could talk a little bit about why you think the book is great and why you recommend it, and what are the special qualities of the book.
Corey: I think the book is really stellar for a couple of different reasons. It’s a kind of a mix of genres. I’m a fan of certain kinds of literary fiction, and I think, unlike a lot of what’s called popular science, this book actually has a pretty serious literary quality. It’s incredibly well written, the narrative is very, very strong. It really reads like a novel, a high quality novel, and also a detective story, knowing also that detective fiction often isn’t extremely high quality. But Dan’s a wonderful writer, and I actually pay a fair amount of time to his sentences and how he paces the book. And that combination of writing a really good science story in a narrative form is probably its great strength.
Steve: So has it been compared to In Cold Blood by Truman Capote, which you probably are familiar with, right?
Corey: It has. When I think about this book, I think what’s hard is it’s in a genre where people often don’t think much about really good writing. People just think of a pop science book as taking an idea and making it accessible to the public, so there’s really very little effort of trying to make a book a work of art. And I think that’s what he’s really succeeded in.
It’s also, I think, a story that’s really in the middle. This book is about discovery of the causes of the disease, prions, showing they look like they’re actual infectious agents. But there’s a whole other half of the story, which we’re still in the midst of, which is we actually don’t know how to stop these diseases. This book was written about 20 years after mad cow disease first hit Britain. It wasn’t actually really noticed for a few years later, but we’re now about 13 years after the book, and not much has actually happened. So I want to check in with Dan to find out about what he’s seen in the research, and also check in with the people he spoke to as part of this discussion. That’s one side. I think that’s why I think I like the book as a whole.
I think it’s also really fascinating just how he executed it, because he’s got some very difficult matters to tread here. He’s got a family that has a disease and that’s suffered a lot through the disease, but also suffered from the publicity that’s happened. They were basically harassed in their own community. And he’s got two scientific characters who aren’t terribly sympathetic, one of whom’s a pedophile. And how do you write about that, when someone so central to your character is so morally problematic? I spent a lot of time reading the book trying to figure how is he going to deal with this guy.
Steve: I never read the book but I’ve read a lot of Max’s articles in The New Yorker over the years, and I agree with you, he’s a really fine writer and tells a good story. Are there any other scientific facts you’d like our audience to know? Maybe about what prions are or mad cow disease, bovine spongiform encephalitis?
Corey: So prions are really… Recognizing their existence is really a fundamental paradigm shift in our understanding of infectious agents. Since Pasteur, we assumed that things that infect you have to be alive, they’ve got to have DNA or at least RNA, and so they get inside you, they multiply, and they start causing havoc, and you can kill them by finding agents that kill living things. It took a very long time for people to realize that prions weren’t like this, that there’s a whole different category of infectious agents. In that way it’s a classic scientific story of a real fundamental change in paradigm and how hard it is, reasonably, to get people to accept that, because you don’t want people dropping paradigms. It took some painstaking work.
Steve: If I recall properly, proteins are produced from RNA and they’re long and linear when they first are produced, but then they have to fold up and —
Corey: They have a conformation.
Steve: Yes, and one of the mysteries is exactly how nature comes up with proteins that unerringly fold into the right functional shape. Here we have misfolded or malformed proteins, and somehow they’re able to cause other proteins to then assume that wrong shape, and with eventually downstream health consequences.
Corey: Yeah, it’s a remarkable phenomenon. You bump into something in some complex way, and it kind of becomes you. I think that’s something that people had a very hard time accepting, as much as they were not DNA or RNA based. But yeah, the question was how it was transmitted actually. I think there’s been increasing understanding of that over time. But again, that’s also just very basic research that doesn’t quite tell you how you stop them, and that’s something that, as Dan underscores in our talk, we haven’t made a whole lot of progress on.
Steve: And as a general rule, maybe we should not eat the brains of our own species nor feed the brains of another species to those creatures. Is that a good rule of thumb?
Corey: I remember being offered brain about 30 years ago in Managua. I think I turned it down then, and I think I’d probably turn it down now.
Steve: Okay, very good. Well, on to D.T. Max.
Corey: I’m Corey Washington.
Steve: And I’m Steve Hsu.
Corey: Our guest today is Daniel, D.T., Max, staff writer of The New Yorker, author of The Family That Couldn’t Sleep, which is our topic for today, and Every Ghost Story is a Love Story: A Biography [Life] of David Foster Wallace, and also of numerous articles in The New Yorker that I read whenever they come out. Welcome to Manifold, Dan.
D.T. Max: Thanks very much. It’s Every Love Story is a Ghost Story.
Corey: Did I screw that up?
D.T. Max: I think you had it backwards, but maybe not. It’s reversible so it’s true, but —
Corey: Let’s get a little background. How long have you been working at The New Yorker? And can you describe the types of pieces you write there?
D.T. Max: Yeah, I’ve been a New Yorker staff writer probably for about 10 years now, and before that I did a very similar job at The New York Times Magazine. I’ve been a feature magazine writer for most of my professional life. I do different stuff. I’ve been one of those people who just cannot stand to get cornered, so I’ve written about artists, I’ve written about scientists, I’ve written about science, I’ve written about cartoonists recently, before that I wrote a piece about Oprah Winfrey’s book club — I’m just going back years here, back and forth — I write about Italy often. I did a piece recently about the international — this is going to maybe sound more boring than it was — but the international economics of the Chinese garment industry in Prato, Italy. I don’t know what you’d call that piece, it’s a labor piece in a way.
Steve: Was it about fast fashion?
D.T. Max: It was actually. It was about fast fashion, and about ways in which Prato had essentially become kind of a Chinese city, and about both the culture and economics of that. I went along on a couple of police raids where they chased the Chinese workers who don’t have proper papers and that kind of thing. So I do different stuff.
And in fact, if you look at the two books, The Family That Couldn’t Sleep is a science book — what in England is called, I think without meaning to be dismissive, popular science. Here that has a little bit of an undertone, but there they just call it a popular science book. And then Every Love Story is a Ghost Story is a biography of the writer David Foster Wallace. So you can see in there there’s no branding.
Corey: When I describe your writing, I tell people you’re a literary nonfiction writer. There’s a well-known genre of literary fiction — people like Tolstoy, Dostoevsky, Camus, Marquez — and then there’s journalism in pop science. But your writing has a deep literary quality, and The Family That Couldn’t Sleep reads a lot like a novel.
D.T. Max: Yeah. I think that it’s true, and in fact in the academic world now, literary nonfiction is almost an accepted category for instruction. But of course it’s very hard to say what exactly is literary nonfiction. Back in the ’70s, nonfiction writers employed the techniques of novel writers to invigorate the field in so-called “new journalism,” but the journalism tended not to be that accurate. They went in much further than I would ever go in terms of using novel aesthetic techniques. But the things that novels are interesting for, which is character, change over time, the telling detail, those are the things that have always been most exciting for me.
