Daniel Max on Writing a Literary non-Fiction Classic and Prion Diseases Then and Now  - #15

Daniel Max on Writing a Literary non-Fiction Classic and Prion Diseases Then and Now – #15

so Cory our guest today is an old friend of yours DT or Dan Max and 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 periodic disease that makes them insomniacs and eventually kills them now I would 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 so I I think the book is really stellar for a couple different reasons it's a kind of mix of genres I'm a fan of certain kinds of literary fiction and I think unlike a lot of it's called popular science this book is actually 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 detect a story knowing also the detective fiction often isn't extremely high quality but Dan's a wonderful writer and just I back to pay for my time two sentences and how he paces the book and that combination of writing a really good science story in a narrative form is probably it's great strength so has it been compared to in cold blood by Truman Capote which probably you probably are familiar with that right or it hasn't I think when I think about this book I think what's sort of hard is it's in a genre or people often don't think much about really good writing people just think of a pop science book is sort of taking an idea and making it you know accessible to the public and so there's really very little effort of trying to make a book kind of 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 these disease prions showing they look like they're actual infectious agents but there's a whole other half of the story which which we're still in the midst of which is we duck so you don't know how to stop these diseases and this book was written about 20 years after mad cow disease first hit Britain it wasn't actually really notice for use later but we're now about thirteen years after the book and not much as axes happened so I kind of want to check in with Dan to find out about what he's seen in the research in also checking with the people he spoke to as part of this discussion it's a mean that's that's kind of once I think that's why I think I like the book as a whole I think it's also really fascinating just is a is how he executed it right because he's got some very difficult matters to tread here right he's got a family that has a disease and that suffered a lot through the Z's but also suffered from the publicity that's happened you know they were basic arrests in their own community and he's got to scientific characters aren't terribly sympathetic one of whom is a pedophile and how do you write about that when someone so central to your character has such is so morally problematic I really expend a lot time reading the book tried it for me how is he gonna deal with this guy so i I've 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 so prions a really they understand recognizing their existence is really a fundamental paradigm shift in our understanding of infectious agents you know since Pasteur we assume that things in fact 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 and it took a very long time for people to realize that prions weren't like this there's a whole different category of infectious agents so it's in that way it's kind of a classic scientific story of a real fundamental change in paradigm and how hard it is reasonably right to get people to accept that because you don't want people dropping paradigms and it took painstaking work so if I recall properly the proteins are produced from RNA and there long and linear when they first are produced but then they have to fold up and the confirmation yes and and one of the mysteries is exactly how nature comes up with proteins that unerringly fold into the right functional shape here we have Matt miss folded or malformed proteins and somehow they're able to cause other proteins to then assume that wrong shape and width eventually downstream health consequences yeah that's it so it's a remarkable kind of phenomenon right 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 it is as much as that they were not you know DNA RNA base but yeah of course was how it was transmitted actually yeah I think that there's there's 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 is then underscores in our talk we haven't made a whole lot of progress on 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 you know I I remember being offered brain about 30 years ago in uh in Managua and I think I turned it down then and I think I'd probably turn it down now okay very good well on to DT max I'm Cory Washington and I'm Steve Schuh and our guest today is Daniel DT max staff writer – 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 of David Foster Wallace and also of numerous articles in New Yorker that I read whenever they come out welcome to manifold n thank you very much it's a every love story the ghost story oh did I screw that up I think you had it backwards or maybe I mean it's reversible so it's true but let's get a little background how long have you been working at The New Yorker and you can just can just grab the tips of pieces you're right there yeah I mean I've been a New Yorker writer I've been a staff writer probably for about 10 years now and before that I did a very similar job at the New York Times Magazine I mean 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 corners and so I've written about artists that I've written about scientists I've been about science I've written about PO cartoonist recently before that I wrote piece by 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 sort of the International this is gonna become more boring than it was but the International Economics of the Chinese garment industry in Prato Italy I don't know what you call that piece it's kind of a laborer piece in a way was it about fast fashion it was actually it was about it was a fast fashion and about ways in which sort of the Chinese had really pronto it on essentially become kind of a a Chinese city both culture and economics of that and went along on a couple of police raids where they chase the Chinese workers who don't have proper papers and that kind of thing so I do different different stuff and in fact you look at the two books so the family they couldn't sleep it's a science book what in England is called I think without meaning to be dismissive popular science here that has like a little bit of a undertone but there that's just it's just they call it a pom prescience book and then every love story the ghost story is a biography of the writer David Foster Wallace so you can see him there there's no branding you know when I described 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 and pop science but your writing has a deep literary quality and the family couldn't sleep reads a lot like a novel yeah I mean I think that it's true and in fact in the academic world now literary nonfiction is almost kind of an accepted category for instruction but of course it's very hard to say what exactly is literary nonfiction and back to the 70s nonfiction writers employed the techniques of novel writers to kind of invigorate field and so-called new journalism but the journalism tended not to be that accurate I mean they really they they they went in much further than I would ever go in terms of using novelistic techniques but the things that novels are interesting which is character change over time the telling detail those are the things that have always been most most exciting to me and in the case of the family couldn't sleep so this is the book write about something called a prion disease I remember saying to my editor when I first began writing about the subject which I did initially for our New York Times Magazine article I said you know at last I found a disease that has character in