A Life Without Fear: Dealing With Williams Syndrome Isabelle, 9, was born with a rare genetic disorder that, among other things, means she is biologically incapable of not trusting people. Her pathological trust of everyone has made for some serious challenges in raising her, but Isabelle's parents say there are also rewards to a child with boundless love and trust.
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A Life Without Fear

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A Life Without Fear

A Life Without Fear

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It's MORNING EDITION, from NPR News. I'm Steve Inskeep.


And I'm Renee Montagne.

Today in "Your Health," we have the story we have a story of a mother raising a daughter with a rare disability. It causes her to trust virtually everyone she encounters. It's caused by a genetic disorder that makes it nearly impossible to feel distrustful. NPR's Alix Spiegel has our report. And we'd like to note that for privacy reasons, we're not using the family's last name.

ALIX SPIEGEL: The day before I met her, Jessica had gone to pick up her daughter Isabelle at her drama class. It was the usual scene, parents and kids hanging around, chattering. Then, out of the corner of her eye, Jessica noticed Isabelle carefully making her way towards an elderly woman that Jessica had never seen.

JESSICA: Isabelle sidled over to this very nice, kind of grandmotherly-looking lady and said, you know, hi, I'm Isabelle. And they started talking. And then she said, will you take me? Can I go home with you?

SPIEGEL: Isabelle, Jessica's 9-year-old, has Williams syndrome. Williams is a genetic disorder with a number of symptoms. The children are often physically small; there are developmental delays. But also, kids and adults with Williams love people and are pathologically trusting. They have no social fear.

Researchers theorize that this is probably because of a problem with their limbic system, the part of the brain that regulates emotion. Somehow, one of the chemicals that signals when to trust and distrust has been disrupted. This means that it is essentially biologically impossible for kids like Isabelle to distrust - distrust, Jessica says, anyone.

JESSICA: They don't have that kind of evolutionary thing that other kids do, like that - just little twinge of anxiety like, who is this person? What should I do here? Am I going to be OK? They just don't have it. And so she doesn't have that like, biological warning system.

SPIEGEL: And for Jessica, Isabelle's mother, there are good things and bad things about parenting a kid like this. For instance, when Isabelle was little, she was chronically happy. She smiled at anything. She loved everyone: family, friends, strangers.

JESSICA: All it took for her is like, the barest beginning of a grin, and then she'd reach out for them to hold her. And you know, the people who didn't know her - like at the grocery store - just thought, oh, she's so adorable.

SPIEGEL: And in those days, Jessica says, she and her family were basically tolerant of Isabelle's trusting and loving nature.

JESSICA: We would try to restrain her, but it was somewhat half-heartedly, because I think we were - we didn't want to embarrass her by kind of calling her on the carpet about how open she was.

SPIEGEL: But as Isabelle got older, the negatives of her trusting nature began to play a larger role. For example, a couple years ago, Jessica and her family were spending the day at the beach.

JESSICA: She had been begging me to go to Dairy Queen, and I said maybe later. We'll see. We'll see. Well, she had overheard a lady just down the beach telling her kids, OK, let's go to Dairy Queen. And Isabelle went over and got in the lady's van, got in the backseat, buckled up, and was waiting to be taken to Dairy Queen with that family.

SPIEGEL: Jessica and her kids were frantically searching for Isabelle when the driver approached her and explained that she'd been starting her car when she looked up and saw Isabelle's face in the mirror. The woman, Jessica says, was incredibly angry.

JESSICA: She said, I am a stranger, you know.

JESSICA: And I was thinking, well, yeah, lady. Like, what do you think? You know, I'm an adult. I understand these things. You know, so you get people kind of blaming you for like, don't you understand the risks? And it's like, my friend, you have no idea.

SPIEGEL: In fact, because of Isabelle, Jessica has had to rethink even the most basic elements of her day-to-day life.

JESSICA: I don't take her to the dog walk every day with my other kids. I try not to go to the store with her as much as possible. I give her the script about what we're supposed to do beforehand. We're always looking after her because she's like a time bomb waiting to blow her joy over whoever she sees.