In the case of The Family That Couldn’t Sleep, this is the book about something called the prions disease. I remember saying to my editor when I first began writing about the subject, which I did initially for a New York Times Magazine article, I said, “At last I found a disease that has character.” In other words, I never felt that I was somebody who was going to be able to write… There’s a book called The Genome: (I’ve got this slightly wrong) The History of Genes in 23-and-a-Half Chapters [Genome: The Autobiography of a Species in 23 Chapters], do either of you know that book?
Corey: I’ve heard of it.
D.T. Max: Yeah. Anyway, the point is it’s Matt Ridley. I could not do that book. I admire that book. But I needed a disease principle that was as weird and interesting as, say, viruses were seen to be 100 years ago. I needed something that itself could be given a personality. In fact, years ago I wrote a piece about a marvelous chef named Grant Achatz, who has tongue cancer. It was a piece about how he — this was for The New Yorker — how he had elected not to have his tongue removed, in order to maintain his ability to taste. In that original draft of that story, I made cancer a character, almost like a person, searching for his weaknesses and finding occasions to grow. I toned it down in the end, but that’s always been a prerequisite for me in writing a science piece.
Corey: So you kind of answered two questions I was planning to ask you, which was —
D.T. Max: Ask them anyway.
Corey: I know in the case of Every Love Story is a Ghost Story that there was a trial-balloon article. You wrote a short piece for The New Yorker — actually I don’t know if it was short — and then that later —
D.T. Max: Long.
Corey: Yeah, then that later developed into the book. And I was wondering whether there was a similar, I don’t know if you’d call it a trial balloon, for The Family That Couldn’t Sleep.
D.T. Max: Yeah, it did begin with an article, it began with an article in The New York Times Magazine. This is in the early days of the web — I think it’s 2001, March of 2001 — and even then you could see the response. It’s weird. Before the web, it was very, very hard to know whether an article you had written had gotten any response or not. It took so long for the responses to get to the magazines and newspapers. But by 2001, you could get a sense that something was interesting people. And of course, by the time I wrote the article on David Foster Wallace, that was considerably further along.
But one reason you would do it this way was really financial, which is that it costs a lot of money to do reporting. I’m not making a pitch here for donating to various nonprofit reporting institutions, but if I can, I would. It takes a lot of money. You had to send me to Italy for The Family That Couldn’t Sleep, and it wasn’t really something I would have been able or inclined to do on my own. And then you get the professional editing, you get people who can tell you where the story is. It’s not something every writer always knows, and I don’t think I knew it exactly.
I remember the beginnings of — this will be your third question but I’ll answer it now — how did this story actually begin? So it begins this way, it’s wonderfully humdrum, which is that The New York Times Magazine was doing an issue on diseases that cross borders, and I was considering going on the Hajj because the Hajj is apparently a very high-infection environment. Any environment where people are crammed together, there’s a whole lot of disease. And I don’t remember why that didn’t happen, but I typed into the internet, in the web — maybe I used AltaVista for all I know — “world’s most terrifying disease” or something. Nowadays you really can’t do that: there’s too much on the web, you’d have to dig and dig and dig and dig. It doesn’t work anymore. A paper published in The New England Journal of Medicine came up about something called fatal familial insomnia. That’s actually how I first heard about it was just by typing it into the web. Again, I don’t think that works anymore. There’s so much on the web now, you really have to have the ability to sort material in a way that you didn’t have to have in 2001.
Steve: So at that moment were you already aware of mad cow disease, and was it an accident that the thing you found was a prion-related disease?
D.T. Max: Yeah, absolutely. I don’t want to claim more talent than I possess. As Corey points out, fundamentally I have to relate things to what is most central to me, which is — if you want to condemn me to literary nonfiction I accept the sentence — but it’s some sort of nonfiction that is more than the sum of its data, let’s put it that way. So in this case, the title, fatal familial insomnia, immediately of course makes you think of things that are very different from disease techniques. It makes you think about families right there. Now that’s just the happenstance of how they named it. As one of the people who named it said to me, “We could have called it familial fatal insomnia, but it didn’t sound quite as good.” They were aware even then that the naming of the disease would be useful in getting it publicity, frankly, or attention, because it’s rare. It was rare when I wrote about it, and it remains rare now. It was only later, as I began researching, that its connection to mad cow disease became clear. And also the fact that prion research, simply put, attracted the most interesting researchers in the world for a while. There’s nothing like the story of Carleton Gajdusek, who we’ll get to a little bit later.
Corey: I think I’d heard about a prion disease back when I was about 8. My favorite book at the time was The Guinness World Book of Records. In 1974 the rarest disease in the world was laughing sickness, and it arose from cannibalism.
D.T. Max: Yeah, kuru.
Corey: Kuru, exactly, yeah. That was, again, one of the —
D.T. Max: So that’s in The Guinness Book of World Records?
Corey: Yup, ’74 edition. It’s back when they were big and fat.
D.T. Max: Yeah, I had that book.
Corey: Yeah, they’re fantastic.
D.T. Max: Remember the man with the world’s largest mustache?
Corey: Of course, of course. And back then the heaviest guy in the world was Robert Earl Hughes, who was 1,069 pounds, and he’s about 700 pounds lighter than the most recent heaviest man in the world.
Steve: And he was buried in a piano case as I recall.
Corey: That’s right.
Steve: We all read the same book. [laughter]
Corey: This was your first science piece, is that right?
D.T. Max: No, I don’t think that’s quite true. I’d actually have to think about it. It was certainly my first piece on prion disease, but I think I had done science pieces. You also need to know that I myself had developed a rather nasty neurodegenerative disease shortly before, and so I was much more interested in disease than I would have been, I think, otherwise. I don’t remember how old I was — I figure I was 40 — and so I was in, I wouldn’t say a morbid frame of mind, but I was very open to the question of disease and trying to process, I think for myself, whether I was uniquely afflicted or just on the road we were all on. The answer is obvious, but it’s really how you emotionally experience that information. So the last chapter of The Family That Couldn’t Sleep is actually a little bit of biography. It’s a little memoir about my getting a diagnosis at Columbia Presbyterian Hospital. Everyone loves memoir, but I didn’t want to let people cheat and skip ahead. So I think that that chapter is called “A Note on the Author,” so that you would expect it would just be “D.T. Max lives in New Jersey with his wife and two children” but it’s actually a whole chapter.