other words they never felt that was somebody who's going to be able to write you know there's a book called the genome I got to slide along the history of the history of genes and 23 and a half chapters well you know that book effort isn't in it yeah anyways the point is it's Matt Ridley I could not do that book you know I admired that book but I needed I needed a I needed I needed a disease well that was as weird and interesting as say viruses were seemed to be you know years like I needed something that itself could be given a personality and in fact years ago I wrote a piece about a marvelous chef named Grant Achatz who has tongue cancer was piece about how he this is the New Yorker how he had how he had elected not to have his tongue removed in order to maintain his ability to taste and in that original original draft of that story I made cancer a character almost like a person like searching for his weaknesses and finding occasions to growl and and that kind of thing we found two dead on the end but that's always been a kind of a prerequisite for me in writing a science piece so you kind of answered two questions I was planning to ask you which was after 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 X I don't know if it was short and then that later yeah then that later developed into the book and I was wondering whether there was a similar I don't know if you call the trial balloon for the family that couldn't sleep yeah it does an article in The New York Times Magazine and this is of a sort of early days of the web I think it's 2001 March of 2001 and even then you could sort of see there with the response it's weird before the web it was very very hard to know whether an article you've gotten any response or not so long for the responses to get to the magazines and newspapers but I 2001 you you sort of get a sense that something was interesting people and of course by the time I wrote the article and David Foster Wallace that was much even considerably further along but you know one reason you you would do it this way was really financial which is it cost 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 like you had to send me to Italy for the family couldn't sleep that wasn't really something I would have been people or inclined to do on my own and then you get the professional editing you get people who 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 for the landscapers now how did this story actually begin so begins this way it's wonderfully humdrum which is that the New York Times magazine was doing issue on diseases that crossed borders and I was considering going on the Hajj as the Hajj is apparently a very high infection environment so all those people are creating any environment where people are crammed together a lot of disease and I don't remember why that didn't happen but I typed into the internet the web maybe I use alt or vista for all i know you know world's most terrifying diseases or something now did you really can't do that there's too much on the web you'd have to break you know you have to dig and dig dig dig it doesn't work anymore and and a paper published in the New England Journal of Medicine came out about something called fatal familial insomnia that's actually how I first first heard about it was just you know just by typing into the lab again I I don't think that works anymore I mean I you know longer there's so much on the web now you really have to have you have to have the ability to short material in a way that you didn't have to have 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 or yeah absolutely I mean I don't want to claim more more talent than I possess I looked at it as quarry points out like fundamentally I have to relate things to what is to what's most central to me which is if you want to if you want to condemn me to littering on picture and I accept the sentence but but it's some sort of it's some sort of nonfiction that is more than the sum of its data let's put it that way and so in this case you know the title fatal familial insomnia immediately of course makes you think of things that are very different from disease you know techniques or you know it makes you think about families right I mean right there now that's just a happenstance of how they mean it's one of the people who named it said to me he said we could have called it familial fatal insomnia but it didn't sound quite as good I mean they were even they were aware and then that the naming of the disease would be useful in getting it publicity frankly or attention because it's rare I mean it's it was rare when I wrote about it and it's and it remains rare now it was only later as I began researching that it's connection to mad cow disease became clear and also the fact that you know prion research simply put I mean attracted the most interesting researchers on the world for a while I mean there's nothing like this very potent guy – like who could get to close that later you know I think I'd heard about a prime disease back when I was about eight in a member reading the 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 cannibalism a.m. kuru kuru exactly yeah and that was again one of the yep 74 edition it's back when they were big and fat right yeah yes they're fine they're fantastic I remember man with the world's largest mustache of course of course and being able to go here we go and back then the heaviest guy in the world was Robert Earl Hughes who's one thousand 69 pounds and he's about 700 pounds lighter than the most recent heaviest man in the world and he was buried in a piano case that's right so you were at the time this was your first science piece is that right no I don't think that's quite true I would actually have to think about it it's certainly my first piece on prion disease but I think I had done science pieces and you also need to know that I myself had developed a rather nasty gyro the degenerative disease shortly before and so I was much more interested in disease than I would have been I think otherwise I mean I was I remember how old I was I figured I was 40 and so I was kind of let's 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 you know uniquely afflicted or just sort of part of what we were on the road we were all on I mean the answer is obvious but it's really how you emotionally experience that information and so the last chapter the family couldn't sleep is actually a little bit of biographies it's a little memoir about my getting a diagnosis at Columbia Presbyterian Hospital but I didn't want you know everyone loves ma'am or her but I didn't want to get let people cheat and skip ahead so I I think that that's that chapter is called a note on the author so that you wouldn't expect it would just be you know DT max lives in you know in New Jersey with his wife and two children but it's actually a whole chapter I remember you're hinting at it in various parts in the book and you do it the last chapter focused on it it's funny because you know I experienced this book in slightly different ways you know you have a neurodegenerative disease I'm a chronic insomniac and it's funny because I read some of your descriptions about how sleep naturally takes its course and so you're just eventually tired enough you fall asleep and that's not true for insomniac s– 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 Coria 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 that's right so I'll leave it to Danielle to tell us a little bit about the disease you're punting okay so right so I told you how it started so what this is what the book is after that first half foray into the internet I called a neurologist in Chicago who tended to have interest in kids so I asked him if he'd seen any interest in cases he told me he just had it he just had a patient who had died from insomnia and I told him that you know