Nancy WEXLER: I think for my mother and for our family, the whole family was very important. You know, she was very kind to us, and she was very loving and very warm. I think a lot of my warmth 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, Leonore Wexler. She was 53 at the time.
WEXLER: Mom was on jury duty crossing a great big street in Los Angeles, at 8 o’clock in the morning, very well dressed, very well-kempt, 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 gait was one of the very first signs. She called my dad in a panic and said, “I think this horrible thing is happening,” and she was diagnosed that afternoon with Huntington’s.
Huntington’s disease is a horrible thing, a fatal neurological disorder.
WEXLER: 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, obsessive-compulsive disorders. And also cognitive problems, so difficulty thinking, remembering, executive functions, 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 percent chance of inheriting the gene, which means you’ll 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.
WEXLER: 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 our entire family is that my father, being totally brilliant and optimistic, said, “Well, you know, let’s just find a cure.” So in 1967, our family started the Hereditary Disease Foundation. Curing Huntington’s has 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 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 U.S., the disease affects about 1 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.
WEXLER: 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—IT15. It was on the short end of chromosome 4.
WEXLER: In the middle of this totally tiny, teeny tiny, stilt village in the middle of Lake Maracaibo. It was these families on the stilt 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 and 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, at the age of 63. The test had not been available in her lifetime.
DUBNER: 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?
WEXLER: Why should she have?
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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?
Emily OSTER: 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?
OSTER: 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 you were going to 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, and you’ve got one parent who has Huntington’s disease, you have likely already started to see its effects on your mother or father, and you know that you have a 50 percent chance of getting sick, yourself. Emily Oster was intrigued by how people responded to this dilemma. She teamed up with two neurologists, Ira Shoulson 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?
OSTER: So we see differences on a few dimensions between people who know that they carry the Huntington’s mutation and [those who] 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.
DUBNER: What about if you go back to people who are younger and acquiring human capital, more college, more job training, things like that?
OSTER: 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 that 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.
DUBNER: What about marriage and having children?
OSTER: 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 analyses of this data, it looks like fertility is relatively unaffected by the disease.
DUBNER: Really? So that’s surprising to me, is it to you?
OSTER: 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 sort of quite difficult for me to think about.
DUBNER: Now, let me ask you 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, according to the Mayo Clinic, as well as difficulty learning new information. But also, depression and 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.
OSTER: When we look at education in particular one of the tests that we do involves taking people who have no symptoms who are tested early in life before they’ve completed their education decisions, but at that time are completely asymptomatic, so there is no sense in which there is a difference in whether they’re sick. And so then when we compare 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 Freakonomics Radio: 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?
WEXLER: You know, I think this is something that is horrific information, very, very powerful information.
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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.
OSTER: So for the people who had not been tested and know they have a parent, 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 a 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 just in our data, this is 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 BRCA testing for breast cancer markers where we haven’t seen as much testing as people might have thought.
DUBNER: When you say very few you mean how few, Emily?
OSTER: So in our population, something like 5 percent of people who are at risk get the test. So these are people, of people with a 50 percent chance of developing Huntington’s, only about 5 percent of them choose to find out whether that will happen.
DUBNER: That number was to you, I assume, shockingly low, yes?
OSTER: Yeah, that number seemed very low. I certainly would not have expected it to be 100 percent. But I think 5 percent seemed really very small. And so I think sometimes, you know, as economists we think information is good. I think we do need to ask the question, is information, in fact, good, would it makes 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.
DUBNER: And I guess also you need to ask, think about, is it better off for whom? Is it better off for them or is it better off for the rest, you know, their family, friends, society, too?
DUBNER: So do you have an answer to that question at all?
OSTER: Yeah, so I think in some sense, in some sense we do. So what we do in our paper about testing is we try to think about, exactly as you asked, the question of why people don’t want to undertake this test. And we go through some stories I think economists would like, like is it because 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 and that that may be very valuable.
DUBNER: What does that say to you just about 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?
OSTER: 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, or standard economic models would basically say we only value the second thing, the only thing that’s beneficial of a cruise is the literal experience of being on the cruise. I think that what this says 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 benefits, or real costs for you.
DUBNER: Well, let me ask you this then, and we should make clear, your parents are both economists aren’t they?
OSTER: That’s true, and my husband.
DUBNER: And your husband. And you have a child who’s what, a year or so old?
DUBNER: Two, that child will almost certainly then be an economist.
OSTER: Right, it’s almost inevitable.