Corey: I remember you’re hinting at it in various parts in the book, and you do have the last chapter focused on it. It’s funny, because you and I experienced this book in slightly different ways: you have a neurodegenerative disease, and I’m a chronic insomniac. And it’s funny because I read some of your descriptions about how sleep naturally takes its course, so if you’re just eventually tired enough you fall asleep. And that’s not true for insomniacs. There’s a separation between fatigue and sleepiness, and you get to the point where you can just be dead tired and your brain just won’t fall asleep.
Steve: Corey, I think for the audience, I would suggest that you explain what’s in the book and what the disease is all about. I think not everybody’s read the book.
Corey: That’s right, so I’ll leave it to Daniel to tell us a little bit about the disease.
D.T. Max: You’re punting. Okay, I told you how it started, so this is what the book is. After that first happy foray into the internet, I called a neurologist in Chicago, who tended to have interesting cases. So I asked him if he’d seen any interesting cases, and he told me he’d just had a patient who had died from insomnia. And I told him that I thought that was impossible, that nobody’s ever died from lack of sleep. And he said, “That’s what you think. There is such a disease.” I realize this is slightly inconsistent with the previous story, but they are both true. So then I said, “I really want to come out to Chicago and meet with you.” There’s nobody as avid as a journalist who sees a good story. And he said, “Wait, wait, wait, wait,” and my heart was sinking, I thought he’d tell me he couldn’t. But he said, “There’s a family in Italy who have had this condition genetically for 200 years.” And I said, “Well, where in Italy?” And he said, “Venice.” And immediately I thought death in Chicago? Death in Venice, that sounds like something, and I went and got my passport.
So, what’s the book about? The book is about prion disease. A prion is a weird infectious protein that behaves like a virus, so you can transmit a prion and cause prion disease without any of the traditional RNA or DNA substances which we have always understood to be able to cause disease. This is a book which focuses on that Italian family and covers that disease from 200 years ago to the present, how they deal with the disease, how they learn about the disease, how it changes their lives, how it doesn’t change their lives. And then, finally, the book is really also about the way in which prions are relevant to all of us, because the principle of the prion, which is a protein that misfolds and causes disease, is relevant in other conditions, like Alzheimer’s and Parkinson’s, conditions that are vastly more common than prion diseases are.
In England the book is called The Family That Couldn’t Sleep: Unravelling a [Venetian]Medical Mystery, and I actually think that’s a better title because it really is a search. The book is a search. I search with the family to try to figure out what on earth is happening to them. Then I take you into the science and what’s known about the science, and then finally that these are things we might eventually learn from prions.
Steve: Is it known in the case of this Italian family how the intergenerational transmission takes place? Are they passing the prions down to their kids, or is there a genetic condition that they’re passing on?
D.T. Max: Yeah, it’s a single point, a genetic point mutation. It’s a very simple error in genetic code. And they’re not the only family, but it’s fairly rare, extremely rare. I think it’s one in a million is the instance of a genetic mutation.
Steve: So because of the mutation, their bodies tend to produce misfolded proteins? Is that how it works?
D.T. Max: Yeah. There’s also an age component, so even if you have the gene, you generally won’t show any symptoms until mid-50s — 50, 55, 60, that’s the typical age of onset. I think the reason for that is probably just that the body does everything less well as you get older, and so you’re producing proteins that are less exact and your body is less good at clearing deformed proteins through the lysosomes. That’s the probable reason. It’s not as if the gene is only activated then.
Corey: So there are a couple strands throughout your book. One is the story of this family, and then there’s the scientific story of discovering and understanding the operation of prions, and then they come together when you begin to — maybe this is all after your book was published — when they begin to actually understand the neurological effects of it. I want to follow the strand of the family a little bit. And I have to say I’m amazed that you gained their trust and that they allowed you such access. You were allowed to read letters, you attended a family reunion, which you said is a rather unusual thing in Italy because you need to disperse before you have reunions, and a lot of Italian families don’t disperse. [D.T. Max laughs] But the family was understandably very skeptical of allowing outsiders in.
D.T. Max: I think it depended on who. The hero of the story on the Italian side was a doctor named Ignazio Reuter, who was married into the family. Ignazio did an enormous amount of after-hours research and, in fact, he’s named on the original paper with the neurologist, the original fatal familial insomnia paper that’s published in The New England Journal of Medicine.
I’ve met with lots of families with genetic diseases, and I’ve come to recognize things that I think are at least typical. And I think one thing that’s true is that families go through periods of openness and then closure. The Italian family was in a period where they were very open and very, very hopeful. Those always, I think, go hand in hand. They also were big admirers of the United States, and our ability to solve problems. In fact, that was true of everybody in the story, including an Italian neurologist named Pierluigi Gambetti, who’s very prominent in the story and who’s now the head of the US National Prion [Disease Pathology] Surveillance Center at Case Western. Pierluigi was really the most important, or one of the two or three most important scientists in figuring out that they had a prion disease and what it was. Anyway, Pierluigi told me a story about when he was a little boy in Italy and the American GIs came through throwing candy from their tanks. And I think candy from the tanks was something on some level that all of the Italians remembered. It was part of the very positive image that the United States had and, to some extent, still has in Italy.
Corey: And you approached them through a contact, or was it a cold call you did?
D.T. Max: I went through a neurologist. Maybe the fellow in Chicago actually may have been the first one who contacted the Italians. The world of people interested in fatal familial insomnia is pretty small. And I speak Italian: an uncle of mine was a screenwriter, lived in Italy and was a screenwriter in Cinecittà, and so I spoke Italian. Obviously that made a huge difference.
But I really think that what mattered most was just that they were just in this moment where they were hopeful. They had made this discovery about what they had, and so they were interested in letting people know about fatal familial insomnia, I think in the hopes, really, of starting a nonprofit or a fund. This is before GoFundMe, but the idea was something like that would work for them, that getting the disease attention would help them to get the resources, because these diseases are so expensive to investigate. That, I think, was probably the biggest thing of all.
And then I think, last, it’s also true that Ignazio Reuter, who was the doctor who’s married into the Italian family, is a true humanist. I always thought of him as being like the Erasmus of the Veneto. He liked information and he liked stories, and he was supportive of it and I think he, in a lot of ways, brought the family along. It certainly wasn’t my technical knowledge, god knows. I learned from him. He really taught me about prion diseases.
Corey: I was actually surprised when I heard that the family had given you old letters written from —
D.T. Max: Wonderful letters, yeah. There’s a moment in The Family That Couldn’t Sleep when the Americans are bombing the docks near Venice, and I’m quoting from these letters that the generation I was working with had [from] their grandfather who’d been the fascist mayor of a little town, and he was taken. He had the bad luck to have a roaring prion disease at the same time the Americans were bombing the hospital area. And it’s an extraordinary letter. All of the letters back and forth among the family members… This is nearer when phone calls were unreliable, but even during the breakdown of everything else in Italy, the mail appears to be — I don’t know if the mail ever works in Italy, but it was working as well then as ever. And the family kept the letters, and they gave them to me and allowed me to quote from them.
