Williams+syndrome

=A Genetic Drive To Love, Yet Distanced By Differences= by [|Alix Spiegel] [|www.npr.org] May 3, 2010

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[|Morning Edition] [4 min 9 sec] Enlarge iStockphoto.com Though people with Williams Syndrome are known for being hyper-social, the disorder has many other facets, some of which are incredibly isolating. May 3, 2010 People with Williams syndrome — a rare genetic disorder with a variety of symptoms — are known for being almost compulsively loving and trusting. The syndrome is often called the "anti-autism" because people with Williams, rather than isolating themselves from others, are hyper-social. Children with Williams, for example, often climb into cars with perfect strangers or tell random salespeople that they love them. But this hyper-social quality is not the only thing that characterizes Williams disorder, there are a wide variety of much less-publicized symptoms that affect their interactions with people and handling of daily tasks. And, one of the most unfortunate aspects is that even as people with Williams thrive on emotional closeness, other symptoms of the syndrome make it really tough for them to develop relationships. Karen Levine is a psychologist based in Massachusetts who specializes in the treatment of Williams syndrome. Several years ago Levine was attending a conference on Williams that was open to both researchers and people who suffered from the disorder, and one of the activities on offer was a boat tour of the city of San Diego. Levine, her colleague Sarah, and about 20 adults with Williams crowded onto a small sturdy boat and set off. "We were out in the harbor taking our little boat tour ride and everybody was having a good time," Levine says. "Then all of a sudden everybody, except Sarah and I, looked at each other and gasped!" While the boat-goers with Williams looked terrified, and held out their arms to comfort each other, Levine frantically scanned the boat. There was nothing. Then she scanned the water but again found nothing. The problem, it turned out, was a sound, a sound only people with Williams would be aware of. "Turns out, way off in the distance on the shore there was a siren — an ambulance or fire engine or something," Levine says. And because peoplewith Williams have something called hyperacusis, which makes them extremely sensitive to noise at certain frequencies, they heard that background noise as if it were directly in front of them. Hyperacusis is a somewhat challenging aspect of Williams syndrome, but some symptoms can be viewed as strengths. On the good side, most people with Williams have an extraordinary memory for faces, are extremely verbal and sensitive to music, and are often incredibly empathic. Consider this story from a Williams specialist named Barbara Pober, a doctor based out of Mass General in Boston. Pober says that several years ago she went to work at her clinic at the hospital. "It was a typical clinic day and a patient walked in. He was someone I had seen before, and he immediately asked me, ' What's wrong Dr. Pober, you look sad,' " Pober says. In fact just that morning Pober had gotten some terrible news that she'd resolved to hide from her office. And she'd been successful. All day long none of her colleagues, none of the people she met, had any clue. Nobody until her patient with Williams. "It's part of the complexity of that is Williams syndrome," Pober says. "Patients are very in tune, very empathic, very sensitive to the emotions of others." Williams is uncommon, only about 1 in 10,000 people have it and it doesn't run in families though people with Williams will likely give birth to a child with Williams. It's caused by a deletion of around 26 genes on a single chromosome. This alteration produces real strengths but also serious medical, emotional and cognitive problems. Pober gives another example, the story of a very verbal and engaged teenage girl who struggled with simple, daily tasks. "I remember it with great clarity ... the girl bounded in and said, 'I've just seen a really nice reproduction of the Mona Lisa at the Museum of Fine Art,' " Pober says. "Yet this very same person couldn't navigate from the museum to the hospital, which is a straight shot with one turn." The problem is that people with Williams are bad at spatial relationships, and most have to carry a calculator because they can't make simple change. It's also hard from them to organize and sequence or plan activities, and more importantly, they have intense trouble concentrating. Their attention jumps around continuously, it's sometimes hard for them to maintain focus for more than a few minutes. This makes it difficult not only to hold a job as an adult, but also, Pober says, to fulfill one of their most intensely felt needs: because they are compulsively social they yearn to be surrounded by dear friends. "To me it's one of the heartbreaks, the sadnesses of Williams syndrome," Pober says. "Most are rather socially isolated." Pober says this is because in order to get to know someone you have to do turn taking in dialogue. "You listen to what I say, I listen to what you say, and then we build on that," Pober says. "But to sustain the attention and build on the dialogue enough to really get to know someone is hard for many folks with Williams syndrome." Pober says few people with William's syndrome marry, and even fewer have children. But this may be changing, Pober says. Now there are new social training programs for people with Williams. These new treatments might ultimately transform the long term prospects of people with Williams.
 * A Great Sense Of Empathy**
 * Struggling With Day-To-Day Tasks**
 * The Paradox That Isolates**