I thought that was impossible and nobody's ever died from lack of sleep he said well that's what you think there is such a disease I realize this is slightly inconsistent with the previous story but they are both they're both true so man I said I really want to come out to Chicago and meet with you I'm there's nobody as avid as a journalist who sees a good story and he said wait wait wait and my heart was sinking I thought he'd tell me you know I 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 you know death in Chicago death in Venice that sounds like something and went and got my passport so what's the book about well that is the book is about prion disease so prion is a weird infectious protein that behaves kind of like a virus and so you can transmit a prion and cause prion diseases 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 kind of cover is that um from 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 your license and finally the book is really also about the way in which crayons are relevant to all of us because you know the principle of the prion which is a protein that Miss folds and causes disease is relevant in other conditions like Alzheimer's and Parkinson conditions that are vastly more common than prion diseases are so you know it's in England the book is called the family couldn't sleep unraveling a medical mystery an accident sort of a better title because it really is a search book as his search I search with the families they try to figure out what on earth is happening to that and then I take you into the science and what's known about the science that finally they these are things we might eventually learn from Creon 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 yeah genetic mutation genetic mutation it's a very simple error in genetic engineering code and they're not the only family but it's fairly rare I mean extremely rare I think it's one in a million is the genetic so because of the mutation their bodies tend to produce misfolded proteins is that how yeah there's 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 and 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 you're also let's go to curing those proteins your body is less good at clearing people on proteins to the lysosome so that's the probable reason that it's not as if the gene is only activated so you know there couple strands throughout your book one is story this family and then there's the scientific story discovering and understanding the operational primes and then they're kind of come together when you begin to and maybe this is all in the after your book was published when they begin to actually understand the neurological effects of it but I want to follow this strain of the family a little bit and I said I'm kind of amazed that you gain their trust and that they allowed you such access you you're allowed to read letters you attended a family reunion what you said is a rather unusual thing in Italy because you need to disperse before have reunions and a lot of Italian families don't disperse but the but the family was very understandably very skeptical of allowing Outsiders in I think it depended on a huge so that the kind of the hero of the story on the Italian side was a doctor named Ignacio Reuter who was married into the family and Ignazio did an enormous amount of kind of after-hours research and in fact he's named on the original paper with the neurologists the original fatal familial insomnia paper that was published in New England Journal of Medicine the family was in the moment I mean nice and some have met with lots of families with genetic diseases and I've come to recognize things that I think are at least sort of typical and I think one thing that's true is that families go through periods of openness and then closure and and the Italian family was in a period where they were very open and very very hopeful those always I didn't go hand in hand they also were big admirers of the United States and our ability to solve problems and in fact that was true of everybody in the story including there was an Italian neurologist named Pierre Luigi and Betty who's very prominent in the story and who's now the head of the National the u.s. national prion surveillance Center at Case Western to appear Luigi was really the most important or one of the 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 the Italians remember I mean it was part of the of the very positive image that United States had and to some extent still has in Italy and you um you approached them through a contact or was a kind of a cold call you did I went through a neurologist maybe the fellow in Chicago actually named him the first one and contacted the Italians because you know the world of people interested in fatal familial insomnia is pretty small and you know I speak Italian I've an uncle of mine was a screenwriter lived in Italy was a screenwriter it's Utah and so I spoke Italian so obviously that made a huge difference but I really think that that that what mattered most was just that they were just in this moment where they were hopeful you know they had made this discovery about what they had and so you know they were interested in letting people know about fatal familial insomnia I think in the hopes really of kind of starting a non-profit or fun no this is before GoFundMe right but it was like the idea was like something like that would work for them but getting the disease attention would help them to get the resources because these these diseases are so expensive to investigate and so that would that I think was probably the biggest the biggest thing of all and then I think last it's also true that that Ignazio Reuter who was the doctor who's married into the Italian family is it true humanists I mean he always thought it was sort of being like the the Erasmus of the the venit l-like he really he liked information he likes stories and he was supportive of it and I think he a lot of ways brought the family the family along you know I'm certain there wasn't I it was certainly wasn't my technical knowledge God knows I mean I learned from the I learned from him he really told me about prion diseases it was actually kind of surprised when I heard that the family had given you all letters written from all wonderful letters yeah yeah know that I mean there's a there's a moment in the family couldn't sleep when the Americans are bombing the docks near Venice and I'm quoting from these letters that this the generation I was working with their grandfather had been a 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 as Americans were bombing in the hospital area and it's an extraordinary letter I mean all the letters back and forth among the family members you know this is this is near when phone calls were unreliable but even during the breakdown of everything else in Italy the mail appears to me well I mean I know the mail ever works in Italy but it was working as well as well then as ever and so and the family kept the letters and they and they gave them me allowed me to quote from them are you still in touch with any members of the family I haven't been for some years so as they say you know families go through I think periods and this didn't have anything to do with 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 gonna carry the banner of ffii any longer I wouldn't be surprised the new generation rises up that it will do it again I just think at that moment the the courage it takes you know kind of comes and goes for a family you'll see names of families and different diseases from time at a time and the paper and then you may notice those those people aren't always the same the same family that that marches forward if you take a disease like Huntington's disease or whatever and you also should remember that when I wrote the family 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 