Corey: Are you still in touch with any members of the family?
D.T. Max: I haven’t been for some years. As I say, families go through periods. This didn’t have anything to do with me, but at some point the family, I think, began to feel like the public exposure was not getting them what they had hoped for and was causing them a fair amount of emotional upset. And they, I think reasonably enough, decided to give it a rest. I think they decided they weren’t going to carry the banner of FFI any longer. I wouldn’t be surprised if a new generation rises up that will do it again. I just think at that moment… The courage it takes comes and goes for a family. You’ll see names of families in different diseases from time to time in the paper, and then you may notice those people aren’t always the same family that marches forward, if you take a disease like Huntington’s disease or whatever.
You also should remember that when I wrote The Family That Couldn’t Sleep, I think there was an enormous amount of hope surrounding prion disease. And I think one thing that’s been shown — and I think The Family That Couldn’t Sleep sort of predicts it and sort of doesn’t — is that research has gone very slowly. This book was published in, is it 2006? Is that right?
D.T. Max: 2006. So I was, for the purposes of our podcast, looking through some of the more recent publications on prion diseases, and I remember that there was at least one researcher who, back when I researched the book, which is around 2004, said, “In a decade we’ll have a cure.” And then the Italians would laugh and they’d be like, “A decade ago they told me that.” But the Italians are cynical and worldly in general — the researchers, not the family. And of course now I looked and I thought, it’s amazing how little has actually gone on, which is another way of saying how hard the research is.
One of the things that The Family That Couldn’t Sleep tries to show you is how funding has changed in the world of medicine. There’s this guy I’d like to talk about more later named Carleton Gajdusek, who discovers kuru. And it’s actually incorrect, although it’s in his obituary everywhere, that he found out what caused kuru, which was mortuary cannibal feast, funereal mortuary feast. He basically was funded by the US government with a check, whereas the later generations of prion researchers had to fund — you know, what’s very familiar to researchers today, I don’t have to tell you science researchers, you have to fund your lab, you’ve got to write your grant proposals. So Carleton Gajdusek wins the Nobel Prize basically for his prion research, but it wasn’t called that, in 1976, and his successor, Stanley Prusiner, also wins the Nobel Prize for his research in prion disease, but he does it partially with funding from private companies, grant proposals… I mean, there should be a Nobel Prize for getting grants. The amount of work his lab puts in to the funding is just extraordinary compared to Carleton Gajdusek. So the book’s also a tale of two science industries, two science cultures in the United States and how they changed in 20 years.
Corey: I think you say that in 2001 Prusiner was the most highly funded researcher through NIH, to the sum of like 50 million dollars.
D.T. Max: Did I say that? He’s certainly gifted and good at it. One other thing he predicted that came true, and it’s in the book, is that, I forget what year, but he’s talking about a meeting of prion researchers — and it’s at that height of the mad cow concern, because mad cow is a prion disease — and he says something like, “This is the largest number of prion researchers who will ever be gathered in a room.” And everyone looks around wondering, what does that mean? Will we all be in different rooms? No, what he means is funding has crested, because they wanted a test for cows most of all. I don’t know how many of your listeners will remember what mad cow disease is or was, but this is a disease in the cattle in Europe, principally in England, in the ’80s, which causes cows to have aggressive symptoms, like charging their handlers, staggering as they go around, symptoms that a careful researcher could see have something in common with fatal familial insomnia and other prion diseases. After some very, very, very good veterinary research, it’s traced to a decision that the British made a few years before to put rendered cow protein into the feed given to cows. And prion diseases are hard to transmit. It helps if the receiving protein is similar to the infecting protein, so there’s nothing more similar than a cannibalistic meal. Now when humans start eating cows infected with BSE, which is the bovine form of mad cow disease, 170 or so get sick and die with appalling symptoms. Again, your capable listeners will correct us both, but to me that 170 still remains on some level the largest clearly delineated, single-innovation food-disease error committed in our lifetimes. I’m sure more people die from salmonella poisoning every year, but a specific change in something that results in such a catastrophic result is very unusual. It’s an unusually clear scientific outcome.
But what’s also interesting is that it was only 170 or so people. In getting ready for our discussion, I looked up how many people had died since the book came out, and I don’t remember exactly, but if there’s 177 known human deaths from eating infected cattle, that’s only about 10 or 15 more, I believe, maybe even less, than when I wrote the book. So essentially the number of deaths from mad cow stopped in humans, even though the infection is widespread. Recent studies have shown that there are hundreds of thousands of people infected by prions. When people have their appendices out or appendixes out in England, they often do tests to see the presence of malformed prion proteins. And they continue to do them and continue to find an astonishingly high rate of infection. But people aren’t dying from the disease.
Corey: You have a figure in the book where I think you say that in the late ’80s and ’90s, the British ate about 640 billion doses of BSE, and it’s really an astonishing public health mistake. They were nationalistic about it: they didn’t want Americans involved, the bureaucracies involved, and they didn’t want to harm the British agricultural industry, so they didn’t want the information to get out. I think you’re suggesting that the only thing that stopped this from being a massive catastrophe is this stuff is pretty hard to transmit, and even if it gets in your body —
D.T. Max: Exactly. I was going to ask you guys, what’s the most efficient foodborne illness? Anyone have any idea? I don’t.
Steve: E. coli?
D.T. Max: Does E. coli always get you sick if you consume it?
Steve: I don’t know, I’m just guessing, but —
Corey: There are different strains of it also. This may be an example of the least, you’re trying to say, right?
D.T. Max: I’m just saying it’s extraordinary. It also suggests, and The Family That Couldn’t Sleep tries to bring this out, that there’s a lot of things we don’t know. For instance, a recent study of infectivity in British people who had consumed doses of BSE completely reversed a previous one and showed that there are people who have polymorphisms of the relevant prion gene, and [while] it was [previously] shown that those who had veiling at some spot on the gene were far more likely to contract BSE than those who didn’t, the new study showed just the opposite. So now they’re supposing that maybe it’s possible that non-lethal presence of prion proteins in the appendix is a sign of one form of homozygosity. Anyway, the point being we really don’t know a lot today. I looked up some of the names who I’d been very familiar with in that time, whether they’re still in the field, and most of them still are and most of them are still publishing. But I think it’s just evidence that progress is slow in this area. And that’s obviously very bad news for the Italian family, and also bad news for the other families who have the condition. I forget, the book, what does it say, about 20 families worldwide who have the inherited form of FFI, is that too low?
