I think for my mother and for her family the whole family was very important You know she was very conned to us and she was very loving and very warm I think a lot of my work I get from her. That's Nancy Wexler. She's a professor of neuropsychology at Columbia University One morning in the late 1960s something strange happened to her mother Leonor Wexler. She was 53 at the time Mom was on jury duty causing a great big street in Los Angeles You know eight o'clock in the morning very well dressed very well camp and the policeman just screamed at her and said You know aren't you ashamed of yourself being drunk so early in the morning?
And she realized that her unsteady gate was one of the very first signsum She called my dad in a panic and said I think this horrible thing's happening and She was diagnosed that afternoon with Huntington's disease is a horrible thing a fatal neurological disorder Huntington's affects every aspect of mind thinking and body It causes all kinds of uncontrollable movements throughout your body your face Wiggles your arms move and then also people very often have very severe depression hallucinations delusions paranoia of sexual compulsive disorders and also cognitive problems so difficulty Thinking remembering of executive functioning, you know, so every aspect really of mind and body and thought One more thing about Huntington's it is purely hereditary Which means that if one of your parents has it you have a 50% chance of inheriting the gene which means you almost certainly get the disease itself Nancy Wexler's mother had already seen all three of her brothers die from Huntington's as well as her father You know, she knew the fate that was before her. In fact at one point my mother said I'm not living this way and she tried to commit suicide I think the only thing that really saved her entire family is that my father being totally brilliant and optimistic and Well, you know, let's just find a cure. So in 1967 our family started the hereditary disease foundation Caring Huntington's is like turned out to be vastly more difficult than we thought but I've been working on it ever since 1967 Nancy Wexler had just graduated from college She became a psychologist and she took over the hereditary disease foundation that her father had started She funded research organized symposia in 1972 Wexler learned from a colleague about some villages in Venezuela where Huntington's ran rampant In the US the disease affects about one in 10,000 people But in these Venezuelan villages where families had intermarried for years the rate was far far higher in nearly every household At least one person had Huntington's so Wexler began traveling to Venezuela She collected blood samples and she started to build huge family trees. She needed to learn where exactly Huntington's came from You know, we were looking for the gene because we knew if you could cure the gene, that's the cure for Huntington's The breakthrough took a long long time, but finally it happened in 1993 a research team led by Wexler found the Huntington's gene It was on the short end of chromosome 4 in the middle of this totally tiny teeny tiny still village in the middle of Lake Mark It was these families on the still village who literally it was their DNA that gave us the gene their DNA The idea was that finding the gene for Huntington's disease would lead to a cure for Huntington's disease But it hasn't although Wexler many others are still working on it What finding the gene did enable was a test a $300 blood test that allows you to learn whether you will get Huntington's Wexler's mother had died years earlier in 1978 the age of 63 the test had not been available in her lifetime If there had been a test available then a test like the one that you helped develop later Do you think your mom would have taken it at a young age?
No, why? Why should you have? From WNYC and APM American Public Media This is for economics radio the podcast that explores the hidden side of everything Here's your host Steven Dubner If you could take a test that could foretell your future at least your medical future Would you would it be valuable for you to know if something bad was going to happen or would it be more valuable to not know? This is actually quite a broad question of general interest to economists Emily Oster is an economist at the University of Chicago's Booth School of Business She's been doing research on Huntington's disease.
Why? The question we started with was How does knowing that your lifespan is limited impact your choices about investments in the future? Basically ask the question if you knew that you were gonna die at 60 rather than at 90 Would you change the amount of education that you got and that has actually quite broad implications for understanding things like as countries get richer And people get higher life expectancy. Will that impact their education and then impact economic growth?
People with Huntington's usually start to get sick in their 30s or 40s But there's a lot of variance even a toddler can start to show the symptoms It worsens over time and eventually will kill you So if you're in your 20s, let's say you've got one parent who has Huntington's disease You've probably already started to see its effects on your mother or father and you know that you have a 50% chance of getting sick yourself Emily Oster was intrigued by how people respond to this dilemma. She teamed up with two neurologists Irish Holson and Ray Dorsey to find and interview people at risk for Huntington's some of whom had already tested positive and some of whom had a Parent with Huntington's but hadn't been tested themselves Besides asking these people about the disease itself Oster also wanted to know things like are you going to college? Are you getting job training? Are you getting married or planning to have a family?
