"It would scare me to death to have him work there."
Terry Monkaba is talking about her son Ben, and the prospect of him finding a job at a Las Vegas casino. Many parents might feel that way, but Monkaba's anxiety goes deeper. That's because Ben, 28, has Williams Syndrome.
Once called "cocktail personality syndrome," Williams Syndrome—particularly as it affects children—has captivated science writers for the past decade. In 2008's Musicophilia: Tales of Music and the Brain, Oliver Sacks describes visiting Berkshire Hills Music Academy, where he was immediately received by unusually friendly children. "They all seemed extraordinarily sociable and inquisitive," he writes, "and though I had met none of these campers before, they instantly greeted me in the most friendly and familiar way—I could have been an old friend or an uncle, rather than a stranger."
Strangers were also the topic of Alix Spiegel's 2010 NPR series on Williams Syndrome, where she told the story of a nine year-old girl named Isabelle. In one segment, Isabelle, who has Williams Syndrome, practices role-playing exercises that teach her the concept of distrust.
"Hello little girl, do you want to see my puppy?" Isabelle's mom practices with her.
"No way," recites Isabelle obediently.
"But my puppy's so cute! Are you sure?" prods her mom, the "stranger."
"Yes," says Isabelle.
"Come over here, I've got some candy too!"
[Sound of internal struggle from Isabelle]
"C'mon, come into my car and I'll show you," her mom tempts.
And with that, after holding out against puppies and candy, it's the offer of companionship that finally breaks Isabelle down. "Ok," she relents, as though she can't help but accept.
But adults with Williams Syndrome have received less attention in the media, partially because their condition predisposes them to diseases that shorten their life span, like diabetes and congenital heart defects, and partially because, as a science writer, children who instantly tell you they love you make for adorable story subjects. Adults with Williams, however, have a vastly different set of challenges than their younger counterparts. After a lifetime of parents and caretakers constantly watching out for their safety, can they learn how to protect themselves? Move independently in the world? And can they take on that hallowed marker of adulthood: a job?
To answer that last question, it's important to understand the scientific basis of Williams Syndrome, and what makes individuals with the condition pathologically vulnerable. Why does Monkaba, for example, fear her son Ben getting a job in Vegas? It has to do with the way his brain is structured, which diminishes his social wariness to an extreme degree—and for Ben, that could be dangerous.
The human body contains roughly 30,000 genes, but at conception, the deletion of just 26—a string comprising less than one percent of your genetic code—along chromosome seven results in Williams Syndrome. Their absence manifests in a variety of characteristics, and Dr. Colleen Morris, genetics section chief at the University of Nevada School of Medicine, is quick to point out that it's a "multisystem disorder"—meaning it affects lots of areas of the body besides the brain, and looks different on different people.
"That being said, there are some general commonalities," Morris says. "They get easily fatigued, and we're still trying to understand why. There's also a higher incidence of diabetes and hypothyroidism, as well as cardiovascular disease."
"Friendliness, oh my gosh. Lots, lots of social interest. High levels of empathy. Distractibility too, which makes it harder for them to focus and get work done."
Like Morris, Dr. Barbara Pober, a medical geneticist at Massachusetts General Hospital, was another one of the doctors turning her attention toward Williams Syndrome in the late '80s. In 1987, she began treating patients at Boston Children's Hospital, which opened one of the first Williams Syndrome clinics in the country. Today her patient base contains individuals of many ages with Williams Syndrome, including adults. Several don't have jobs.
"Sometimes that's due to external factors, like finding transportation, since most adults with Williams Syndrome don't live on their own and don't drive," Pober says.
The not-driving could be attributed to the fact that Williams Syndrome affects visuospatial construction, as evidenced by several studies, including one published in Brain in 2009, of which Morris was a part. But there's something else going on here. On the job, just how easy is it to take advantage of an individual with Williams Syndrome?
Work is an arena where relationships are forged, and alliances are built. The modern office is a test lab for social discretion, where we quickly learn who we can trust, and who we should avoid. But for individuals with Williams Syndrome, who often trust indiscriminately, this can be a problem.
"I've had many hard experiences at my jobs," says Amy Koch, 48, a resident at Marbridge, a nonprofit residential community in Austin, Texas. "To tell you the truth, I was very hesitant about going for another."