Corey: That’s a little low, but—
D.T. Max: It’s quite rare.
Steve: To the extent that one does actually understand the genetics and the specific mutation that causes this, it seems like you could eradicate it all at once by just screening the babies or embryos of these families to make sure that it’s just not passed on.
D.T. Max: Yeah, well that brings us to the subject of an American couple, who I wrote about in The New Yorker, some years after this book was published. The book was published, and a few years later I received an email from a guy named Eric Minikel, who just told me the story that his wife’s mother had died of FFI, and that she had tested positive. I was looking for different ways people respond to disease, because that’s in a way what the book is really about. And I was intrigued by the fact that both Eric and his wife, Sonia, quit their day jobs — she was a lawyer, he was an urban planner — and began getting their PhDs in protein research. An extraordinary gesture. And they’ve got their PhDs now actually.
But the reason I bring it up now is that they had a child, and they did embryo selection. I don’t think in 2006, I don’t believe that was quite the option it is today.
Steve: Yeah, that’s very likely.
D.T. Max: I don’t think that all the same, Steve, I don’t think you’re going to see the eradication of the gene any time soon, partially because I think the level of education necessary to do embryo selection, the cultural specifics for people willing to do embryo selection, are quite narrow. A number of the Italian families did not have children at all, a form of embryo selection rather than… But nobody would have accepted embryo selection among the more Catholic members of the family. Some were, some weren’t.
Corey: You talk in the book about how members of this family had a difficult time often getting married because word had gotten out that there’s some disease. And this is pretty common in a lot of traditional societies. Families basically do due diligence on potential mates. It’s less common here.
D.T. Max: They didn’t even have to. Even in Italy today, people — this is something that’s so different between the US and Europe — people don’t go that far from their homes. Even if they work in Paris, they go back to their villages where their families are for the summer. And that’s also true in Italy. You wouldn’t have to do due diligence, everyone would have known the family and everyone would have known who are the cousins and who are the second cousins. It’s a very small-town environment even today. And even though many of the more educated go into the cities, they still retain their relationship with their hometowns in a way that I don’t think has any counterpart in the US. People really move in the US. I’m not saying they don’t have relatives they leave behind, but they move.
Corey: We haven’t added that this disease is autosomal dominant. If someone’s a carrier, then their child has a 50% chance of inheriting the disease.
D.T. Max: Correct, that’s correct.
Steve: Sorry, geeky question, what happens if you get two copies? Is it really bad? Do you die early? Is that known?
D.T. Max: That’s a great question. I remember asking that question and I don’t remember the answer. There is a slow and a fast form of the disease, but that has to do with, again, the hetero/homozygosity question at the gene. I don’t know, but if you think about it, what are the chances of getting two copies?
Steve: Yeah, very rare, but maybe sometimes people marry their cousins in Italy. I don’t know.
D.T. Max: I think they probably do, but… I don’t know, I think that it’s sufficient that one of the unhappy parts of the disease is that essentially 100% of those who carry the gene develop the disease. That’s the other bad news.
Steve: On a slight tangent, I was once involved in a study of a rare mutation which causes face blindness, so the inability to differentiate other people’s faces. It runs in this family, and they said when they had family reunions they would all wear name tags because they couldn’t actually tell each other apart.
D.T. Max: [laughs] There’s a lot of people in my world who claim they have face blindness, but I just think that they don’t really care who you are.
Steve: It’s a continuous trait. Some people are better than others. Some are super recognizers. There are apparently people who can meet somebody, and then ten years later they pass them on the street and they can remember the face.
D.T. Max: Yeah, at The New Yorker we did an article about how the British have this squad of people called super recognizers who can match video images with drawings. I don’t remember the exact details, but like those dolphins in The Day of the Dolphin who are able to intelligently communicate and are then turned to weaponized uses.
Steve: See, now we don’t need those people, because AIs are so good at face recognition.
D.T. Max: That’s right. They’re on their way out, like cashiers.
Corey: There was a professor at my undergraduate college, Stanley Rabinowitz, who would every year memorize the 400 people in the student face book. I was in my hometown about ten years ago, and I’m walking down the street and I see this guy, and he just starts staring at me, and he smiled at me. It was Rabinowitz. I hadn’t been there in 25 years.
Steve: Yeah, so he’s an outlier of super ability. A kid that I knew since childhood, since I was probably five or six years old, didn’t tell us until well after high school that he actually is face blind and didn’t know who we were, except he had to memorize, for example, the kinds of coats that we wore. So he was kind of an oddball guy in high school, but now we understood why.
D.T. Max: It seems a complicated talent to recognize a face. It’s interesting that AI is now good enough at it, because it just seems like one of those things that’s much harder than it would at first appear. Anyway, that’s not this particular family’s problem, they were quite good at recognizing each other.
Corey: So let’s look at the science in the book. As you say, you’re pretty character driven, and your two central characters, Carleton Gajdusek and Stanley Prusiner, are complicated to say the least. I think at some point in time you quote a colleague who says that Gajdusek is an egoist, and Prusiner is an egotist. But more deeply, Gajdusek’s a pedophile, and Prusiner seems like a jerk. And so you’re writing this book and you’re faced with the fact that these two people who could presumably, at least under other circumstances, might be heroes of your book, just aren’t really suited to it.
D.T. Max: I think that’s a very good observation, because I think in a funny way The Family That Couldn’t Sleep is a book that’s searching for a hero who hasn’t yet appeared. Maybe to some extent Eric and Sonia, who I referred to before, who are the American couple who quit their jobs and become prion researchers, are the heroes that The Family That Couldn’t Sleep doesn’t have. So the hero of The Family That Couldn’t Sleep, I would say, is simply the human instinct to survive, but that’s not quite the same thing as having somebody named Eric and somebody named Sonia.
There were a number of difficulties in having two such unsympathetic star researchers. But for me, to be honest, they were preferable to having more perfect people in that role. Carleton Gajdusek, who died a few years ago, just to give a little background on him, I think he’s the only Nobel laureate, at least in medicine, who later wound up in a county lockup. I could be wrong, people can fact check these kinds of things now very easily on their laptops. But it’s at least true that he wound up in jail, and he wound up in jail with one charge — I don’t know the technical charge, but some charge of basically molesting a minor.
He was a guy who grew up in Yonkers, always wanted to be a great scientist, stenciled the names of great scientists on the staircase up to his attic. When he saw that there was a group of Papua New Guineans who were dying mysteriously of something called the laughing disease, he saw an opportunity not to be missed, and he immediately convinced his minders at the NIH to fund, basically with an open check, all the research he wanted to do in Papua New Guinea on this tribe that was dying out.
