Do You Really Want to Know Your Future? (Ep. 131)

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(Photo: Micah Baldwin)




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?

Oster worked with neurologists Ray Dorsey and Ira Shoulson to survey people at risk for the disease. They found that people who know they carry the gene do in fact make different choices about education, retirement, and marriage (paper available here). But a second paper found that although a $300 blood test can tell people whether they will get the disease, only 5 percent of those at risk choose to do so.

To explain why that number would be so low, Stephen Dubner talks with Nancy Wexler, a professor of neuropsychology at Columbia and president of the Hereditary Disease Foundation. Wexler’s mother died of Huntington’s in 1978, and Wexler led the team that located the gene for the disease in 1993. The discovery enabled genetic testing, but she understands why so few people want to know their future.

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 … there is nothing, nothing whatsoever that you can do that makes any difference whatsoever. 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.

You’ll also hear from three women at risk for the disease about why they haven’t taken the test.


I took one of those. It offered risk diagnoses. So I altered my behavior re eating/exercise. As far as another test goes, I know the objective results of taking that one. It's the other stuff that's the problem. I say that I am not going to worry about it, but I do. But then I see how some people I admire respond to success. The fact that I know nothing about their private lives means, from my point of view, they succeeded. As my husband would say, who cares who Richard Gere is married to, you like his' films. Well, not altogether true. I do like knowing about people as people. It's very much a part of what I do. But not in the way we celebrate success here in the US. We raise some people so high up on a pedestal- as if not human. And then the result is some are so full of themselves. That, I can do without. As for Huntington's disease, or any other disease, we all are going to die from something. My understanding of such tests is that they predict within a range of certainty and not absolute certainty. Left open is the matter of individual behavior and that, other than a gene altering pill, there's what you do and how/where you live. I had a friend whose mom died `young' from lung cancer. Her mom never smoked. My friend moved to warm climate. The question for me is of how to reduce such stress? Answer- my book is limited to science. For those with a serious interest, it will be of interest. For everyone else- probably boring. I mean- who really wants to know the future in advance!



As someone who underwent genetic testing for Huntington's disease, this research is extremely relevant to me. This topic is controversial in the HD community, especially in regards to the decision to have children. However, it is extremely important to open the communication channels because there are many unspoken implications resulting from the test. These channels must certainly be more open between the generations, who have contrasting views on the decision. We won't be able to improve the quality of life for those at-risk until the community itself can openly talk about it to each other and the general public.


Take this one step further... if you could get enough clairvoyance to find out you will live until you are 90 years old, but you will absolutely not live a day a longer, would you take that deal to live until 90. I initially thought I would say yes without flinching. I've given it a great amount of thought... and I think the rest of my life would become a literal countdown and I would therefore prefer to take my chances and just see how long I may live. Im curious to know what others would do

Geoff Clausen

I took part in the prototype study in 1986 over 9 months with the result in return for my time and belonging to a family that has suffered greatly to Huntington's Disease. My time at John Hopkins in Baltimore were well worth the reality.
I was so afraid of the answer that I waited 3 years for the results. My reasoning was based on fact , not fiction. If I had important information offered to me would I accept the truth or would I live in wonder. Fact was the lever was then I could plan in real time based on real information and plan my life thus. I decided that to know was to defeat denial and the unknown. There was no other option and I needed all the info. I could take.
I understand why some choose the option to remain in the dark , I believe one of the Wexler sisters or maybe both choose to not take the test they helped create because it would not change things and why get bad news if you can pick no news. One of this sisters said " I would not be any happier in life if I got the good news but I would be devastated should the outcome be bad news" . To each there own as there is no answer that fits all mindsets .
As for the % of is the test valid, I was told 99.something is the answer. It has not changed my life as I am still a caregiver and I live the demon everyday. As for the countdown theory I would not invest in it as it pays no dividends. Life lasts until it ends due to dynamics way beyond your control. You can make better choices with information. Look both ways before stepping off the curb.
May serenity be your friend, Geoff



Coincidentally enough, just a few days ago I re-read this Robert Heinlein story:


My answer to the question? I'd get the test, you betcha. Maybe it's because I'd expect pretty good news; both my parents are alive & in good health for their ages (74 & 81), all my grandparents lived to ripe old ages (70+ to 90+). The only downside is that based on their experiences, I'll die of some form of cancer. (Although I tell the joke; when I go, I want it to be peacefully in my sleep, like my grandfather. Not panicked & screaming, like the passengers in his car...)


