Smith-Magenis Syndrome

Keys to teaching may be in the genes

By STACEY BURLING

Knight Ridder Newspapers

Sunday, September 2, 2001

Media, Pa. -- In her 16 years as a genetic counselor and researcher at Elwyn Inc., an institute that teaches hundreds of mentally retarded children and adults, Brenda Finucane has seen firsthand the role that genes play in how people learn and behave.

Children with Fragile X syndrome are almost always shy. Those with Williams syndrome are usually friendly and articulate. Prader-Willi syndrome makes people eat so uncontrollably that they often die of obesity. Children with Down syndrome usually learn best by having tasks broken into tiny parts, but those with Smith-Magenis syndrome seem to do better when they see the task all at once.

Research into the relationship between genes and behavior -- including personality and learning style -- has blossomed in the last decade. But Finucane and other geneticists and psychologists say the new knowledge is underused by doctors and educators working with those with mental retardation.

About 2.5% to 3% of the population has an IQ under 70, and about two-thirds of mental-retardation cases can be attributed to a specific cause. About 750 genetic disorders have been linked to retardation.

"The people in special ed . . . are generally unaware that it's something that should be of use to them," said Finucane, who is the only school-based genetic counselor in the nation, according to the National Association of Genetic Counselors.

And most doctors, she says, while quick to refer children with developmental delays to early-intervention programs, are less likely to order genetic tests unless there are obvious physical abnormalities or the parents want another baby.

Support organizations for genetic syndromes agree that diagnoses are still made late and that schools often do not realize the value of those diagnoses.

"There's a huge body of knowledge that is now available about providing intervention to kids with Fragile X," said Robert Miller, executive director of the National Fragile X Foundation. "Schools, by and large, are not making use of it."

But Douglas Fuchs, a Vanderbilt University professor who edits the Journal of Special Education, says the information on syndromes and learning styles is not yet finely tuned enough to be useful.

"To date," Fuchs said, "we do not have the evidence to support the claim that we know enough about education, that we know enough about genetic syndromes, to match the two in some profitable way."

Why genes matter

Finucane, who has written two books and who frequently consults with school districts about teaching children with certain disorders, tells a story about two syndromes to illustrate why genes matter:

A researcher asked two children with the same IQ to draw a bicycle. One who has Down syndrome drew a recognizable bike, with two wheels, a line across the top, handlebars and a basket. The other, who has Williams syndrome, drew some of the components of a bike -- wheels, a line, pedals -- but spread them across the page, without connection.

And if you ask children with these syndromes to describe something, you get notably different results. A child with Down syndrome is likely to answer in short, simple phrases or sentences. A child with Williams syndrome may wax eloquent, using long, grammatical sentences full of detail.

These children, Finucane says, are likely to respond best to different teaching approaches. Teachers need to know, she says, that children with Williams syndrome are not as bright as they seem and that their poor spatial skills may make it hard for them to ever write well. Their time might be better spent learning to use a computer, which they tend to find far easier than using a pencil.

Such ideas are gaining credence among psychologists and educators. The American Journal of Mental Retardation devoted a recent issue to genetic syndromes and their link to behavior and personality, a field called behavioral phenotypes.

This is "a growing movement, both in this country and abroad," said Elisabeth Dykens, a psychology professor at the University of California, Los Angeles, who edited the issue and co-authored a book with Finucane.

Obstacles

Many psychologists have a long-standing distrust of genetics because of its association with the eugenics movement, infamous for its drive to sterilize people with low intelligence, says Elliott Simon, a psychologist and Elwyn's executive director of clinical services.

The field of behavioral phenotypes also runs into the trend in special education to de-emphasize labels to avoid stigmatizing children, says Robert Hodapp, a research psychologist at UCLA who has worked with Finucane.

Educators say that it is helpful to know what causes a child's retardation but that they still look at each child individually because there is so much variation within each syndrome.

Susan Mateka, assistant principal of the Child and Career Development Center in Coatesville, Pa., for example, notes that her school has three children with Angelman syndrome, in which those affected are characteristically cheerful but do not speak, and that all three function at different levels.

"I think having the information on any child's disability syndrome is helpful for a lot of reasons," Mateka said, "but I can't say that it's crucial to planning a program for them."

First-hand knowledge

Unlike most genetic counselors, who diagnose abnormalities and talk to couples about the odds of genetic problems in their offspring, Finucane is in a position to watch her patients as they work and learn at Elwyn's sprawling campus southwest of Philadelphia, where there are both day and residential programs.

While there is no scientific proof that specific teaching methods are more effective for people with specific syndromes, Finucane says common sense tells us that children learn more when teachers play to their strengths rather than their weaknesses.

For example, she says, it makes little sense to insist that children with Fragile X make eye contact since they find it stressful. For the same reason, teachers should not insist that a child with the syndrome participate in morning circle time.

"What they really should be doing is letting him stay outside the circle until he's ready," Finucane said. Over the year, "little by little, he'll start creeping back."

When it comes to Angelman syndrome, Finucane realizes that teachers will want to try to teach these children to speak, though none has ever used more than a handful of words. But, she says, it is wise to also begin teaching alternative forms of communication as early as possible.

Using their knowledge

A tour of Elwyn one morning illustrated some little ways that educators can use information about a syndrome. First stop was the kitchen, where two teenagers were preparing lunch, as part of their pre-vocational training.

Joseph "Bull" Traitz, an 18-year-old with Fragile X syndrome, spooned pineapple into plastic cups, then put tops on them. In the past, special-education teachers would have taught this task by breaking it into smaller segments, said Simon, the clinical-service director, but people with Fragile X are great imitators, so Traitz learned by seeing the whole job performed several times.

Also in the kitchen was Marsha Blakney, 17, with Prader-Willi syndrome. That Blakney, cheerful with a broad, gentle smile, works around food is "amazing," Simon says, because people with Prader- Willi are often so obsessed with food that they even steal scraps from trash cans.

With close supervision, Blakney has been able to succeed. Her job, placing generous helpings of different foods into a segmented tray, plays to some of her strengths: good eye-hand coordination and spatial skills.

Nearby, in the Elwyn gym, a big group of students was competing loudly and chaotically in various games, a particularly stressful situation for children with Smith-Magenis syndrome, who are emotionally immature.

Simon pointed out that a teacher was giving student Shanna Murray, who has the syndrome, a long hug. The teacher might have been slower to do so had she not known Murray's genetic history, he said.

Minutes later, Lori Knight, 14, a friendly girl who also has Smith- Magenis and likes reading Stephen King novels, returned from the gym. She sat inches from teacher Mark Gottesman -- an arrangement he calls "Siamese twins" -- as he talked to the class from the front of the room.

It's a way to give Knight the attention she craves and still get something done, Gottesman says. It's also a tactic that many teachers who do not understand genetics would resist, Finucane says, for fear of appearing to be playing favorites.

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