These trips to the South Pacific coincided very nicely with his interest in underage boys, and he came to believe, I don’t know with how much basis in fact, that there were man-boy sexual relation traditions on some of these islands. He comes back to the United States — I’ll get to his science in a minute — and he sets up in suburban Maryland, right near the NIH offices, a huge house full of young men. And it was a question even then in a more naïve time, what did Carleton Gajdusek want with all these young men from the South Seas, from Micronesia and so on. He educated them, he sent them to western schools, and they would often go home to their countries that they came from and be major participants in the life of those cultures, because they came with these blue-chip American academic degrees which were very, very valuable, and a cultural knowledge that many other people in their islands didn’t have.
He molested some of them, there’s absolutely no question about that. And he was caught in the case of one, mostly because he kept these endless diaries. I don’t believe the diaries are explicit, but I was not able to read all the diaries. I read many of them. They’re somewhere, you can read them, I can’t remember if it’s the Library of Congress or something like that.
Anyway, Carleton went to jail, and then after he went to jail he went to Europe. I caught up with him in the Netherlands, where he was living in a kind of student hotel, a residence, and enjoying life as a highly esteemed Nobel laureate. So although his reputation had been destroyed in the United States, I think in Europe there were still people who saw his talent and were interested. He was a big ideas guy. I would sit with him — I spent a lot of time with Carleton — and he would sputter on about how protein conformation was a model for life. He was a big picture guy. The idea that he could actually be funded today seems almost laughable. It would be so far from what you would fund.
So the one thing people know about Carleton Gajdusek today, if anything, is that he discovered that kuru, among this Papua New Guinean tribe called the Fore, was caused by mortuary cannibalistic feast — in other words, they would celebrate the deceased by eating choice parts of the deceased, and there was a protocol in terms of who got what: so you know, the uncle got the arm… And that’s wrong. As the book points out — and it’s interesting how hard it is actually to change what people think is true, almost impossible — the book points out very clearly, and I’m not the first one to point it out, that Shirley Lindenbaum, who was an anthropologist hired by the Australians, is actually the one who figured it out.
So Carleton goes, and he was a maniac for bleeding and he took blood samples from thousands of Fore, and he was looking for potential contaminants in the wood smoke at their fires, in the water, he did all the standard epidemiological things. He knew that they practiced these mortuary feasts, but he never associated the one with the other. He may have thought the feasts were too infrequent. But a really good researcher who was a little less full of himself would have noticed that the people who were getting sick were the women and the children. Protein was prestigious in the Fore world, Papua New Guinea, so the men got the best protein and the women and children got the mortuary… Even then, I don’t think eating your uncle was considered a really proud source of protein. So the reason women and children were dying predominantly of this disease was precisely because the vector was the funerals.
And Shirley Lindenbaum, who was an anthropologist, knew how to ask questions. Carleton never really was a good questioner. I would sit with Carleton and I would feel like I wasn’t even in the room. Again and again he’d go on about things that were abstract and interesting, but they were theory stuff, really graduate-student bull sessions. So that’s Carleton.
So Carleton wins the Nobel Prize in 1976, and basically he’s right. He basically says there are kinds of viruses, he calls them slow viruses, that can exist in the system for 20 years before causing damage, which would fit the case study for kuru. But he didn’t actually recognize where the infection came from. That was this other woman who did not get the Nobel Prize. And if you look at the obituaries of Carleton, they still say that he figured out that cannibalism caused kuru, when in fact he just spent a lot of American taxpayer dollars not figuring that out.
So fast forward 10, 15 years and Stanley Prusiner comes on the scene. When I say Carleton egoist, I mean Carleton is a narcissist or was a narcissist. He really saw nobody but himself. And that’s a very powerful theoretical scientific mind, but it’s not a very good epidemiological scientific mind for obvious reasons.
Stanley was the reverse, in that Stanley was proud the way people are proud who have achieved a lot and know it, and know they’re smart and have always been rewarded for it, and are good at playing the game. He was just an egotist. You referred to him as a jerk I think. He was good at getting credit, he was good at doing the work, he was good at getting talented postdocs to work for him, he was good at publicizing prions. And he named them. Prions before that were called slow viruses, and he renamed them for proteinaceous infectious molecule — I forget what prion stands for exactly, that doesn’t seem to quite work, but something very similar to that — and he knew he had a great term. Imagine the nerve, to name a disease principle before you even know what it is. What if researchers were always naming things different things just because they thought they were different? So-
Corey: You suggest actually, Daniel, that he named it prion because it sounded a little bit like Prusiner.
D.T. Max: I thought it was because it sounded space age. No?
Corey: It’s in the book about —
D.T. Max: Yeah, I remember that, but in retrospect I think it sounded sexy because prion… Look, in a lot of ways biology wants to be physics, especially [because] the discoveries in physics are sexy compared to the discoveries in biology. So by making it sound like it was some sort of quark-type discovery, I think he woke up a lot of journalists who would have otherwise yawned at the idea that there was… If I tell you that there’s a protein that causes infections like a virus does, that may excite YouTube or maybe not, but it’s one step shy of what excites the front page of The New York Times. He was good at that. I’m not criticizing him. I think that’s a useful talent. In a way, the book means to be a parable about what scientific talent was useful right after the war, and what scientific talent is useful now.
But going back to your question about narrative, they couldn’t really be the narrators for that reason. They couldn’t be the protagonists of the story. The prion really had to be the protagonist of the story.
Corey: Did you talk to Prusiner?
D.T. Max: I talked to Prusiner briefly and obliquely and through cutouts, and there were some emails we exchanged. I never got his wholehearted love. He was just brutalized in a piece that had come out a couple years before by the science writer Gary Taubes in Discover Magazine, and he’d never recovered, I think, from the idea that journalism can go both ways for you. I don’t think he expected it. I wasn’t going to brutalize him, but I don’t think he ever quite felt comfortable again with the odds being even.
Corey: So you traveled to Italy, you talked to Gajdusek, did you ever go to Papua New Guinea? Because a lot of the book takes place there.
D.T. Max: I didn’t. I would have liked to have gone there, but the substitution was I like to deal with written records a lot. There’s no one left in Papua New Guinea. What would you be looking at exactly, except getting a sense of the landscape? And the world there is so different. But the original reports from the case officers under the Australian government are also lodged at San Diego — one of those odd things, somebody copied the entire case report from the colonial officers — and so I was able to get access to those reports and I used them. I also interviewed the few people who were still alive from these kind of crazy… It would make a terrific Werner Herzog movie to have Carleton Gajdusek going through the forest, the jungles of Papua New Guinea with a needle, plugging everybody he could find and taking their blood before they could give consent. Whereas Stanley Prusiner as a movie is very difficult to imagine and requires a much higher level actor, I think, and a much higher level director. Can you guys think of a single movie that makes lab work sexy in the modern era?
