Williams Syndrome

DISABILITY CATEGORY: Mental Retardation—Williams-Beuren Syndrome

TYPES: Mild to severe – depending upon the limitations of each individual due to the various conditions that may occur as a result of (WS)

DEFINITION:

Our nation’s special education law, the Individuals with Disabilities Education Act (IDEIA) defines mental retardation as...

            “...significantly subaverage general intellectual functioning existing concurrently with deficits in adaptive behavior. And manifested during the developmental period that adversely affects a child's educational performance.” [34 Code of Federal Regulations §300.7(c)(12)]

Other Definitions:  Williams Syndrome is a genetic disorder characterized by mild mental retardation, distinctive facial appearance, problems with calcium balance, and blood vessel disease. (Source: Medline Plus)

Facts and Stats: Williams syndrome (WS) is a rare genetic disorder which is estimated to which causes both medical and developmental problems.  Estimates as to the commonality of Williams syndrome vary.  The Williams Syndrome Association reports that WS occurs in 1/20,000 births and the Williams Syndrome Foundation (UK) reports the occurrence of WS in 1/25,000 births.  Williams syndrome was first recognized as a distinct entity in 1961. It is present at birth, and affects males and females equally. It can occur in all ethnic groups and has been identified in countries throughout the world. (Source: Williams Syndrome Association and Williams Syndrome Foundation (UK)

Causes:  Williams syndrome is caused by a random genetic mutation in which a small piece of chromosome 7 is deleted.  Although WS is not usually an inherited condition, individuals who have WS have a 50 percent chance of passing it on if they decide to have children (Source: National Institute of Neurological Disorders and Stroke or NINDS)

Prevention: There is no known method of prevention for Williams syndrome.

Characteristics: Williams syndrome often affects individuals both mentally and physically.  Most people with WS have some degree of mental retardation and experience developmental delays.  Cardiovascular problems are among the most common physical problems that occur with WS.  The missing gene that causes Williams syndrome is also the gene that produces the protein elastin, which gives blood vessels and other tissues their elasticity. This typically results in narrowing of the aorta or narrowing of the pulmonary arteries.

Individuals with Williams syndrome often have very distinct physical characteristics that include a small upturned nose, long philtrum (upper lip length), wide mouth, full lips, small chin, and puffiness around the eyes. Facial features become more apparent with age.

Other common characteristics associated with Williams Syndrome are as follows:

• Hypercalcemia (elevated blood calcium levels)

• Low birth-weight/low weight gain

• Feeding problems in infants and young children

• Irritability (colic during infancy)

• Dental abnormalities

• Kidney abnormalities

• Hernias

• Hyperacusis (sensitive hearing)

• Severe phobia, distress (Source: NINDS)

• Musculoskeletal problems

• Overly friendly (excessively social) personality

• Learning disabilities (according to Medline Plus, more than 50% of individuals with WS have attention deficit disorders)

A very unique characteristic of Williams syndrome is an affinity to music.  Often, people with Williams syndrome are naturally drawn to music.  Music has been shown to help children with Williams syndrome overcome obstacles.  All individuals with Williams syndrome that have an affinity for music are not necessarily gifted in regard to music performance, but many of them are. Higher percentages of individuals with WS have also been shown to have perfect pitch than individuals in typical populations. (Source: Williams Syndrome Association)

Medical Treatment: Currently there is no medical treatment for Williams syndrome itself.  However, the specific medical conditions that may occur as a result of WS (e.g. cardiovascular problems) may require medical attention.  The amount and type of treatment required depends on the severity of each case and varies among each individual.

Prognosis: The prognosis for individuals with WS varies. Some adults are able to function independently, complete academic or vocational school, and live in supervised homes or on their own; most live with a caregiver. Parents can increase the likelihood that their child will be able to live semi-independently by teaching self-help skills early. Early intervention and individualized educational programs designed with the distinct cognitive and personality profiles of WS in mind also help individuals maximize their potential. Medical complications associated with the disorder may shorten the life spans of some individuals with WS. (Source: National Institute of Neurological Disorders and Stroke)

Educational Implications: As with any disability, the ultimate goal for children with Williams syndrome is to lead independent lives in a community.  Educational implications for children with WS are the much same as those for children with mental retardation and other developmental disorders.  Material should be taught at a slower pace with small approximations.  The varied personality traits of children with WS such as excessive social behavior and severe phobia, as well as well the commonality of learning disorders such as ADHD can create challenges in the classroom.  Teachers will need to observe students at all times to make sure that they are on task and may have to take steps to reduce chatter or overexcitement in the classroom.   Frequent breaks may be appropriate. In some cases, additional teachers may need to provide support.  Students with severe medical conditions may also require the support of additional teachers.

Due to the fact that many children with Williams syndrome have an affinity to music, music therapy may be very beneficial.  The Williams Syndrome Association suggests that a thorough music therapy assessment be performed by a licensed music therapist.  If music therapy is deemed appropriate for a child with Williams syndrome, the music therapist should become a part of the child's IEP team.  The Williams Syndrome Association also states that nearly all children with WS can benefit from individual speech, occupational and physical therapy. Interdisciplinary evaluations should be administered to determine the needs of each particular child.

Organizations and Websites:

Williams Syndrome Association
PO Box 297
Clawson, MI 48017-02971-800-806-1871
http://www.williams-syndrome.org

The WSA is the only group in the US devoted exclusively to improving the lives of individuals with Williams syndrome and their families. The WSA supports research into all facets of the syndrome, and the development of the most up to date educational materials regarding Williams syndrome.  The WSA website provides general information about Williams syndrome and special pages especially for parents and teachers of children with Williams syndrome.  There is also a special page for Williams syndrome and music (link - http://www.williams-syndrome.org/forteachers/musicandws.html)

Williams Syndrome Foundation  (USA)
University of California
Irvine, CA 92697-2300
(949) 824-7259
http://www.wsf.org

The Williams Syndrome Foundation (WSF) seeks to create or enhance opportunities in education, housing, employment and recreation for people who have Williams Syndrome and other related or similar conditions. The WSF identifies, initiates, funds and provides strategic guidance for major, long-range development projects, either by itself, or by cooperating with other organizations. The WSF website provides useful information about various aspects Williams syndrome as well as resources for new WS parents and families. The WSF website also includes a section devoted entirely to music with pages about music education, music research, articles of interest, musicians with WS and also audio recordings by musicians with Williams Syndrome (http://www.wsf.org/music/wsfmusic.htm).