couldn't sleep sort of prediction and sort of doesn't is that research has gone very slowly and so this book was published in is it 2006 is that right yep 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 is at least one researcher who back when I researched the book which is around 2004 said you know in a decade we'll have a cure and then the Italians would laugh and they'd be like ah ha you know a decade ago they told me that but the times are cynical and worldly in general the researchers not the family and of course now I looked at you know it's amazing how little has actually gone on which is another way saying how hard the research is I mean one of the things that the family couldn't sleep tries to show you is how fun has changed in the in the world of medicine so there's a this guy I'd like to talk about more later than currently guy to Shaq who discovers kuru and it's actually incorrect although it's in his obituary everywhere that he that he found out what caused kuru which was mortuary cannibals cannibal feast venereal mortuary feasts he basically was funded by the US government you know with a check whereas the later generations of prion researchers had to find you know what's very familiar to researchers today I don't have to tell you science researcher Jenna you have to fund your lab you got to write your grant proposals you know and so his successor so come on guys check wins the Nobel Prize basically for his prion research what wasn't called that in nineteen think it's 1976 and his successor Stan Lee proves there also wins the Nobel Prize for his research in prion disease but he does it partially with you know funding from private companies grant proposals I mean you know there should be there should be a nobel prize for getting grants I mean that the amount of work his lab puts into the funding is this extraordinary compared to Carlton gotta shake so the books also A Tale of Two side to science industries to science cultures in the United States now they've changed in 20 years I think you say that in two thousand one prisoner was the most highly funded researcher through NIH to the some of he's certainly gifted and good at it and I think what's also one other thing he predicted I think trigger word that's in the book is that I forget what year but he's talking to a meeting of prion researchers if and it's kind of at that height of the Mad Cow concerned because Mad Cow is a prion disease and he says something like you know this is the largest number of prion researchers who will ever be gathered in her room and everyone looks around the panel wondering out what is you know what was that mean but we all be in different rooms you know and know what he means is funding has crested like you know this mad cow disease has has because they wanted to test for cows most of all I don't know how many of you are listeners when we remember what mad cow disease is or was but this is a this is a a disease in the cattle in Europe principally in England in the 80s which causes cows to have aggressive symptoms like charging 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 and after some very very very good veterinary research its traced to a decision that the British made a few years before to put rendered cow protein into the feed given to the feed given to cows and prion diseases are hard to transmit it helps if the protein if the receiving protein is similar to the infecting protein so there's nothing more similar than a cannibalistic cannibalistic needle so now when humans start eating mad cow disease he starting cows infected with with BSE which is the bovine form of mad cow disease a hundred and seventy or so who gets sick and died with appalling symptoms and I mean I don't know that there's it again I mean you're you're you're capable listeners will correct us both but to me that a hundred and seventy still remains on some level like the largest clearly delineated you know single innovation food disease error you know committed in our lifetimes I mean I'm sure more people die from salmonella poisoning every year but with a specific change in something that results in such a catastrophic results it's very very unusual right I mean you rarely it's an unusually clear scientific outcome but what's also interesting is that it was only a hundred and seventy or so people write and I interpret in 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 a hundred and seventy seven known causes known human deaths from eating infected cattle that's only about ten or fifteen more I believe maybe even less than when I wrote the book so it's essentially the number of deaths from mad cow stomped in humans even though the infection is widespread recent studies have shown that you know whatever that that there are hundreds of thousands of people infected by prions they did they take when people have their appendices out or appendices out in England they often do tests to see the presence of malformed prion proteins and they continue to do them in did you find an astonishingly high rate of infection but people aren't dying from the disease you have a figure in the book where I think you say that the late eighties and nineties the British ate about six hundred forty billion doses haha a BSE and it's really kind of astonishing Public Health mistake right they you know they they're kind of nationalistic about it they didn't want Americans involve the bureaucracies involved they didn't want to harm the British agricultural industry so the direct information to get out and the result the you know I think you did he say you're kind of suggesting that one thing stop this from being a massive catastrophe is this stuff is pretty hard to transmit and even gets in your body you know I was gonna ask you guys what's what's the most efficient foodborne illness anyone have any idea I don't eat coli is e.coli or does he call I always get you sick if you consume it I don't know I'm just guessing but there are different strains of it also right this may be a it's making the example the least you're trying to say right I'm just saying you know it's extraordinary but you know what it also suggests you know and the Family Clinic tries to bring this out is that we don't there's a lot of things we don't know so for instance a recent study of infectivity in in British people who had conceived doses a BSc completely reversed a previous one and showed that there's a sort of there are people who have polymorphisms at the the relevant you know prion gene and it was shown that those who had valine at some spot on the team head were far more likely to contract vs even those who didn't and 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 convey appendix is a sign of one form of homozygosity anyway the point being we don't know we really don't know a lot today and i looked up some of the names where i've been very familiar with in that time whether they're still in the field that most of them still are and most of them are still publishing but I think it's just evidence that you know progress is is 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 who have the condition III forget the books wasn't say about twenty families worldwide who have the inherited form of F advice that's a low so that's a little low but yeah it's a it's quite it's quite rare I mean to the 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 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 so book was published and a few years later I received an email from a guy named Eric mythical who just told me the story that his his wife his mother had died of FFI and that she was positive she tested positive I was kind of looking for 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 that both Eric and his wife Sonya quit day jobs she was a lawyer he was a I think 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 