Though I believe it is important to have open communication and honest debates about these topics, I do not believe it is fair to ask those who are not at risk whether or not they would take the test. I do not want to belittle or undermine their opinions, it is just similar to how a person cannot know what it is like to lose a parent or a child when they haven't. Being at risk for HD myself, there is no way for me to truly impress upon anyone how much turmoil is brought about by thinking about the test, nor the vast amount of complexity that the very nature of this incurable disease adds to one's life.


It seems to me that this is an ethical issue and not an economic one.

It's difficult to understand why someone would choose to have a child with a 50% chance of such a horrible disease. Are there unfair rules prohibiting adoption to couples with one HD+ parent? It is understandable why HD has persisted in the population as it affects people after reproductive age, at least prior to the mechanism of transmission being understood. Though the researcher talks about the test as a tool on the way to a cure, it seems to me that in a ethical population the test IS the cure.

Lots of talking about how having the information makes people feeeeeeel... No question it's depressing...but isn't? it also navelgazing? How about how your potential son or daughter will feel at 55? Obviously these comments do not apply to non-parents, but a 5% rate of testing? Really?


I was very surprised and disappointed that your story didn't refer back to a very recent episode that would have completely illuminated the subject and reinforced the power of the Wexler quote that ends the piece above. This discussion reminded me of the response to the start of the AIDS crisis in the 1980s. After several years of confusion and lack of information, HIV was identified as the cause and soon after that a test was developed for the virus. For a few years after that, there was no treatment available to fight the virus. There was a very vigorous debate in the gay community about the value of getting the test and most people I knew chose not to do it primarily because there was nothing to do with the information other than wait and worry. Safer sex practices were already being encouraged and whether you knew you were positive or not you knew what you needed to do to protect yourself from possible infection or from infecting your partner if you were positive. As soon as AZT, the first effective treatment for the virus was developed, everyone I knew went out and got tested. I think the critical issue is always going to be whether having the knowledge will allow you to do something to address the issue. Having the knowledge for its own sake, especially when it can be so devastating, really has no value and, in fact, can be nothing more than debilitating. But having knowledge that can inform your actions and allow you to take steps to do something positive to avoid an otherwise terrible result will always drive us to action. I'm sure you could have found many other examples of diseases for which there were tests before there were treatments and then treatments came along and you would find the same result.


Greg Stearns

This issue is Prospect Theory (http://en.wikipedia.org/wiki/Prospect_theory) at work. Specifically, the Four-Fold Pattern's top right quadrant. 50% is drastically higher than 1in 10,000 (the base population rate) and the potential loss is enormous when both physical and cognitive degeneration are at stake.

I have a 50/50 risk for Spondylitis, which isn't anywhere near as debilitating as Huntington's and I haven't been tested (though at 31 without symptoms, I'm likely to be in the clear).


If I was at risk, I think I'd take the test. Because in my mind the potential positives out way the potential negatives.

The podcast did not talk about what happens when people are tested negative, (will not get the disease). Getting this information would be amazing, because instead of living with uncertainty, you could live as preparing for a long life. There would be no questions about having a career, getting married, having children. All of which would still be in question had the test not been taken.

While the cost is high, the benefits, in my opinion, outweigh them.

Ben Saunders

Great episode! I have to admit, I was irritated listening to Wexler; the argument "you'll never understand because you don't have HD" just doesn't fly with me. She needs to leave her emotions at the door; she's a scientist, and the data collected on individuals like her and her sister are invaluable.

One thing that shocked me (obviously, I haven't thought a lot about HD until now) is that some of these people knowingly have children, despite the incredible risk they're taking. Dubner, I'm surprised you didn't show more outrage... this seems clearly unethical. I have a son; I can't imagine exposing him to a 50% chance of Huntington's. It's selfish, plain and simple. I googled "why do people with huntington's have children?", and this is an excerpt from the top hit: "Deciding whether or not to have children who might be at risk of inheriting Huntington's disease is a dilemma that still faces prospective parents. Doing things the 'old-fashioned way' remains an option, and is of course free of charge and fun!" Jesus! How is it a dilemma? Who cares if it's free or fun? Exposing your child to a 50% risk of HD isn't a dilemma, it's just a terrible choice.

I know I'm going to take a lot of flak for this, but is there some way we can incentivize these individuals not to have children "the old fashioned way"? I'm not even sure if this is legal, but can we (the government) pay for in vitro fertilization and any costs it may incur? I hate to sound like a eugenics monster, but I feel there's a tremendous advantage to "breeding out" this gene...


