Williams Syndrome is Rare, Presents Challenges

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A new study finds that children with Williams syndrome do not stereotype people based on their race, a finding that has researchers taking a closer look at the origins of prejudice. Another topic that deserves closer examination is, What is Williams syndrome and what challenges does it present to the children who have it and to their families?

Williams syndrome is a rare neurodevelopmental disorder caused by 25 missing genes. According to the National Institutes of Health, the condition occurs in about 1 in 8,000 births; other sources say 1 in 10,000 births. It affects males and females equally. The syndrome was first recognized as a distinct condition in 1961, and it appears to cross all ethnic boundaries.

Most children with Williams syndrome share similar facial characteristics that include a small upturned nose, wide mouth, full lips, small chin, puffiness around the eyes, and a long upper lip length. These characteristics become more obvious as the children age.

At birth, most children with the syndrome have a slightly lower birth weight than their peers, and slow weight gain typically continues for several years, which leads many of the children to be diagnosed as failing to thrive. Compounding this failure to thrive are feeding problems that are associated with low muscle tone, poor suck and swallowing abilities, severe gag reflex, and other challenges. Fortunately these feeding difficulties tend to improve as the children get older.

While colic tends to last only a few months in normal infants, babies who have Williams syndrome typically experience the condition for four to 10 months. Hypercalcemia (elevated blood calcium levels) is sometimes believed to be the cause of the extended colic, and this calcium abnormality may stay with the child throughout his or her life.

One of the most serious problems children with Williams syndrome face is heart and blood vessel problems, including narrowing of the pulmonary arteries or the aorta. The degree of narrowing in either case can range from mild to severe, and in the latter case may require surgery. These children have a lifelong increased risk for developing blood vessel narrowing or high blood pressure.

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Parents of children with Williams syndrome may also find that their child has dental problems that can include bite abnormalities and widely spaced teeth, both of which can be corrected with orthodontics. These children also have a slightly increased risk of kidney problems and groin and umbilical hernias.

Low muscle tone and joint laxity is common in young children, and with age stiff joints may develop. Another condition that improves with age is hypersensitivity to sounds. Certain frequencies or noise levels can be painful and/or startling to children with Williams syndrome.

Developmental delays, including walking, talking, and toilet training, are common. By mid-childhood attention deficit is often a challenge, but it tends to improve as the children get older. Most people with Williams syndrome also have some degree of learning disabilities.

Despite all of these challenges, people with Williams syndrome have a very sweet, endearing personality and are extremely friendly and polite. They are typically not afraid of strangers and express an excessive interest in other people, more so with adults than with other children.

Parents need to work with their school system, often through Individualized Education Programs (IEPs) if pursued through the public education system, to ensure their child gets the education he or she needs and deserves. With a combination of physical therapy, occupational therapy, speech therapy, and therapies that focus on emotional, educational, and social skills, children with Williams syndrome often can go on to graduate from high school and enter academic or life skills oriented programs.

People with Williams syndrome and their families face a lifetime of challenges. Many go on to live happily on their own or in supported living environments, maintaining full or parttime jobs that they enjoy. For more information about this syndrome, contact the Williams Syndrome Association.

SOURCES:
National Institutes of Health
Williams Syndrome Association

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