So we see differences on a few dimensions between people who know that they carry the Huntington's mutation and know that they don't So one is that we see more early retirement among individuals who know that they carry the mutation We see more divorce. We see individuals reporting changes in their recreation activities They're more likely to borrow money. What about if you go back to people who are younger and acquiring human capital go more college more job training things like that? Yeah, so when we look at that what we see is that individuals who find out that they carry the mutation actually get much less education than individuals who find out they do not So we see Much lower rates of college completion among individuals who know that they will develop Huntington's We also see much lower rates of engagement in job training Both of which are sort of typical markers for human capital investment.
What about marriage and having children? So we see on average quite similar rates of overall fertility in these different groups That's something that's not actually in either of these papers because it's a little hard to think about what direction you'd expect that to go But from other analysis of this data it looks like fertility is relatively unaffected by the disease Really? So yeah, that's surprising to me. Is it to you?
Yeah, I found that surprising although. I think it's difficult to think about How you would react to this disease? I think it's it's quite difficult for me to think about now. Let me say this I also see that Huntington's is accompanied by a variety of disorders including cognitive disorders For instance difficulty planning organizing and prioritizing tasks as according to the Mayo Clinic as well as difficulty learning new information But also depression things like that so persuade me that you know for a fact that what you're seeing among these people who are diagnosed Isn't in fact a result of the condition itself as opposed to a conscious decision to not invest in themselves When we look at education in particular one of the the tests that we do involves taking people who have no symptoms Who are tested early early in life kind of before they've completed their education decisions But are at that time completely asymptomatic so there's no sense in which there is a difference in whether they're sick And so there when we compare the the educational choices of people who test and find out they carry the mutation to those who test and find out they Don't we can be at least somewhat confident that it is not about symptoms of the disease Now that's different than the question of depression, which I think is also quite important And largely what we can say about depression is actually in the data We see evidence on whether people are depressed and we see people are initially the first year after they get a bad test result They are somewhat more depressed But then they basically return to baseline for quite a long time Most people are kind of about the same amount of happy most of the time Coming up on freedom you would think that if you stood a 50-50 chance of getting a fatal disease, you'd want to know wouldn't you or would you?
You know, I think This is something that is horrific information very very powerful information From WNYC and APM American Public Media. This is free economics radio. Here's your host Steven Dubner The economist Emily Oster was trying to figure out what kind of life decisions are made by people who are at risk for Huntington's disease Now some of these people had had the blood test and knew they would eventually develop symptoms and die from the disease Others had a 50-50 chance of having the genetic mutation but hadn't been tested So for the people who would not been tested and know they have a parent the the choice that we were most interested in was the choice to undergo testing So one theory you might have and this would be a theory that would be informed by standard Economics is that people should value information and they should want to get the test because even if it is a bad result Bad in the sense that they have the mutation it will still help them make better decisions in their life But in practice what you see and this isn't in our data This is in many people have shown this is that in fact there's very little testing very few people seem interested in finding out Whether they carry the mutation or not and that's actually similar to some other settings like bracket testing for breast cancer markers Where we haven't seen as much testing as people might have thought when you say very few you mean how few Emily So in our population something like 5% of people who are at risk get the test So these are people of people with a 50% chance of developing Huntington's only about 5% of them choose to find out whether that will happen That number was to you. I assume shockingly low.
Yeah, that number seemed very low I certainly would not have expected it to be 100% but I think 5% seemed really very small And so I think sometimes you know as economists we we think information is good I think we do need to ask the question is information in fact good Would it make these guys better off to know this and so should we be really really encouraging people to do this or in fact Does it seem like it would really be making them worse off in which case we definitely don't want to do that And I guess also you need to think about is it better off for whom is better off for them or is it better off for the rest You know their family friends and society too. Yeah, exactly So you have an answer to that question all? Yeah, so I think in some sense we do so what we do in our paper about testing is we try to think about Exactly as you ask the question of why people don't want to undertake this test and we we go through some stories I think economists would like like is it because of the test is expensive or people don't know about the test or And those don't seem to be stories that really hold up the test isn't very expensive Everyone seems to know that it is something that's available and then we try to think about Think about a model like an economic model of why people might not get this this test And I think what we come down to is the view that in fact Largely the reason that people don't want to get this test is because while they are untested They seem to be able to basically pretend everything is fine. Mm-hmm And that that may be very valuable.