DUBNER: Well, here’s what I wanted to ask you. I mean, we do know that economists think about the world differently and 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 and maybe there is something to be said for it.
OSTER: No, absolutely, I think that I have come to think that in fact 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 would have said some people have that preference. 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 some many people.
DUBNER: And it doesn’t weaken your preference for certainty at all.
OSTER: 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 was “Limited Life Expectancy, Human Capital and Health Investments.” Now, if you’re an economist, that’s the way you think. If you listen to Freakonomics 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.
WEXLER: 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.
WEXLER: You know, “Genetic Adverse Selection: Evidence from Long-term Care Insurance and Huntington’s.” 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 percent of the people at risk for Huntington’s decide to take a blood test, the blood test that she helped develop, to see if they will get the disease.
WEXLER: You know, I think this is something that is horrific information, very, very powerful information. If you’re somebody who has a 50 percent risk as most people at risk around the world, there is nothing, nothing whatsoever that you can do that makes any difference whatsoever, nothing, nothing, nothing, nothing, nothing, nothing. There’s no treatment you can take. There’s nothing to forestall 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 has written about Huntington’s disease within the Wexler family. Now, remember, because their mother had Huntington’s, they were both born with a 50 percent chance of getting it.
WEXLER: Well, I think that both my sister and I, we both thought, well, wouldn’t [it] be fun since we found the marker and the gene, wouldn’t it be fun being the first getting tested? So we all, you know luckily my father is a psychoanalyst and said, “What are you doing? What are you doing? Fun? What are you talking about fun? What kind of fun is that?” And I think, you know, having him actually express a reality, you know, 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, was helpful. I think another big, huge stress that people do not appreciate enough is being watched because once you’re a public figure like me, like my friends, everybody watches you. You know, “Oh does she have Huntington’s, does he have Huntington’s?” You know?
DUBNER: Now, 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?
WEXLER: Well, I’ll tell you I usually say that is 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.” But I think since you’re being very friendly, I haven’t, but again, I think it depends an awful lot on what kinds of treatments actually would make a difference.
DUBNER: Of course. 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 one parent who has Huntington’s. 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?
WEXLER: Well, the reality of our country is that if a person is known to have even a risk for a genetic disease, for Huntington’s in the future, they quickly become an uninsurable person. If they have a job, if it’s a job that requires education, or even dexterity, or skill, you can lose the job. And G.I.N.A., the Genetic Information Nondiscrimination Act, or GINA, 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 GINA. People think, I think, everyone thinks really that in some secret soul of their bodies or their minds that they’re truly unattractive and that nobody if they know this, they won’t love them And so people will just get into relationships that are brutal, violent, disparaging. I know a woman who dated every single horrible person she could possibly find. No, literally. She thought, “Well, nobody’s going to like me, so I might as well lose my virginity on a shit.” And that you know, I think that we do that. We have our identity very much tied to who we think we are. And especially, you know, when the outside world is saying this is repugnant, this is repulsive, then we act it out in bad ways. It is toxic knowledge, it is difficult. If we could have more conversations about the knowledge, you know, before people are saying, “O.K. Well, it’s too expensive for you to live in ambiguity so line up here. I mean, I think we have concentration camps where everyone has to line up here and 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 physiologically or genetically different. The only way 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, my kids, you know, and it’s not going to have this kind of incredible retribution. Then I think, well you know, that’s a 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 Huntington’s but haven’t been tested. We thought you might want to hear from them, too.
MELINDA: My name is Melinda Maher, I’m 25-years-old and I’m living at risk for Huntington’s Disease.
CHRISTIE: My name is Christie, I’m from North Carolina, I’m at risk for Huntington’s Disease.
SHANA: My name is Shana Martin. I’m from Madison, Wisconsin. I’m 33-years-old. I’m a world champion lumberjack athlete. So I compete in the sports 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.
MELINDA: 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 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 would be afraid of that independence.
SHANA: I’m getting married in a few months, and we’re starting to explore having kids. And we chatted about it, I’m still quite firm that I don’t want to know. So firm that I’m probably going to invest quite a bit of money in in vitro fertilization just to not find out and to make sure my kids don’t have it.
CHRISTIE: I get up every day wondering, do I want to be tested for Huntington’s. And I’m 40-years-old, a college graduate, working for a large university and very happy with what I’m doing, but I can’t say it doesn’t haunt me every day. And so the longer I procrastinate against testing, I guess the longer I can say, “Well, I don’t have Huntington’s.” Does that make sense?