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Angela McManaman
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Lab research opens doors for undergraduate students Undergrad lab workers

Undergraduate research assistants in UWM’s Child Neurodevelopment Research Lab. Read more about students' work in the lab.

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Genes at play: Assessing abilities & potential in children with genetic disorders

Photo by Peter Jakubowski

Child Psychologist and Associate Professor Bonnie Klein-Tasman and Emerson, 2½-years-old, in UWM’s Child Neurodevelopment Research Laboratory.

At first glance, the genetic disorders Williams syndrome and Neurofibromatosis-1 (NF-1) don’t look much alike.

Many people who have Williams syndrome sport an abundance of curly hair and a facial shape described as “elfin.” The picture of NF-1 can include café au lait spots, tumors on or under the skin, or freckling in unusual places.

What the two genetic disorders do share is an unusual ability to shape emotional and cognitive development – and the expertise of University of Wisconsin–Milwaukee Associate Professor and Clinical Psychologist Bonnie Klein-Tasman.

Fifteen years of research and clinical practice have made her one of the nation’s few experts on Williams syndrome – work that is funded, in part, by the National Institutes of Health and the Center for Addiction and Behavioral Health. Armed with funding from a UWM Research Growth Initiative grant, Klein-Tasman and UWM child psychologist Hobart Davies are now collaborating with Children’s Hospital of Wisconsin to study emotional and behavioral implications of NF-1.

Looking beyond genetics

Klein-Tasman's and Emerson laboratory is stocked with toys, books and other tools that help Klein-Tasman assess fine and gross motor skills, receptive language and speech development in young children.

“We know the genetic origins of Williams syndrome and NF-1 can profoundly shape how children think and learn and look,” says Klein-Tasman. “But we don’t know enough about the profile of abilities of each disorder.

“Learning more about how each affects emotional and behavioral development early on gives us the information we need to point children to the right support resources.”

This learning process includes play-like assessments of children 2 and older in UWM’s Child Neurodevelopment Research Laboratory.

Both Williams syndrome and NF-1 are associated with elevated risks of learning disorders, emotional difficulties, attention-deficit hyperactivity disorder and behavioral issues. So Klein-Tasman evaluates a child’s fine and gross motor skills, receptive language and speech development, plus indicators of emotional and behavioral development.

Nicole Beaumier found Klein-Tasman’s name on the Internet, shortly after her infant daughter was diagnosed with Williams syndrome.

“I was interested in any information Bonnie had to give,” says Beaumier, who has organized two fundraisers to benefit the Child Neurodevelopment Research Laboratory. “Her knowledge of the disorder is so much greater than what any pediatrician or general practitioner could give us.”

“Bonnie can really empower parents,” agrees Pamela Trapane, clinical geneticist and professor at Children’s Hospital of Wisconsin and the Medical College of Wisconsin. As director of Wisconsin’s only Neurofibromatosis Specialty Clinic, she is co-investigator on the NF-1 study.

“I’m there to monitor the child’s health, but as a geneticist I’m at a disadvantage,” Trapane continues. “I rely on people like Bonnie to have input on additional resources to address the needs of the whole child.”

A window into Williams syndrome
An example: For children with Williams syndrome experiencing language delay, Klein-Tasman suggests incorporating speech therapy into a treatment plan.

But once the talking starts, it never really stops. This amiability, says Klein-Tasman, gives Williams syndrome its compelling profile and makes her patients charming companions inside the laboratory and out.

“Learning more about how each affects emotional and behavioral development early on gives us the information we need to point children to the right support resources.”

“When I have a child here, they’ll pop their head in every professor’s door on the way to the lab, just to say ‘hello.’ Kids with Williams are very interactive; I rarely have a child who says ‘I don’t want to do this,’ or who won’t participate.”

Today, Emerson Beaumier speaks in two and three-word bursts – but her mother already sees clear signs of what researchers call the “Williams personality.”

“In a grocery store she’ll walk up to another person, anybody, and pull on their pant leg and say ‘Up. Up,’” explains Beaumier.

This extreme sociability is paired with cognitive deficits – also characteristic of Williams syndrome. Small talk comes easily, but may not progress into more complex, interpersonal conversation.

Study of the precise relationship between genes and the Williams syndrome personality continues. More is known about how the disorder’s genetic trademark affects other functions: the deletion of 20-odd genes results in abnormal brain structure and function.

“Visuospatial processing difficulties are tied to abnormal brain function in the dorsal region,” Klein-Tasman explains. “Walking, writing, assembling puzzles and comprehending patterns are challenges for people with Williams syndrome.”

To help children learn and live with Williams syndrome or NF-1, every assessment results in a list of therapeutic resources and task-oriented recommendations

“Things like: ‘Separate the task of learning to write from the task of learning,’” Klein-Tasman explains. “It’s not always possible for a child to learn to construct a sentence and write by hand at the same time.”

What’s next for NF-1
Less is known about how NF-1 affects emotional and cognitive development. Its physical manifestations are better documented and include tumor growth, which can lead to various complications. These symptoms tend to progress with age.

“We know that half of people with NF-1 also have learning disorders,” says Trapane. “But can we find early predictors that help us better diagnose kids under the age of 5?”

As principal investigator on the NF-1 study, Klein-Tasman is collaborating with Trapane and Davies on a three-year, 30-child study designed to help answer that question.

Play-like, structured interaction with children ages 3-6 forms a cornerstone of this research study. But unlike Williams syndrome, in about half of cases NF-1 is genetically inherited. This can make the research process both more complicated and more relevant for the parents involved.

“We meet families who have been severely affected by learning disorders associated with NF-1, and they’re not always able to understand the broader scope of this diagnosis,” Trapane explains. “But they do know that they were isolated as children with a learning disorder, that they struggled as children and may continue to struggle now.

“This research should help them be able to advocate better for their own children, like modifying school curriculum, so their children have a better experience.”