Steve: The movie about Elizabeth Holmes and Theranos. [laughter] Just kidding. For all the wrong reasons.
D.T. Max: Right. But you know what I’m saying? As a narrator, you know that narrations of great importance are going on in labs. It’s obvious. That’s where the knowledge is being constructed, much more so than out in the field. Carleton Gajdusek would be completely obsolete today. He was a guy who loved to be out in the field.
But it’s very, very difficult to make any of that stuff… It’s getting harder too all the time, I think. Look at the reporting on CRISPR, that’s an interesting example, and where that reporting is good and where that reporting is not so good, because actually CRISPR comes up, I noticed, in connection with prion disease and the possibility that you could edit the prion gene. I noticed this also, we still don’t really know what the prion gene does, which is interesting. We don’t know what the prion protein does in the body.
Corey: I think you say at some point that they deleted it in the mouse and didn’t see any phenotype. I don’t know if that’s still true, but it was true —
D.T. Max: No, that is still true.
Corey: What’s happened since is that I think this idea of infections in prion has actually spread quite a lot more, because there’s some discussion as to whether Alzheimer’s disease might have an infectious component.
D.T. Max: There always was. There’s a chapter in the book about why you should care, since you’re very unlikely to have a prion disease and you’re lucky enough not to have eaten beef in England during a brief window in the ’80s. So why do you have to care? And you’re right, I think a certain amount of research has gone on that shows that there are other proteins, as they suspected, that behave rather like prions, which is to say that at least the bare-boned science of it would be that if one malformed protein touches an adjacent, properly formed protein, it causes it to change form into the pernicious or disease-causing form of the protein. There’s no doubt that amyloid plaques are an embodiment of that principle. The question is, are they the disease? Can you really refer to amyloid plaque as an infectious disease? It doesn’t seem to quite work in that way.
I did meet one person, a veterinary researcher. One thing I loved about this book was it was just so weird. I can’t emphasize to you how much I like going down strange dead ends. And so a lot of the book, a portion of the book, is about how they might have diagnosed fatal familial insomnia with the instruments and the tools and the understanding they had when the earliest patient that the family identified, who was this doctor in Padua, may have had the disease. It’s not a bad guess. But I spend a little bit of time also, for instance, on the British curates, the priests of their towns, who would notice that something weird was going on with the sheep, because scrapie is a prion disease in sheep.
Similarly, I met a veterinarian, not a super prominent veterinarian, somebody who was in the literature for prion disease — but as you guys know, for every one researcher the world knows, there are hundreds of researchers doing even equally important work who just aren’t in the paper, and the people in their field know who they are — so this woman played a role when primates were a focus of prion research, and she claimed that she had successfully transmitted Alzheimer’s from one monkey to another. Now of course they can’t use monkeys anymore in any of these experiments, god knows.
Corey: What year was this?
D.T. Max: Her name is Rosalind Ridley, I believe. I’d have to look up her name. It’s Rosalind something. It’s in the book, this is mentioned. But it’s never been replicated, if anyone tried to replicate it. Probably nobody’s tried to, it just flies in the face… And I think probably it’s discardable, but I found it interesting. And I found it interesting to meet a person who knew this, and felt that she wasn’t getting a hearing on it.
Corey: One of the people we just interviewed is Stuart Firestein. He’s a professor at Columbia in neuroscience, and Stuart’s theory is that the way you should teach science is through narrative, rather than simply giving classic experiments and results. You want to present the mystery, what something looked like at the time, and why people believed a particular theory. I think actually the case of prion disease and your narrative is a classic example of that, because at each stage you can really see why people might hold on to this particular wrong conception, and how it took a lot of effort to show that DNA or RNA were not involved. You could always find some way of suggesting that still it was involved and undetected.
D.T. Max: Huge amount of money, too. Huge amount of money to come as close to proving that — and I think we’re all pretty satisfied with the proof at this point — but a huge amount of money just to check off that box. And some of the old timers still don’t believe it.
Corey: There was a recent study where I think they just used genetically modified E. coli, and they basically put in the malformed gene and it pumped out the prion protein, and then they injected that. That’s about as close as you can get to finding something that clearly has no RNA or DNA.
D.T. Max: Is that the Ohio State thing?
Corey: It’s possible, yeah.
D.T. Max: But just back to the byways, I enjoy the failures too. In a way Prusiner, bless his soul, was the least interesting character to me, because he’s a guy who never quite missed but also in a way never quite… His joy was very, not muted, but very modern in the way that modern things are so hard to narrate. Whereas Carleton Gajdusek was really like a 19th century figure. He was a madman, just an absolutely insane guy. You could say that any successful scientist is the sum, much like an artist, of his or her unsublimated urges. So we can argue that Stanley Prusiner — and I’m making this up — wanted to be famous in grade school because whatever, and so he was driven by this desire to have glamorous women and a huge grant budget. This is probably true. But Carleton Gajdusek, a man who is so clearly unable to sort out fact from fiction, he used to tell me with great pleasure that he should be dead and I should be dead, because we had passed the age of child rearing or I’d had already had children, and so we had served our genetic function. He used to tell other researching friends that for his health he had to have one orgasm a day — not always one, but anyway… By the time I had met him I believe he had had prostate cancer, and I think that was no longer on the agenda. But anyway, my point is that he was eminently narrable. I would love to see somebody narrate Prusiner but he won’t let you, that’s the other thing. And if he won’t let you, it makes it even harder.
I actually think one of the great distortions in science and in the reporting on science, not that you asked, is that personality plays such a big role. The ability to tell your science is what gets my attention, but that’s not the same thing as getting good results or important results. I remember when I was looking for researchers to help me with the book, I would go into some labs and talk to people whose English wasn’t really very strong, and I couldn’t figure out what they were saying. So I would naturally gravitate to the one or two native speakers in the room — a science lab today is so polyglot — and I would take their versions of events, so obviously I was in the process of discarding other ideas, other theories, other focuses. And I was aware of it at the time, but I just couldn’t understand from some people what it was they were doing — literally, but also not just on the level of the words, but also on the level of the kinds of questions that a narrator asks.
I think that what was wonderful about Carleton… I’m not sure I didn’t… I think Carleton was in some ways really an awful man. I think that that’s important, and I’m not remotely trying to say he wasn’t. I think he damaged a lot of young boys’ lives. But that wasn’t really exactly what I was looking at, nor [were] the other writers writing on prion diseases. He energized the field really. He carried it through this period when it really was nothing. It was just a scientific curiosity, it was just a few people, it was a tribe. It was just a curiosity, and his work was important for people like Prusiner. The insane guy was Gajdusek, but there would have been no Prusiner without him for sure.