=A Life Without Fear= by [|Alix Spiegel] [|www.npr.org] April 26, 2010

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[|Morning Edition] [8 min 33 sec]  Jesse Neider for NPR text size A A A April 26, 2010 The drama class had just gotten out, and everybody was standing around talking when Jessica noticed her 9-year-old, Isabelle, making her way over to an elderly woman Jessica had never seen. The woman was neatly dressed, most likely just a well-meaning suburban grandmother who had come to retrieve a grandchild on behalf of an over-extended parent, most likely a perfectly harmless person. Isabelle, as she usually did, exchanged hellos and struck up a conversation. It was the usual post-drama-class conversation until about two minutes in. Then Isabelle dropped the bomb. "Will you take me? Can I go home with you?" Jessica heard Isabelle plead. Jessica's daughter, Isabelle, has Williams syndrome, a genetic disorder with a number of symptoms. Children with Williams are often physically small and frequently have developmental delays. But also, kids and adults with Williams love people, and they are literally pathologically trusting. They have no social fear. Researchers theorize that this is probably because of a problem in their limbic system, the part of the brain that regulates emotion. There appears to be a disregulation in one of the chemicals (oxytocin) that signals when to trust and when to distrust. This means that it is essentially biologically impossible for kids like Isabelle to distrust. (NPR is not using full names in this story for privacy and safety reasons.) "They don't have that kind of evolutionary thing that other kids have, that little twinge of anxiety like, 'Who is this person? What should I do here?' " Jessica explains. "They just don't have it. She just doesn't have that ... early-warning system." For Jessica, there are good and bad things about parenting a child with this kind of personality. For instance, when Isabelle was younger, she was chronically happy. She smiled at anything. She loved everyone: family, friends, strangers. She reached for them all, and, in return, everyone loved her. Strangers would stop Jessica to tell about how adorably loving Isabelle was. In those days, Jessica says, she and her family were more or less tolerant of Isabelle's trusting and loving nature. "We would try to restrain her, but it was somewhat half-heartedly, because we didn't want to embarrass her by calling her on the carpet about how open she was," Jessica says. But as Isabelle got older, the negative side of her trusting nature began to play a larger role. A typical example happened a couple of years ago, when Jessica and her family were spending the day at the beach. Isabelle had been begging Jessica to go to Dairy Queen, and Jessica had been putting her off. Then Isabelle overheard a lady just down the beach. Enlarge Jesse Neider for NPR Isabelle practices training the family dog, "Betsy," with her dad. "She was telling her kids, 'OK, let's go to the Dairy Queen,' " Jessica says. "And so Isabelle went over and got into the lady's van, got in the back seat, buckled up and was waiting to be taken to Dairy Queen with that family." Jessica had no idea what had happened to Isabelle and was frantically searching for her when the driver of the van approached her and explained that she had been starting her car when she looked up and saw Isabelle's face in the rearview mirror. The woman, Jessica says, was incredibly angry. "She said, 'I am a stranger, you know!' " Jessica says. Essentially, the woman blamed Jessica for not keeping closer watch on her daughter — for neglecting to teach her the importance of not getting into a car with someone she didn’t know. But the reality could not be more different. "It's like, 'My friend, you have no idea,' " Jessica says. In fact, because of Isabelle, Jessica has had to rethink even the most basic elements of her day-to-day life. She can not take Isabelle to the dog park. She tries not to take Isabelle to the store. And when the doorbell rings, Jessica will leap over a coffee table to intercept her. It's not just Jessica and her family who must be vigilant. Every teacher at Isabelle's public school has been warned. Isabelle is not allowed to tell them that she loves them. Isabelle is not supposed to tell other schoolchildren that she loves them. And there are other restrictions.
 * Driven To Trust**
 * The Danger Of Unconditional Trust**