so it is it wasn't I don't think in 2001 that was uh sorry 2006 I don't believe that was quite the option it is today yeah that's very likely I don't think that of all the same Steve I I don't think you're gonna see the eradication of the of the gene anytime anytime soon partially because look I think but look the level of education necessary there to do embryo selection the cultural specifics for people willing to remember your selection are quite narrow I mean I don't even know you know the member of the Italian family did not have children at all a form of embryo selection rather than but but I didn't you know nobody would have accepted I think embryo selection among more catholic members of the family that's some work some work yeah you talk in the book about how members of this family had a difficult time often getting married because word had gotten out there of some disease and this is pretty common a lot of traditional societies you know families basically do due diligence on potential mates um it's less common here even in Italy today you know people this is something so different between the US and and and and Europe is that people don't go that far from their homes I mean even if they work in Paris they go back to their villages where their families are for this for this for the summer you know and that's also true in Italy so like you wouldn't have to do due diligence everyone would have known the family and everyone have known who the cousins and who are the second cousins you know it's it's it's a very small town environment even today and even though many of the more educated going to the cities they still retain their the relationship with their hometowns in a way that I don't think has any counterpart in the u.s. I mean you know people really move in the u.s. I'm I think they don't have relatives they leave behind but they move so I guess we haven't added that this disease is autosomal dominant right if right if if if someone's a carrier then children has a 50% chance of inheriting the disease correct so let's take up that's sorry a geeky question what happens if you get two copies is it really bad you die early does that is that no that's a great question I I remember asking that question and I don't remember the answer there there is a slow and a fast form of the disease but that's not that has to do with your um again the hetero homo zygosity question at the at the at the gene I don't I don't know but if you think about what are the chances of getting true copies yeah very rare but you know maybe sometimes people marry their cousins in Italy I don't know I think they probably do but but I just I don't know I I think that that that it's sufficient that the you know the unhappy parts of the disease is essentially a hundred percent of those who carry the gene develop disease that's the other bad news 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 and it runs in this family and they said when they had family reunions they would all wear name tags because it couldn't actually tell each other apart that you know there's a lot of people in my world claim they have face blindness but I just think that they don't really you know it's it's a continuous trend so I don't care you are it's it's a continuous trait some people are better than others some are suitable recognizers there are apparently people who can they meet somebody and then 10 years later they pass them on the street and they can remember the face yeah at the New York we did an article about the the British have a squad of people who called super recognizers who can match I guess video images with drawings I don't remember the exact details but but like those dolphins and day of the dolphin who you know are able to intelligently communicate and are then turned to weapon I've uses see now we don't need those people because the AIS are so good at face recognition that's right your honor they're on their way out there was a professor Mike shears undergraduate college Stanley Rabinowitz who would every year he'd memorized the 400 people in the student face book and I was in my hometown about you know 10 years ago and walking on the street and I see this guy and he just starts staring at me and he's like um he smiled at me as Rabinowitz I hadn't been there in 25 years 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 coach that we wore and so he he was kind of an oddball guy in high school but now we understood why it seems a complicated talent to recognize the face it's interesting that AI is now good enough at it yeah you know it's uh 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 they were quite good at recognizing each other so let's look at the science in the book and as you say you're pretty character driven and you're two central characters Carleton GAD your second Stanley prisoner um are complicated to say the least I think at some point in time you quote a colleague who says that God you SEC is an egoist and prisoner is an egotist right but you know more deeply guide your sex a pedophile and prisoner seems like a jerk and so you're writing this book and your face to the fact that these two people who could presumably under other circumstances might be heroes of your book just aren't really suited to it I think that's a very good observation because I think in a funny way the family couldn't sleep is a book that's searching for a hero who hasn't yet appeared and maybe to some extent Erica Sonia who I referred to before who is or the American couple who quit their jobs become prion researchers or the heroes that the family couldn't sleep doesn't have so the hero of the family couldn't sleep I would I would say is is simply the human instinct to survive but if that's not quite the same thing as having somebody named Eric and someone named Sonya there were a number of difficulties in having to such unsympathetic star researchers but for me to be honest they were they were preferable to having more perfect people than that role because first time in Carlton took Rowan guy to shake who died a few years ago right 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 the county locked up I could be wrong people can fact check these kinds of things never very easily on their laptops but but it's at least true that he wound up in jail and he wound up in jail with one charge I know the technical charge was I'm sure Bayesian molesting a minor because when he got so he he was a guy who grew up in yonkers always wanted to be a great scientist stencil the names of great scientists on the on the staircase up to his attic and 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 we wanted to do in half an hour getting on on this on this group of this tribe that was dying young so this coincided these trips to the South Pacific coincided very nicely with his interest in underage boys and he he came to believe I don't know what how much basis in fact that there were man-boy love or 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 because at the United States and he sets up in suburban Maryland right near the NIH office is a huge house full of young man and it was a question even then in a more naive time what did Colton guys should want with all these young men from the spunda from the South Seas you know from Micronesia and so on he added them he sent them to Western schools and they don't often go home to their countries that they came from and be major you know participants in the life of those cultures because they he came with these with these blue-chip American academic degrees which were very very valuable and a cultural knowledge that many of their 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 and I don't believe the diaries are explicit but I was not able to in all the Diaries I read many of them they're there they're there somewhere you can read them i camera at the Library