What does that say to you about just the way that human beings as kind of decision makers Think about the future how they budget for the future in terms of you know time and human capital and money and all that I think what it tells us is that People care both about what they think will happen in the future and what actually happens So, you know, I enjoy going on a cruise But I also value the time leading up to the cruise where I get to think about how great the cruise is going to be I think often our normal sort of standard economic models would basically say we only value the second thing Only the only thing that's beneficial about a cruise is the literal experience of being on a cruise I think what this says is is in fact something that's probably more comfortable for most non-economists Which is that there's some value of kind of what you think is going to happen that that has like real Real benefits or real cost for you. Well, let me ask you this then and we should make clear your parents are both economists, aren't they? That's true and my husband and your husband. You have a child who's what a year or so old too that child will almost certainly then be an economist It's almost inevitable.
Well, here's what I want to ask you I mean we do know that economists think about the world differently. We appreciate that especially on this program I mean we love that on the other hand There is this assumption among economists and within economics that people do value information and that they eschew Or try to get rid of uncertainty because economists see that uncertainty brings about bad things but I'm just curious if the rather strong evidence that so many people embrace uncertainty in their own private lives may Have changed or nuanced a little bit the way that you as an economist think about the downsides of uncertainty Maybe there is something to be said for it. No, I think I know I absolutely I think that I have come to think that in fact for for a much larger share of the population than I would have expected It seems like this preference for living with uncertainty is quite strong And I I would have said some people have that preference It's it seems surprising to learn that basically at least in this population It's like the vast majority of people appear to be much more interested in living with uncertainty Which isn't something that I think would be true for me And I would not have thought would be true for so many people and it doesn't weaken your preference for certainty at all No, I don't think so One of the papers that Emily Oster co-authored was called optimal expectations and limited medical testing evidence from Huntington's disease Another it was limited life expectancy human capital and health investments. Now if you're an economist, that's just the way you think If you listen to free economics radio, which you do you are you probably at least appreciate to some degree that kind of thinking But if you're someone who comes from a Huntington's family Who's seen a parent die maybe and who's been tormented for years about whether the same future awaits you The economist's way of thinking can be hard to stomach I was just totally offended by the titles of these articles that came out That's Nancy Wexler again.
She's the Huntington's researcher whose mother and three uncles died from the disease You know genetic adverse selection evidence from long-term care insurance and heightened and it talks about I mean, these are friends of mine And I think every single one of these articles was terrible Wexler also understands why only 5% of the people at risk for Huntington's Decided to take a blood test the blood test that she helped develop to see if they will get the disease, you know, I think This is something that is Horrific information very very powerful information if you are somebody Who has a 50% risk as most people know at risk around the world There is nothing nothing whatsoever. You can do that makes any difference what so ever nothing nothing nothing nothing nothing Nothing. There's no treatment you can take. There's nothing to first solve it And if we actually had something that made a difference in treatment, I think that would make a huge difference Nancy Wexler is 67 years old.
She has an older sister Alice who's written about Huntington's disease within the Wexler family Now remember because their mother had Huntington's they were both born with a 50% chance of getting it well I think both my sister and I We will thought well wouldn't it be fun since we found the marker and the gene wouldn't be fun to be the first getting tested and then we So we all you know luckily my father's a second one was Fun what are you talking about fun? What kind of fun is that like? And I think you know having him Actually, you know, express the reality You know, he all of us could find out both of us actually might get Huntington's and die and that was terrible You know at least having a 50-50 hope, you know that ambiguity would go your way You know it was it was helpful And I think what another big huge stress that people do not appreciate enough is being watched because once your public figure Yeah, like me like my friends Everybody watches you, you know, oh does she have Huntington's does he have Huntington's, you know No, and you've I just want to clarify if you want to clarify for me Is it true that you've never taken the test yourself then? Well, I tell you I usually say that is Um a private decision because it's something that I think gives permission To everybody to say I have privacy.