"She's not allowed to go to the bathroom alone at her school, because there have been numerous instances of girls with Williams syndrome being molested at school when they were alone in the hallway," Jessica says. "And these are like middle class type schools. So it's a very real problem. And, you know, I'd rather her be overly safe than be on CNN." Jessica spoke with me for over an hour in the family's home in their woodsy, suburban neighborhood while we waited for her three children to come home from school. Then, just after I turned off my recorder to take a break, I felt two small arms circle my neck from behind. It was Isabelle. She had crept in from school and was giving me a hug. I turned around, and quite suddenly, the room was filled with questions. Who was I? What was I doing here? Which TV show did I like? Did I know the Muppets? Then Isabelle took my microphone in her hands. She had decided to sing me a song: "You're my friend ... You're my friend in the whole world," she crooned. "You look so nice and so beautiful and so sweet." When Isabelle speaks, she has a slight nasal slur. She also has some cognitive issues. Though she goes to a regular school and sits in a regular third-grade class, her attention is very jumpy, and she needs aids to help her. These cognitive issues make Jessica's job more difficult. Jessica has decided that the most important thing for her to do is to teach Isabelle how to distrust. For years, that has been her life project — a battle pitched against biology itself. Jessica and her husband have made Isabelle books about how to behave around strangers. They have rented videos, they have bought educational toys. They have modeled the right behaviors, constructed sticker charts and employed every other trick they could possibly think of. But distrust, it seems, is almost impossible to teach their child. Sometimes Isabelle manages to remember not to tell perfect strangers that she loves them. Mostly, she doesn't. But Jessica is determined. "We just have to restart every time," she says. "It's just what we have to do." It's what they have to do, Jessica reasons, because she won't be around to protect her daughter forever. 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 when she realizes that she's just an instant away from something terrible. "We live a very sheltered life, but I can think of times when we were at the pool and I turn around to talk to someone, and I see her practically sitting on some man's lap at the pool, and he looks very uncomfortable," Jessica says. "And I just think: This is not good." Fortunately, Jessica says, the experts tell her it will eventually get better. She needs to just keep at it. One day, they tell her, Isabelle will be able to learn not to //feel// distrust, per se, but to master a set of algorithms that will allow her to safely navigate the world. She will learn, for example, not to get into a car with a stranger if she has become lost or disoriented, but to ask some person in a uniform for help instead. In the meantime, Jessica says there are plenty of rewards to this life — a life with a child with boundless love and trust. "She'll ask me, 'So how are you today, my darling?' " Jessica says. "And it just makes you smile." In fact, late in the afternoon on the day I visited, everyone in the family gathered in the kitchen to eat dinner. Isabelle, who loves music, decided to play a CD. The CD player stuttered then came to life, and Isabelle approached her father. "Will you dance with me, my sweetie?" she asked. Her father picked her up in his arms. He spun her round and round.
 * Raising A Child With Williams Syndrome**
 * Unconditional Love, And A Mother's Worry**

=When The 'Trust Hormone' Is Out Of Balance= by [|Alix Spiegel] [|www.npr.org] April 22, 2010

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[|All Things Considered] Jesse Neider / for NPR Isabelle, 9, has a rare genetic disorder called Williams syndrome. One of the symptoms is that people have an inability to distrust anyone, even strangers. Isabelle often picks at her hands as a means of relieving stress. text size A A A April 22, 2010 This is a story about a fickle little hormone that plays a large role in our lives. The name of the hormone is oxytocin, and until recently it was mostly dismissed by scientists. They knew it played a role in inducing labor and facilitating breast-feeding, but otherwise didn't give it much attention. But over the past 10 years, oxytocin has come up in the world, and several researchers have begun making big claims about it. Now dubbed "the trust hormone," oxytocin, researchers say, affects everything from our day-to-day life to how we feel about our government. The narrative of oxytocin — the trust hormone — is being rewritten. To understand the role that oxytocin plays in your own life, consider the experience of a small 9-year-old girl named Isabelle. (NPR is not using the full family name in this story for privacy reasons.) Isabelle lives with her mother, Jessica, in a leafy East Coast suburb. Often, Jessica says, she'll be out running an errand when she'll bump into a well-meaning parent whose child goes to the same school as her daughter. The parent, Jessica says, invariably has news to share about an experience with Isabelle, and the conversations always go the same way: "They'll say, 'Oh, I saw your daughter at school today. She's so cute, she always tells me, 'I love you' when I see her in the hallway,' " says Jessica. "And I'll just be grimacing thinking, 'There we go again.' " Enlarge Jesse Neider / for NPR Isabelle and her dad look for wildlife in the woods. Isabelle's disorder means that her parents, as well as teachers at school, have to constantly monitor her to make sure strangers aren't taking advantage of her. Isabelle, you see, says "I love you" to everyone: to parents at her school, to people from the neighborhood, to the salesman at Circuit City. "Oh, all the time," Jessica says. "To Isabelle, there are no strangers — only friends she's not yet met." The problem is that Isabelle has Williams syndrome, a rare genetic disorder with a number of symptoms. The children are often physically small and often have developmental delays. But also, kids and adults with Williams love people and are pathologically trusting: They literally have no social fear. Researchers theorize that this is probably because of a problem with the area in their brain that regulates the manufacture and release of oxytocin. Somehow, the system in which oxytocin operates has been disrupted in a way that makes it essentially biologically impossible for kids like Isabelle to distrust. And this presents a terrible dilemma for Jessica. Isabelle completely trusts the world, but of course, the world is not worthy of complete trust. And so several years ago, Jessica decided that, Williams be damned, she herself would teach Isabelle to distrust. There were other symptoms of the disorder that Jessica's perseverance had overcome: Though Williams kids often have severe cognitive problems, Isabelle had learned to read. But Jessica says no matter what she did, the trust her daughter offers perfect strangers could not be extinguished. "I just kept thinking if I can just find the right tools, if I can buy a toy, if I can buy her a video, if we can model the behavior and reward it with sticker charts," Jessica says. "But it didn't amount to so much. That willingness to open herself up — we can't get it to go away. " Isabelle's uncritical trust and love of people, Jessica has concluded, is an irreversible, biological fact. From the perspective of Paul Zak, a researcher at Claremont Graduate University, Jessica's problem is not surprising. "I've observed children with Williams, and they are supertrusters," says Zak, who is both a neuroscientist and an economist. "They don't modulate the balance between trust and distrust."
 * Trusting Everyone**
 * Oxytocin: The Trust Hormone**