of Congress or something like that so but what was for me was fantasy about Carlton selling Macomb went to jail and then after went to jail he went to Europe and I caught up with him in the Netherlands where he was living in a kind of student hotel a residence and enjoying life is a kind of 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 than we're interested in he was a Big Ideas guy you know I mean he was you would sit with him I would sit with him I mean I spent a lot of time with Carl and he would you know sputter on about how all of all of life involved conformational you know come from protein conformation was a model for life for things things that you know who's a big picture guy liked the idea that you could actually be funded today seems almost laughable you know maybe so far for what you would fund but so it's really interesting about Carlton was Carl and Isaac the one thing people know about Carlton kind of shook today if anything is that he discovered that for that the kuru among this happen to be getting a tribe called the or array was caused by mortuary cannibalistic feasts 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 uncle about the arm like a neck 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 like the book points out very clearly and I'm not the first one to point it out that surely Lyndon Bell who was an anthropologist hired by the Austrians is actually the one who figured it out so Carlton goes and he was a maniac for bleeding and he took like he you know he he took he took blood samples from thousands of for a and he examined he was looking for you know potential potential contaminants in the wood smoke at there at you know at their um at their fires in the water you know he did all the standard epidemiological things and he knew that they practiced these mortuary feast but he never associated the one with the other he may have thought the feasts were too infrequent but a really really good researcher was a little less full of himself I would have noticed that the people were getting sick were the women and the children it would have looked at who seemed protein was prestigious in the for a world peppered are getting and so the men got the best protein and the women and children got the you know more tray even then I don't think eating your uncle is considered a really proud source of protein so so the reason women and children were dying predominantly of this disease was precisely because the vector was the funerals and surely linen Bell who was an anthropologist never had an ask question Carlton never really was a good I would sit with Carl and I would feel like I wasn't even in the room you know again and again he go on about about you know about things that were abstract and interesting but you know there were theory stuff really graduate student bull sessions so that's Carl so Stanley prisoner ciccone wins the Nobel Prize in 1976 and basically he's right he basically says there are kinds of viruses he calls Onslow viruses that can exist in the system for 20 years before causing damage which would fit the which would fit the kuru you know which would fit would crew the sort of the case study for kuru but he didn't actually recognize where that where the infection came from that was this other woman we did not get the Nobel Prize and if you look at the obituaries of Carlton they still say that he figured out that cannibalism calls caused kuru when in fact you just spent a lot of American taxpayer dollars not figuring it out so fast-forward 10 15 years and Stanley prisoner comes on the scene so you know when I say Carlton egoist I sort of mean Carlton's a narcissist or was a narcissist he really saw nobody but himself and it's a very powerful theoretical scientific mind but it's not a very good epidemiological scientific mind for obvious reasons Stanley was sort of the reverse and 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 you know he was just an egotist he was just well you referred him as a jerk I think you know he didn't he didn't he was good at getting credit he was good at doing the work he was going to getting talented postdocs to work for him he was good at publicizing prions and he named them so prions before that were called slow viruses and he renamed them for proteinaceous infectious molecule what's right for your prion stands for exactly it doesn't seem to quite work but something very similar to that and he knew he had a great term and so you know imagine the the nerve right to name a disease principle before you even know what it is I mean what if what if what if researchers were always naming things different things just because they thought they were different so you suggest actually Daniel sorry that he named it prion because it sort of sound a little like prisoner is that I I thought it was because it sounded space-age no no it's in the book about yeah yeah I mean in retrospect I think he I think it sounded sexy because you know prion look and a lot of ways biology once that wants to be wants to be physics right and so especially you know the discoveries in physics are sexy compared to discoveries in biology and so by making it sound like it was some sort of quark type discovery I think he woke up a lot of a lot of journalists would have otherwise kind of gone at the idea of the theories I mean if I tell you that there's a protein that causes infections like a virus does that may excite you too but or maybe not but you know it's not it's one step shy of what excites the front page of New York Times you know he was good at that I'm not criticizing I think that's a useful talent in a way the book means to be a parable about what what scientific talent was useful right after the war you know 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 protagonist of the story the prion really had to be the protagonist of the story you know did you talk to prisoner I talked to prisoner briefly and and obliquely and through cutouts and and there were some emails we exchanged I never got his wholehearted love he was he was just brutalized in a piece that had come out a couple of years before by the science writer Gary tabs and Discover Magazine and he'd never recovered I think from the idea that that you know journalism can go both ways for you I don't think he expected it I mean was I wasn't going to brutalize him but I don't think he ever quite felt comfortable again with the odd being even so you traveled to Italy you talk together SEC did you ever go to Papua New Guinea cuz a lot of the book takes place there yeah I didn't I I would have liked to have gone there but in uh in substitution was I like I like to deal with written records a lot there's no one left in Papua New Guinea you know I mean what would you be looking at exactly except getting a sense of the landscape and the world they're so different but the original reports from the case officers under the Australian government are also lodged at same one of those odd things somebody copy the entire case report from the colonial officers and so I was able to get access to those reports and I used them and I also interviewed a few people who were still alive from that from these kind of crazy I'm it would make a terrific you know a Verner Hertzog movie there Carlton guy – Scheck going through the the forests the jungles of Papua New Guinea like with a needle plugging everybody could find and taking their blood before they could give consent whereas you know I don't think Stanley Stanley Prue's er as a movie is very difficult to imagine and requires a much higher level actor I think and a much higher level the director I mean can you guys think of a single movie that makes you know lab work sexy in the modern era the movie about Elizabeth Holmes and Theron oh s— just kidding for all the wrong reasons right right but I mean you know what I'm saying it's like it's