I have boundaries. I have rights and but I think since you're being very friendly I haven't but again, I think it depends on what kinds of Treatments actually make a difference, of course. So let's imagine that there's someone listening to this program Let's say they're 18 years old or so with a lot of you know Decisions coming up about what to do what kind of family to be involved with and so on And this is an 18-year-old with a one parent who has Huntington's um talk to me about What risks that person has in testing if the test is positive, you know, how does that affect this person's life? Financially societally perhaps legally labor wise and so on Well, the reality of our country is that if a person Is known if they have even a risk for genetic disease for Huntington's in the future They quickly become an unsurerable person If they have a job if it's a job that requires education or even dexterity or skill, you could lose a job and jina the genetic Information non-discrimination act or jina Is supposed to protect you against that but actually you have to prove that it was Huntington's that caused you to lose your job That's the bad part of jina People think I think everyone thinks really that you know, some secrets all of their bodies are in mind that they're truly unattractive But nobody if I know this they won't love them and so people will just get into relationships that are brutal violent disparaging I know woman who dated every single horrible person she could possibly find because you know literally she thought well Nobody was gonna like me.
So I'm gonna have to lose my virginity out of shit And that's you know, I think that you know, we do that You know, we have our Identity very much tied to who we think we are and especially you know when the outside world is saying You know being this is repugnant. This is repulsive Then we acted out in bad ways It is toxic knowledge. It is difficult Um, if we could have more conversations about the knowledge, you know before people are saying okay, well It's too expensive for you to live in ambiguity. So line up here I mean, I think you know, we have concentration camps where everybody had to line up here, you know, behave themselves or not I think you don't want to do that.
You don't want to eliminate people who are in any way Stisulonically or genetically different, but the only way I think we can make people feel comfortable about talking Is if we really genuinely feel that it's okay to talk about it We genuinely feel that you're not going to take away my health insurance my job my life like kids You know, it's not going to have this kind of incredible retribution then I think Well, that's you know, that the world I want to live in Nancy Wexler is a professor of neuropsychology at columbia And she's the president of the hereditary disease foundation before that emily oster an economist at the university of chicago In the course of reporting this show, we also talked with a few other people who have a 50 50 chance of getting huntingtons But haven't been tested. We thought you might want to hear from them too My name is Melinda bar. I'm 25 years old and I'm living at risk for Huntington's disease. My name is christy.
I'm from north carolina I'm at risk for Huntington's disease. Well, my name is shana martin. I am from madison, wisconsin. I'm 33 years old I'm a world champion lumberjack athlete.
So I compete in the fourth of log rolling and boom running The downside is I've been living at risk for Huntington's disease. My mother just passed away from the disease a few months ago I mean right now at age 25 I hope to get a teaching job and I hope to move away from home And I hope to be able to kind of move out and be on my own and have my own life and independence And still right now I feel like if I were to test positive, I'd be afraid of that independence I'm getting married in a few months and we're starting to explore having kids and and we chatted about it And I'm I'm still quite firm, but I don't want to know so firm that I'm probably going to invest quite a bit of money And in vitro fertilization just to not find out and make sure my my kids don't have it I get up every day wondering do I want to be tested for Huntington's and I'm 40 years old college graduate working for a large university and very happy with what I'm doing, but I can't say that it doesn't call me every day and so The longer I kind of procrastinate against it Testing I guess the longer I can say well, I don't have anything. You know, does that make sense? Hey podcast listeners on the next free economics radio We ask what is the cheapest most nutritious and bountiful food that has ever existed in history technically there are pickles So I think it's vegetables as meat glue fresh reconstituted meat powder and dry and slightly rubbery there you know a piece of synecdoche for american mass bland synthetic corporatism What are they talking about that's next time on free economics radio Freakonomics radio is produced by wnyc APM american public media and governor productions This episode was produced by Katherine wells our staff includes david hermin burelee on susie lekdenberg chris vanen and calling campbell With help from ryan hagen if you want more freakonomics radio, you can subscribe to our podcast on iTunes or go to freakonomics.com Where you'll find lots of radio a blog the books and more