Corey: It’s funny, you have one quote I really like about Prusiner. You say, describing his papers, “Those papers don’t make exciting reading. They’re like postcards from a traveler who writes to you from every local train stop, extending little by little what he can say with confidence about the view out of the window.” It sounds like it’s quite different from Gajdusek’s approach, which—
D.T. Max: Again, not to be a Marxist about it, but a lot of it does come down to the funding. Carleton only had to excite one guy really, which was this guy, [Clarence Joseph] Gibbs, who funded him. Whereas I imagine that a lot of those papers from Stanley Prusiner are papers where he’s showing the appropriateness of the grant he was given to do the research. He’s got results. He says, “We will use this money to try and purify PRP and prove that there’s no X attached,” and then he’s like, “We have purified it and proven that there is no X attached.” Then he’s like, “Now I need the next funding.”
Corey: That’s something that Stuart Firestein actually emphasized a lot. I think something that’s part of our new more modern understanding of science is just how driven it is by really mundane things, like needing to get the next grant.
D.T. Max: I can’t do science pieces anymore without that. I did a recent piece on a guy named Jim Simons and the institute for computational research, the Flatiron Institute, that he’s opened in New York. Jim is maybe the 26th richest person on the planet, and he just funded it. He just funded the thing. And you can see how relieved the researchers are that they don’t have to apply for grant money. I don’t know that there’s that clear of an advantage to being there as opposed to, say, Princeton, except that you don’t have to spend your time applying for grants.
I’ll say one other thing. Even for that institute, the price of electronic subscriptions to journals gives them pause. The 26th richest man on the planet and he’s like, “You’re paying what? And you’re consulting it how many times?” [laughs]
Corey: Do you know this guy, Steve?
Steve: Yes. Jim Simons was a famous mathematician who did some work in low-dimensional… Anyway, he’s famous for something called the Chern–Simons term, that’s actually used in physics. But what’s interesting about him is, he was also a code breaker at the NSA, and then he actually started one of the early hedge funds which was very quant focused. It’s the single most successful hedge fund ever, it’s called Renaissance, and it’s out on Long Island. So when he gave up running that fund, he basically got into science philanthropy, and hence the Flatiron Institute.
Corey: So grant funding has been an issue we’ve talked about. It’s part of the granularity of science, it’s an essential part of how science is done, and I think people are now beginning to discuss it in a way that philosophers of science hadn’t done because they weren’t scientists and didn’t have to get their grants funded for the past hundred years.
One last topic, Dan. I’d like to find out what’s been your experience since the book’s gone away. Have you remained interested in this topic? Do you just let a topic like this go? Do you check the news periodically?
D.T. Max: It’s an interesting question, because I think what’s weird about hard science like this is it’s slow, and it goes up against the human life span. I’m still in touch especially with Eric and Sonia, the Americans — she’s the one who has the gene and they’ve become prion researchers, so I keep up with them. I keep up with some of the researchers. I keep up with the food illness issue. The book was very widely translated and it was translated in Asia, I don’t think because there was any great interest in fatal familial insomnia, but actually because of the chapters on mad cow disease. And so I remain very interested in the question of foodborne illness, and what happened to BSE. I just looked it up, as it happens, and there were no cows found in the United States with mad cow disease for ten years, and then there was one three years ago and one last year. So when I wrote this book, all the institutions that are now under consistent attack were pretty much above scrutiny, and it was the extremists of the food purity movement who, no matter what, wouldn’t believe that the USDA was testing enough cows. The USDA has this weird double mission, that it both is in charge of the business of agriculture and the safety of agriculture.
Corey: The same is true actually of the British organization, the institute, as you point out, that has this huge conflict of interest.
D.T. Max: Yeah. So now we’re in an era where, think about how much less credulous people will be at any government finding. And I do wonder quite a lot if there were another mad cow outbreak, how people would respond if the government assured them their food was safe to eat, both in England and here. So that’s the kind of thing that still really interests me about the subject. Because The Family That Couldn’t Sleep is really about, in a certain way, an unsolved question. The biggest unsolved question is why do we get sick? But there are a lot of smaller unsolved questions, like why BSE isn’t more infectious. Why didn’t it kill all of Britain? There’s this extraordinary prion disease, and yet it really doesn’t matter to anybody in a practical… I often talk about the book to various doctors, and they go, “Oh yeah, we spent a day on prion disease in med school, we spent an afternoon on prion diseases in med school.” And some of them have seen somebody who had Creutzfeldt-Jakob disease, which is another form of prion disease. So I’m always interested in the extent to which this thing has penetrated the culture.
And then of course, as you say, another way to look at the book is that it’s a book about insomnia, not prion disease at all. And in that sense I find sleep research extremely interesting, also very unsatisfying, one of the really great mysteries of life. There’s this wonderful quote from, I think, it’s Allan Rechtschaffen, if I have his name pronounced right, who says, “If sleep doesn’t fulfill any absolutely indispensable biological purpose, it’s the greatest mistake that nature ever made.” And I think about that all the time. We don’t really have that yet. Every so often National Geographic does a cover like, “The Science of Sleep Finally Explained,” and I read it and I’m like, “No, the science of sleep has really not been explained yet.” If I had to see which was explained first, sleep or prion disease, I’d say the more useful thing to learn about would be sleep. But, again, basic research in sleep is very, very hard to fund.
Corey: I think we are getting pretty close to a result that sleep seems to clean out these kinds of complex proteins, tau and beta-amyloid, in interstitial spaces. That’s pretty important, because as those build up you begin to get symptoms of Alzheimer’s, which is why we all feel like our IQ goes down as we’re sleep deprived. And if that’s right, that’s fairly essential over time.
D.T. Max: But is that worth eight hours of lying inert in an open savanna?
Corey: Yeah, because it becomes toxic if you allow it to go over a couple of days. As someone who’s experienced pretty serious insomnia, there’s nothing worse than not being able to sleep for a couple days. You have to actually begin to downgrade the things you do. I can’t write very well, or I can write at a kind of overview level, but I can’t actually begin to compose sentences that I’m really happy with. So I’ll begin to do light editing and so forth. It’s a problem. You just—
D.T. Max: No, I agree with you. I think insomnia is the great undiagnosed disability. My wife doesn’t sleep well. And there’s no question that it affects every aspect of one’s quality of life. You’re looking a little tired there, Corey. [laughter] I think I should go soon, just because I see my children are returning to the hut. Do we have more?
Corey: No, I think we have covered what we need to cover today. Thanks a lot, Dan. This was a pleasure.
D.T. Max: Thank you guys for having me. I enjoyed talking about it.
Steve: Thanks Dan.
Corey: I look forward to it again.
D.T. Max: All right, thanks guys.