He says that in a normal brain, oxytocin is generated only after some concrete event or action: A man you pass on the street tips his hat; a woman smiles. "When someone does something nice for you — holds a door — your brain releases this chemical, and it down-regulates the appropriate fear we have of interacting with strangers," Zak says. Suddenly, you are filled with a sense that the person before you is not a threat. And then just as quickly, according to Zak, it disappears — for very good reason. "If you just had high levels of oxytocin, you would be giving away resources to every stranger on the street," he says. "So this is a quick on/off system." Unless, of course, the system gets disregulated, which is what Zak and other scientists say happens with Williams syndrome. Zak first got interested in trust more than a decade ago after co-authoring a study that looked at trust levels in different nations and their economic stability. The study found that the higher the level of trust, the better the economic status of the nation. The work got Zak thinking more generally about different ways to manipulate trust, and so starting in 2001, Zak began spraying oxytocin up the noses of college students to see if the hormone would change the way they interacted with strangers. It did. Squirt oxytocin up the nose of a college kid, and he's 80 percent more likely to distribute his own money to perfect strangers. This gave Zak an idea. Like some comic-book villain concocting a plan to take over the world by dumping happy pills in the water supply, he wondered if it might be possible to use this molecule — oxytocin — to change the way people felt about the government. "How much does this scale up?" Zak wondered. Could the effect go from the individual all the way up to gigantic institutions like the government? Zak decided to see. He undertook this experiment at a particular historical moment: America was in the midst of the Great Recession. "Trust in government is at an all-time low, and there certainly are kinda macro reasons for that," says Zak. "But could there be biological reasons? That was the question in this study: To what degree does the biology of trust, which we associate with oxytocin, affect trust in government and trust in government officials?" Zak put 130 test subjects through his normal routines. He sprayed half of them with oxytocin, half with a placebo, then ran them through a battery of tests and measurements. "The people on oxytocin did report that they trusted other people more, and the people who trusted others more also trusted their government more. So it's sort of a two-step process," he says. Zak points out that it's well-documented that trust in government declines during times of economic hardship. We also know, he says, that during periods of economic hardship, people are often exposed to prolonged stress and anxiety. And prolonged stress and anxiety, Zak says, are like poison to oxytocin.
 * Does Biology Affect Our Trust In Government?**

"So the underlying biological hypothesis is that stress — particularly stress that does not have a clear ending point — inhibits oxytocin release. So there //could// be an actual biological reason why trust in government is so low." But Margaret Levi is a professor at the University of Washington and also at the University of Syndey who has also spent much of her career looking at trust, and she's skeptical. "I think that biology plays a very trivial role influencing trust in government and distrust," she says. This isn't to say, Levi adds, that the work is without merit. For instance, one of the things that Zak's experiment suggested was that it was mainly through trusting other people that trust in government increases: Those who trusted their fellow man more also trusted government more. And this, says Levi, is consistent with one of the big theories that's dominated this area for the past 20 or so years. The theory was proposed by a Harvard professor named Robert Putnam, who wrote a famous book called //Bowling Alone: The Collapse and Revival of American Community//. Putnam essentially argues, Levi says, that the way in which trust in government is generated is basically bottom up. It's from the relationships that we form with others through various kinds of neighborhood and local organizations, like soccer clubs and choir groups, that we come to have confidence and trust in each other. This ultimately leads to a trust in the institutions of government and the institutions of the economy. Now, this is not Levi's view. From her perspective, it works top down: Governments need to be trustworthy to get people to trust them. "If we're talking about trust in government, the most important factor is whether people believe the government is doing the job they want government to do for them," Levi says. But whether it is government being effective or biology or trust generated through social groups, Zak says it's critically important to better understand trust. "Of all the things that economists have looked at to understand why countries are rich or poor, trust is like that big gun that we've been searching for," he says. "The effect of trust at affecting economic growth is substantial compared to everything else that economists have looked at."
 * Not Convinced Biology Is To Blame**