Andrew Cockerham

One word: hope. Thats what this whole thing is about. The reason this 'uncertainty' is valuable, is because it gives us hope. If we knew the dreadful news, we would lose all hope. And no one can live without hope. Hope requires uncertainty. Or put another way, certainty destroys hope

Jan T Oakfield

As an HD family member who has been a caregiver for the past 30 years, and who tested negative for the gene mutation many years ago while family-planning, I'd like to add a few things to the discussion:
1. While there currently is no cure for HD, there are HD doctors (primarily neurologists and psychiatrists) who are very adept at managing HD and caring for HD patients. They individually, and collaboratively, care for HD families (the patients, the caregivers, the gene-positive but asymptomatic, at-risk (non-tested) adults, and children living w/ HD family members), research the disease, and share information w/ each other and the HD community. There are HD clinics that provide medical care and services including social work, psychotherapy, genetic counseling, speech therapy, OT, PT, nutritional advice and opportunities to participate in research. Short of a cure for HD, medical management of HD, and those who provide this care, are extraordinary. With good medical care, and support from family and friends, quality of life can be achieved by HD patients, depending on how they and their support network deal with HD.
2. HD research continues to advance and be pursued rigorously. We know more and more each day. Unfortunately, brain science is complex, it takes time, for HD and most other diseases.
3. While the laws against genetic discrimination have yet to be tested, they do exist and are there to protect us.
4. I've always believed that a cure is not far off (I'm an optimist, as are my family members affected by HD). Therefore, bringing children into the world with a 50% risk of inheriting the genetic mutation for HD didn't scare me. We would still have a generation to find a cure. What scared me was being a mother who gets sick while raising children...how could I care for my school-age children if I was sick?
5. Information, communication and certainty is better than uncertainty and denial.

Individual decisions to get tested or not are most influenced by our personal experiences. Public figures have a responsibility to maintain objectivity when speaking about such intimate topics as genetic testing, and should not allow their personal experiences to dissuade at-risk people from being tested, especially if one is the heroine of the HD community. Perhaps this is why so few people have been tested? The radio host and the economist should also hear from those who live optimistically with the knowledge that they will, or have, HD.


Steve C

Here's a thought. Let people take the test, then flip a coin to see whether you throw the results, unread, into the bin. If you don't discard the results, you only pass on the results if it is good news.

In this way, one in four people at risk get to hear they are safe, and can stop worrying. These people would be forever free. Three in four never hear one way or the other, but they now know their chances of having HD have risen from 1/2 to 2/3.

I wonder if this 50-50 chance (info or no info) would have a similar psychological effect to the one described in the show. People know the odds of having the disease but seem to live optimistically until shown proof -- the failed test results. But if you promised to never show the negative results, and still provided enough uncertainty for people to imagine themselves free of the disease if they didn't hear, would that psychological effect still function to allow people to continue to live optimistically?



When my sister-in-law's grandfather mysteriously came down with the disease, they deduced that he was not his father's child. Because she was an old woman, the family did not confront his mother.

We wonder about who the real father was. Three of his four grandchildren died of Huntington's, one of their children got tested (negative) the other did not.

Greg Rozansky

If you have HD you have an obligation to get tested. I worked in a pre-implantation genetic diagnostic (PGD) laboratory. Symptoms of Huntington’s disease may only present later in life, often well after carriers procreate. If an individual has a family history of Huntington’s they must get tested so that they can have children through PGD which would then ensure that their children would not acquire the mutation. This is a cure that would one-day completely eradicate Huntington’s. Dr. Wexler’s discovery took place long before PGD was being used and frankly, the ‘should one get tested’ question is quite old-fashioned. In countries like Canada and Israel, governments are fully subsidizing PGD as they recognize this as a means of eradicating genetic diseases that have been sequenced.

Shutish Patel

Greg Rozansky's comment merits consideration by public health policy makers. There should be no difference in our approach to HD as compared to any other fatal genetic disorder such as Tay-Sachs disease. If PGD is a viable approach, it should be offered as a necessary prenatal screening test for at-risk individuals. At this time it would appear to be the only absolute way to eliminate the disorder.


This debate makes me think of the book "Never Let Me Go" written by Kazuo Ishiguro, a science fiction dystopia about the world in which clones are bred only to be used as organ donors around the age of 30. Although the main characters, all clones, know and comply with this fate, none of that matters too much. What truly matters to them is the relationships their form, friendships and romantic love, which make their life worth while.

In a way, most of us will end up having rather predictable lives: we'll work, have families, be more or less happy and die around the life expectancy age for our population. The clones in Ishiguro's book would die around 30, I am (hopefully) likely to die around 82, HD patients around 60. The odds of a longer lifespan might be different, but our priorites and aspirations are probably quite similar. It is what we make out of the time we have that matters.




I want you to start posting what music you are using in each podcast. You have such great selections, especially in this podcast, and you don't leave enough non-voiced-over sections for me to shazam them :)