Does a rare genetic disorder make some children incapable of racial biasis?

What if people were unable to recognize the difference between races? For some people with Williams syndrome, this is the case.

Today we honor the memory of Dr. Martin Luther King, a man who so famously declared: “I have a dream that my four little children will one day live in a nation where they will not be judged by the color of their skin, but by the content of their character.”

As a nation we are still struggling to achieve his dream, but what would life be like if people where completely incapable of recognizing, or caring, that others are different? Such may be the case with Williams syndrome, a rare genetic disorder where people with the condition show little to no personal biases or fear in social situations.

In fact, a study released by Current Biology suggests many children with Williams show no signs of racial biases— or even awareness of race— unlike typically developing children of every other race and culture. As one mother of a child with Williams syndrome put it, to her daughter, “There’s no such thing as strangers, just friends she hasn’t met yet.”

Not long ago National Public Radio did a fascinating string of reports on kids with Williams syndrome, highlighting their unique behaviors and the condition’s treatment. For nearly two decades Children’s Hospital Boston has run a multidisciplinary clinic for people with Williams. In the following interview Leslie Smoot, MD, director of Children’s Williams Syndrome Clinic, explains more about this rare condition.

What are some of the early signs that may indicate Williams syndrome?

Early diagnosis of Williams syndrome in children may come to attention due to findings of cardiovascular disease in infancy, failure to thrive, slow growth or feeding difficulties. In cases where children are diagnosed a little later, it’s usually because of noticeable developmental delays involving speech and motor difficulties. Some Williams syndrome patients also often have a distinct facial appearance, with slightly more narrow features and a broader smile. These attributes, when combined with specific manners or behaviors, are what usually prompt tests for Williams syndrome. Thanks to advancements in genetic testing, which have improved significantly in the past few years, the ability to clinically confirm a Williams syndrome diagnosis is much faster than it was in the past. But even in our high-tech era, it’s also very common for the initial recognition of Williams syndrome to come from someone who lives with or works with another person with Williams syndrome.

Leslie Smoot, MD

Media coverage of Williams syndrome usually focuses on their lack of social fear or personal biases, which can be perceived as positive, but what are the set-backs for this type of behavior?

In the past Williams syndrome has received media attention for some of its more unique aspects, including what has been labeled a “cocktail party personality” – describing the sociability and outwardly friendly behaviors often seen in individuals with Williams syndrome. Things like a lack of racial bias or increased musical talent and empathic behavior have also been ascribed to be more prevalent in Williams individuals. These seemingly positive attributes can pose their own challenges as children and adults try to develop stable, healthy relationships. For example, if left to their own devices many young kids with Williams syndrome could wander off with anyone without knowing that it’s not safe. When you combine the attention difficulties some Williams syndrome kids have with that distinct lack of social fear, they’re at a very high risk of being vulnerable to danger.

Aside from the potential for a hyper-social personality, what are some of the other symptoms of Williams syndrome?

Along with cognitive limitations, you tend to see real difficulties with tasks involving visual-spatial planning and basic math. Direction and planning can be almost paralyzing to some individuals with Williams syndrome, and therefore they may require a good deal of support in day-to-day activities. Increasing anxiety and depression in older children and adults is not uncommon, despite their tendency towards a seemingly extroverted disposition.

How does Children’s care for individuals with Williams syndrome?

The group draws together expertise from multiple disciplines including occupational, speech and physical therapy, cardiology and genetics, neuropsychology, behavioral psychology and pediatrics and general psychiatry. Benefits of the Williams Syndrome Program here at Children’s derive from the fact that we’ve seen hundreds of individuals with Williams syndrome.

At the end of a multidisciplinary evaluation, we meet as a group to form a cohesive assessment of the individual, gaining insight from the observations of others. Hopefully we can offer useful recommendations which the family can bring to their community and local caregivers. Just being able to get a bunch of people together in a room that look at a problem differently can be very helpful. It’s a good example of the phrase, “it takes a village.”