like you walk into a I mean as a narrator you know that narrations of great importance are going on in labs right I mean it's obvious that's where the knowledge is being constructed much sort more so than enough and whatever out in the field right I mean you know Harlan Ida shakes is 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 getting harder to all the time I think I mean 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 in connection with I noticed the connection with prion disease and the possibility that you could you could edit the prion gene I mean we don't really know what I noticed it's 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 yeah I think you say was the one fat so at some point I think they delete it in the mouse and didn't see any phenotype I know if that's still true that's true yeah no that is that is still true what's happened since is that I think this idea of infections and prion has actually spread quite a lot more cuz there's some discussions to other Alzheimer's disease might have an infectious component yeah I mean they're always was there's a chapter in the book about why you know kind of a why you should care chapter since you're very unlikely to have a prion disease and you're lucky enough not to be beefed in England during a brief a brief window in the 80s so why do you have to care and you're right I mean there there is 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 sort of bare-bones science of it would be that if one malformed protein touches an adjacent properly formed protein it causes it to change forearm into the pernicious or disease-causing form of the protein and then that seems to be I mean there's no doubt that amyloid plaques are an embodiment of that principle the questions are they do disease it's you know I mean like can you really refer to amyloid plaque is an infectious disease it doesn't seem to quite work in that way I did me one person a veterinary researcher one thing I loved about this book was it was just so weird you know I mean I can't emphasize for you how how much I like going down strange dead ends and so a lot of the book portion of the book is about how how they might have diagnosed fatal familial insomnia with like the instruments and the tools and the understanding they had when the earliest patients that the family identified I'd it was this doctor and in Padua he may have had the disease you know I mean it's not a bad guess but I spend a little bit of time also for instance on the British hereit's the priests of their towns who would notice the thing that we were trying out with the sheet because scrapey is a prion disease in sheep similarly I met a veterinarian you know not a super prominent veterinarian somebody who's in the literature for prion disease but as you guys know like for every one research of the world knows there are hundreds of researchers doing even equally important work who just aren't in the paper you know what I mean and and the people in their field know who they are so there's this woman and she had played a role and when when primates were a focus of 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 what year was this her name's Rosalyn Ridley I believe I'd have to look up her name it's Rosalyn something you can dig her out of it's in the book this is mentioned but you know it's never been replicated if anyone tried to replicate it you know and it probably nobody's tried to you know I mean it's it's just it just flies in the face and I don't 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 that she wasn't getting a hearing on it you know one of the people we just interviewed is Stewart Firestein he's a professor of Columbian neuroscience and Stuart's theories that the way she teach science is through narrative rather than simply giving classic experiments at results you want to kind of present the mystery right what something looked like at the time and why people sort of believed a particular theory I think actually the case of prion disease in your narrative is kind of a classic example of that because at each stage you can really see why people might hold on to this predict the wrong conception and how it took was it took a lot of effort actually to show that DNA or RNA were not involved you could always find some way suggesting it still was involved an undetected huge amount of money to prove to to come as close to proving that you know and I think we're all pretty satisfied with the proof at this point but I mean a huge amount of money just to check off that that box then some of the old-timers still don't you know believe there was a recent study where I think they just used genetically modified ecoli and they basically put in the malformed gene and it pumped out the prion protein then they injected that and that's about as close as you can get to finding something that has made it's possible yeah but yeah but just back to the byways like this something that I I enjoy the failures too you know what I mean like I mean in a way prisoner bless his soul was the least inter the character to me cuz he's a guy who he's a guy who never quite missed but also in a way never quite you know he his joy was very not muted but very modern in the way that modern things are so hard to narrate where's Carlton guy – Scheck was really like like he was like a 19th century figure he was a he's a madman and it's just an absolutely insane guy who's you know you could say that that any successful scientist is that is the some of his or her unn you know much like an artist of his or her unkind of uh uh sublimated urges and so we can argue that Stanley bruiser and I'm making this up you know wanted to be famous in high school because whatever right and when he and so he was driven by this desire to have glamorous women and a huge grant budget this public's true but Carlton guy – Ecch I mean a man who who is so 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 have passed we had passed the age of child rearing or I'd had children and so we had served our genetic function he used to tell other of researching friends that that for his health he had to have one orgasm a day no wise one but in anyway by the time I met him I I believe he had had a prostate cancer and I think he would he had that was no longer on the no unready agenda but anyway my point is that he was he was eminently narrable I would love to see somebody nary cruiser but he you know he won't let you that's the other thing right if we won't let you makes it makes it even harder I acted in one of the great distortions in science and in the reporting on science not that you were asked to you what is one of the great distortions in science and in reporting on science is that you know personality plays such a big role when personality you know the ability to tell your science is what gets my attention but that's not the same thing as getting good results or important 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 who you know I'm his English wasn't really very strong and I couldn't figure out what they were saying and so I would naturally gravitate to the one or two native speakers in the room you know a science lab today it's a polyglot and I would take their versions of events so obviously I was in the process discarding other ideas other other theories other other focuses and I know and I was aware of it at the time but you know 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 you know the kind of questions that a narrator that a narrator ass I think that what was wonderful about Carlton I'm not sure I didn't sort of I didn't Carlton 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 the other writers written on prion diseases he he energized the field really he carried it through this period when it really was nothing you know what I mean I mean there was no there was it was just a scientific