Two sisters, one horrible disease

From the archives, two sisters make a hard choice: Do they take a genetic test to learn whether they'll get the disease that tortured their mother?

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The following script is of "The Hardest Choice," which aired on October 26, 1986. Diane Sawyer is the correspondent.

As you watch this next story, suppose for a moment that you were facing the choice these two sisters are facing. Someday soon, you may be. Scientists say it won't be too many years before tests will be available that can tell each of us which diseases lie in our future. If the disease that awaited you was fatal and you couldn't do anything about it, would you still want to know? That's the question these two sisters are facing now. There's already a test that can tell them whether they are carrying the same crippling genetic disease that killed their mother, a disease that often strikes in middle age. It's their choice to learn the facts, whether good news or bad, or continue living with uncertainty. What they already know, as does their father, is that the bad news is truly horrible.

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Milton Wexler: It's my opinion it's the most terrifying disease on earth. Nothing can match it.

Diane Sawyer: Why?

Milton Wexler: Because it means the decay of the body, the loss of control of the body and absolute certain dementia. It means that you're going to lose your identity, your sense of self, your feeling about yourself, your memories, your thoughts. It's a total disaster. It's like cancer and Alzheimer's put together.

This is the disease that Milton Wexler is talking about: It is Huntington's disease, an incurable genetic disorder. Its victims do a tortured, involuntary dance and die a slow, agonizing death.

Dr. Wexler is a prominent Los Angeles psychoanalyst. He himself is not at risk for the disease, but he knows about it because his wife died of Huntington's. What he doesn't know, what no one knows, is whether Huntington's will also claim his daughters. Nancy Wexler is a New York psychologist; her sister Alice, a writer and university lecturer in California. They are perfectly healthy now, but they each have a 50% chance that they inherited the Huntington's gene from their mother. Nancy and Alice also know how horrible Huntington's is. They watched their mother suffer for more than a decade.

Nancy Wexler: She tried to commit suicide at one point, made a very serious suicide attempt. I think she knew what was happening. She knew what she had. She knew what was in store. And at the very end, she slept in a hospital bed that we had to pad with lamb's wool because she was moving so much, she would get all black and blue from just thrashing in the bed. And when she finally died, she looked like she was someone from Dachau when she died. But she was also still when she died. And in a funny way, it was like the old Mother had come back because she was still and she wasn't moving anymore. And she wasn't--she wasn't desperate anymore.

Every day of their lives, the Wexler sisters think about Huntington's, a disease that arrives in such innocent ways--a little clumsiness here, a little forgetfulness there.

Alice Wexler: Sometimes if I lose something, like I lost my keys yesterday, or if I trip, the first thing automatically that comes into my mind is Huntington's, and then it goes away.

You know, and then- then, it's-- I don't dwell on it. I'm not obsessed with it. But if anything goes wrong, that thought will just flicker through my mind.

Nancy Wexler: The other day I had a bunch of apples, and I was taking them out of the car, and I said, "Now, these apples are washed. I'm not going to drop them." They were completely in the gutter the next time I turned around. And I think, "Is this what it would be like if I had Huntington's?" But then, there are also little tests when you do things right, like when you walk along the edge of a curb and you don't fall off. Or when you're- I was trying to learn how to play tennis, and when I-- when I get it over the net, I think, "I don't have Huntington's."

For Nancy and Alice Wexler, 20 years of wondering if they were carrying a genetic time bomb. And now, a choice that means they don't have to wonder anymore. Science has handed them a window onto the future: a test they can take which would tell them with near certainty whether they have the Huntington's gene.

Diane Sawyer: Did you think you'd get the test if they ever had one?

Nancy Wexler: I was positive I would, un- unquestionably. It never occurred to me that I wouldn't.

Diane Sawyer: And all of a sudden when you had the option you weren't so sure.

Nancy Wexler: I wasn't so sure, exactly. I even said to people, "You know, when the test is actually here, a lot of people are going to change their mind." It never occurred to me I would be one of them. I mean, that wasn't in the books.

Alice Wexler: I would like to know that I don't have it, but I absolutely do not want to know if I do have it.

Nancy Wexler: This is absolutely the hardest choice I've ever made in my life.

The irony is it's a choice that Nancy Wexler brought upon herself when she set out to find a cure for Huntington's disease. Several years ago, she became president of the research organization founded by her father, the Hereditary Disease Foundation, based in California. She decided not to sit back and wait for Huntington's--that she would go out and fight it. The search for the cause of the disease took her to a little village in Venezuela called San Luis.

Nancy Wexler: There's never been such an intense concentration of Huntington's disease in one place anywhere else in the world. Practically every other house, there's a patient with the disease, several patients with the disease, people who are at risk for the disease. This entire community is just suffused with this illness.

Huntington's is the disease that killed the folk singer Woody Guthrie. In the United States, 25,000 people have it. Another 125,000--Nancy and Alice Wexler among them--are considered at risk because it's in their families. The disease is a rare one throughout the world. But in the village of San Luis, where the families are interlinked and inbred, the disease has become a familiar part of growing older. Here, they call it "el mal de San Vito"--the Saint Vitus Dance.

Nancy Wexler: I feel myself very much one of the people here. Every time that I see somebody new getting the illness, it's like somebody in your own family is coming down with it. You just keep seeing this gene which you can't control and you can't stop, and it's implacable, you just keep seeing it roll over person after person after person, and slam them down. So, I mean, the suicide rate even here, the suicide rate is high. People make attempts. They drink gasoline. They, one man tried to cut his throat with a knife several times.