SPIEGEL: And it's not Jessica who must be vigilant. Every teacher at Isabelle's school has been warned. Isabelle is not allowed to tell them that she loves them. Isabelle is not supposed to tell other school children that she loves them. And there are other restrictions.

JESSICA: She's not allowed to go to the bathroom alone in her school, because there have been numerous instances of girls with Williams syndrome being molested at school when they were, you know, alone in the hallway. And these are, you know, like middle-class-type schools. So it's a very real problem. And you know, I would rather her be overly safe than be on CNN.

SPIEGEL: After I talked to Jessica for a while, I turned off my recorder, and we waited for the kids to come home from school. Then, all of a sudden, I felt two little arms circle my neck from behind. Isabelle had crept in and was giving me a hug.

ISABELLE: So how are you?

SPIEGEL: I'm doing all right. How are you doing today?



ISABELLE: So what are you doing here?

SPIEGEL: I - well, I came to visit you. Do you want to sit down?


SPIEGEL: We sat and Isabelle asked if she could hold my microphone. Then she decided to sing me a song.

ISABELLE: (Singing) You're my friend. You're my friend in the whole world. You look so nice and so beautiful and so sweet.

SPIEGEL: Isabelle speaks with a slight nasal slur and has some cognitive issues. Though she goes to a regular school, her attention is jumpy, and she needs aides to help her. These cognitive issues make Jessica's job more difficult. You see, Jessica has decided that the most important thing for her to do is teach Isabelle how to distrust.

That is her life project, a pitched battle against biology itself. And so Jessica and her husband have made handmade books about how to behave around strangers, rented videos. And then there are the role-playing exercises. Jessica tells Isabelle that they're going to do one of their exercises to show me that Isabelle should pretend Jessica is a stranger.

JESSICA: Hello, little girl. You want to see my puppy?


JESSICA: Oh, my puppy's so cute. Are you sure?


JESSICA: Oh, come over here. I've got some candy, too.


JESSICA: Come on. Come into my car, and I'll show you.


JESSICA: Isabelle, are you supposed to go into someone's car?

SPIEGEL: And this, Jessica says, is how it goes. Sometimes, she remembers and doesn't tell strangers that she loves them; mostly, she does. But Jessica is determined.

JESSICA: It's like we just have to restart every time. That's just what we have to do.

SPIEGEL: Have to do, Jessica reasons, because she won't be around forever to protect her daughter. And though Isabelle trusts the world completely, the world is not a place worthy of complete trust. Even in their current life, Jessica says, there are moments that she realizes that she is just a turned head away from something terrible.

JESSICA: We live a very sheltered life, but I can think of times when, you know, we were at the pool and I turned around to talk to someone, and I see her practically sitting on some man's lap, you know, at the pool - who looks very uncomfortable. And I just think like, this is not good.

SPIEGEL: So Jessica tries, and she says Isabelle is slowly learning. For instance, late in the day, she came up to Jessica after she heard us talking again about the time she was at the beach and got into the van with a stranger.

ISABELLE: Are you mad at me, Mom?

JESSICA: I forgive you, honey. You just try not to do it again.

SPIEGEL: And to be fair, there are plenty of rewards to this life. And Jessica is fully aware of them.

JESSICA: She'll ask me, so how are you today, my darling? And it just - it just makes you smile.

SPIEGEL: Late in the afternoon on the day that I visited, everyone in the family gathered in the kitchen to eat dinner. Isabelle loves music, so she decided to play a CD.

(Soundbite of music)

SPIEGEL: The CD player stuttered, then came to life, and Isabelle turned to her father.

Would you dance with me, my sweetie? she asked. Her father picked her up in his arms. He spun her round and round.

(Soundbite of music)

Alix Spiegel, NPR News, Washington.

ISABELLE: Goodbye.

(Soundbite of music)

MONTAGNE: You're listening to MORNING EDITION from NPR News.

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