curiosity it was just a few people you know it was a tribe I mean it wasn't it was it was just a curiosity and his work was important for people like proves I mean bruiser he's the same guy to check but but there would be no there were having no cruising air without him for sure it's when you have a one court I really like about prisoner um you say describing his papers uh 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 now it sounds like it's quite different from gagoosh ex approach which yeah again you know not to be not to be a Marxist about it but a lot of it does come down to the funding I mean Carlton only had to excite one guy really which was this guy Carlton kids who funded him whereas like you know I imagine that a lot of those papers from Stanley parousia are papers where he's he's showing the appropriateness of the grant he was given into the research right I mean he's got results like he says in this we will use this money you know to try and purify PRP and prove you know that there's no X attached now and then he's like we have purified it and proven that there is no exit at all he's like well now I need the next Monday that's something that Stewart our Stein actually emphasized a lot I think something this part of our new kind of more modern understanding of science is just how driven it is by really mundane things like need to get the next grant 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 open in New York and Jim is like maybe the 26th richest person on the planet and he just funded it the splendid thing and you can see how relieved the researchers are that they don't have to apply for grant money you know it's the biggest I don't know I don't know that there's that clear of advantage being there as opposed to say Princeton except that you don't have to spend your time applying for grants I don't say one other thing even even for that Institute the price of electronic subscriptions to journals is is like it gives them pause the 26 richest man on the planet and he's like you're paying what and you're consulting at how many times do you know this guy Steve yes Jim Simon's was a famous mathematician who did some work in low-dimensional I guess you want to say well anyway he's famous for something called the chern-simons term that's actually used in physics and what's interesting about him he was also a code breaker at the NSA and then he actually started one of the early hedge funds which was very Qantas 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 fun he basically got into science philanthropy and hence the Flatiron Institute so you know grad Feng has been an issue we've talked about it just it's part of the granularity of science it's essential part of how science is done I think people are now being discuss it in a way that philosophers of science hadn't done because they weren't SCI just and didn't have to get their grants funded the past hundred years one last topic dan I'd like to find out what your what's been your experience since the books gone away right have you remained interested in this topic you just let a couple look at this go do you check the news periodically yeah I mean that's a it's an interesting question because I think what's kind of weird about hard science like this is it take is it slow and it goes up against the human lifespan so I'm still in touch with especially with Eric and Sonya the Americans where they she's the one who has the gene and they become premature so I keep up with them I keep up with some of the researchers I keep up with the food illness issue in the book wizza of Barry was very widely translated it was translated fennekin's 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'm I remain very interest in the question of of a foodborne illness and why why what happened to be ASC so I just looked it up as it happens and you know there were no cows down in the United States with would put mad cow disease for like ten years and there was one three years ago one last year so when I wrote this book all the institutions that are now under consistent attack we're pretty much above scrutiny and it was the extremists of the sort of food purity movement who no matter what wouldn't believe that the USDA was testing enough cows you know USDA this weird double mission that it both is in charge of the business of Agriculture and the safety of you know the say mister active the British organization Pradesh organization Institute you point out this huge conflict of interest yeah so so now we're going to 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 break how people would respond if the government assured them that food was safe to eat both in England and here and so that's the kind of thing that still really interests me about about the subject you know because the family conceive is really about it in a certain way an unsolved question biggest unsolved questions like why do we get sick right but the more lovest small or all questions like why BSE isn't more infectious right why didn't it kill all in all of Britain you know why they are there's this extraordinary prion disease and yet it really doesn't matter to anybody and a practical I you know when I go to my doctor I always say I often talk about the book you know the various doctors and I go yeah we spent a day on prion disease in med school we've spent an afternoon on prion diseases in vet school and some of them have seen somebody who had like crate slide Jakob disease which is another form of prion disease and so I'm always interested in the extent to which like this this thing has penetrated the penetrated the culture and then of course as you say like another way to look at the book is that a typical addict insomnia all right I 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 right this is wonderful quote from I think it's Alan rectum stopping 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 you know like we don't really have that yet every so often National Geographic does they cover like the science of sleep finally explained and I read it I'm like no the science of sleep has really not been explained yet and I had to see with twins explain first sleeper in or prion disease I'd say the more useful thing to learn about would be sleep but again basic research and sleep is very very hard to fun you know I think we are getting pretty close to it the result that sleep seems to clean out these kinds of complex proteins town they'd amyloid in interstitial spaces like that's pretty important because as those build up you begin to get symptoms of Alzheimer's which is why you know we all feel like our IQ goes down as we were sleep-deprived right and if that's right that's fairly essential overtime but is that work 8 hours of lying in there in an open savanna yeah because it becomes toxic right if you allowed to go you know over a couple of days well I mean as someone who's experienced pretty serious insomnia there's nothing worse than like not being up sleep for a couple days you actually have to begin to downgrade the things you do you know I actually I can't write write very well or I can write in a kind of overview level but I can't actually begin to compose really sentences that I'm really happy with so I'll begin to do light editing and so forth it's kind of a problem you just yeah I mean I think insomnia is the great undiagnosed disability like my wife doesn't sleep well and there's no question that it affects every aspect of one whose quality of life yeah you look a little tired there Corey I think I should go soon just because I see my children are returning to the to the hut do we have more no I think we have covered what we need to cover today thanks a lot Dan this is a pleasure thank you guys for having me thanks Dan fire enjoy Tom look forward to it again alright thank you well

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