Nancy Wexler: Certainly when I found out I was at risk, I thought about suicide. I thought, "Would I want to live with this disease? Could I go to restaurants and choke? Would I want to impose this on my friends?" And I think that for some of my friends who have Huntington's disease, knowing that they can commit suicide gives them a certain sense of control that they want to feel, that if it gets too bad they can have a way, a way out. They can do something.

From the start of her expeditions to Venezuela, Nancy Wexler knew she was racing against time. To find the cure, you had to find the gene: the medical equivalent of a needle in a haystack. She joined forces for the search with Jim Gusella, a molecular geneticist at Massachusetts General Hospital. At his laboratory in Boston, Gusella had the technology. What he lacked was the people--enough Huntington's families to track the gene through generations. And that's where the people of San Luis came in. Nancy and her team began a marathon process of taking blood, tracing family histories, and sending the disconnected pieces of the genetic puzzle back to Jim Gusella.

Nancy Wexler: It unquestionably opened the door. It truly--- it cracked the story of Huntington's disease.

In 1983, Nancy Wexler and the people of San Luis, Venezuela helped make scientific history. That was the year that Jim Gusella, studying the blood of the Venezuelans, was able to zero in, not on the Huntington's gene itself, but on a marker close to the gene. It was this discovery that paved the way for a

Huntington's test.

Diane Sawyer: What did it mean to discover the marker?

Jim Gusella: What it meant for people who have Huntington's disease is that there was now the chance for some kind of pre-symptomatic or prenatal diagnosis in the disease, and also for them the hope that when the gene is isolated maybe there'll be some treatment or a cure.

And so it was that Nancy Wexler, through her work in Venezuela, brought upon herself, her sister and everyone else who is at risk for Huntington's disease the hardest choice of their lives: Do they take the test and learn the future?

Nancy Wexler: It's almost as if you're watching someone outside of yourself. You're saying, "Well, I wonder what she's going to decide to do," you know. Except that it's me, and I'm going to have to make a decision.

Diane Sawyer: Is there a right decision? Is there a right choice?

Nancy Wexler: I think for each person that there's a right choice, but I think the hard thing is trying to find out what it is for yourself.

Dr. Wexler, who as a psychoanalyst makes a living helping people face the truth, says he doesn't want his daughters to take the test.

Milton Wexler: Well, if you look at these two young ladies, and see how they are now, I know they're at risk, but as far as I'm concerned, clinically, I think they're quite safe.

Diane Sawyer: But are you just wearing blinders? Are you just unwilling to face what you-- the reality that you might have to face? Is it a kind of self-deception?

Milton Wexler: Don't think so. I think it's just measuring risk and reward and saying, if they have a 50-50 risk, and they both take the test, why would I want to--to have that risk-reward kind of ratio where one would come up with a bad answer and all three of us are destroyed by it?

Nancy Wexler: If I found out that I was free and that Alice had the disease, I'd die. I mean, I would-- that would be as bad as me having it.

Diane Sawyer: So, you see yourselves as in this together?

Alice Wexler: Absolutely.

Nancy Wexler: Either both of us are free, or both of us aren't going to be free.

Diane Sawyer: But what is going to make you decide? You've had all the same factors present up till now, and you can't decide. What's going to make you decide?

Alice Wexler: I think one thing that Nancy has spoken about and we've spoken about is trying to imagine the various outcomes, and imagine what it would be like to get bad news, to imagine the unimaginable, to think about the unthinkable, for us. And I don't think I've ever really done that. Now, as I-- as I start to do that, and try to really imagine, well, really, how would I feel sitting down at my desk, in front of the computer, knowing that I have this gene? Would I-- would I go on? And sometimes, some days, I think, "Well, no, I couldn't do that. I really couldn't do that."

Diane Sawyer: Is it a blessing or a curse, though, this kind of knowledge?

Jim Gusella: It's knowledge. I mean, it's-- it's a matter of whether you believe that ignorance is bliss or whether you believe that knowledge leads you to the potential for solving problems.

Alice Wexler: If' you have a certainty that you have the gene, and you have the certainty that you're going to die this absolutely miserable death over many years, I'm not sure it's possible to keep on living, to use that knowledge constructively. I--I'm sure I wouldn't use it constructively. I think I would be devastated by it.

Diane Sawyer: So, some hope is better than possible bad news?

Alice Wexler: That's right. Hope is better than despair.

Milton Wexler: Whom the gods would make mad, they first give some kind of insight into the future. And I don't think always we want to have that-- that kind of power to look into the future. And I think it's a potential for madness. I don't want to know when I'm going to die.

Diane Sawyer: What is your fantasy about a cure? What is it-- what's your dream of what you will do on the day you get that cure?

Nancy Wexler: I think that the day that there's a-- there's a real cure, the day that there's a treatment, I think, first of all, I would get a huge jumbo jet and I'd go down to Venezuela.

Diane Sawyer: Do you dream of their faces?

Nancy Wexler: Oh, yeah! Oh, yeah! You know, once you're part of a family with Huntington's that, you know, we share blood with people all around the world, and that you can't be immune to them. It's- you're not a private family anymore. I think that what would be just an extraordinary feeling would be to see these faces, to say to them, you know, "This is the cure. This is the magic." You know. "Open up, and take this pill, and you will never have to worry about this disease." And I imagine just this incredible joyousness, and everybody hugging each other, and everybody crying for joy. To be able to stop something like this in its tracks is just the most staggeringly-- ecstasy. I can't imagine anything in the whole world that would be better.

Although Nancy and Alice Wexler are still undecided about learning their fate, some others at risk for Huntington's have made the decision to take the test. Of the three people known to have their test results, two learned that they will get the disease.