Guidelines For Employers and Supervisors of People with Williams Syndrome
Produced by the WSF of the United Kingdom
This outstanding guide was written by Dr. Orlee Udwin, Dr. Patricia Howlin and Mark Davies for the Williams Syndrome Foundation-UK. It is reprinted here with the permission of the WSF-UK and the authors. I have edited this guide ever so slightly in order to use words more familiar to the American reader. Howard Lenhoff, Ph. D. 2/5/1998
Williams Syndrome (WS) is a rare, genetically determined condition occurring in approximately 1 in 20,000 live births. It is associated with a number of distinctive physical, behavioral and psychological features which differentiate affected individuals from other adults with learning difficulties.
Mild to severe learning difficulties. Relatively good spoken language, but often with poorer comprehension. Fluent and articulate speech, incessant chatter. Marked visuo-spatial difficulties. Gross and fine motor problems. Marked concentration problems.
Language and Communication:
The relatively good language and communication skills of adults with WS can be misleading and give the impression that individuals are more able than they actually are. On the surface most people with WS appear fluent and articulate, and they often have sophisticated and wide ranging vocabularies. However, their speech can also be inappropriate and repetitive, especially when talking about topics they are particularly interested in. They may also exaggerate and over-dramatize events in conversation with others. In many cases their comprehension is more limited, and inappropriate language may result from the use of words without fully understanding their meaning. In conversation, many adults lack the intellectual ability to imagine what other people may be thinking or to see somebody else's point of view. Consequently their speech tends to be one-sided, which may well antagonize or irritate others. People with WS often use chatter and incessant questions to initiate and maintain social contact with other people and to get attention.
Perceptual and Motor Abilities:
People with WS have particular difficulties with gross and fine motor coordination, with visual discrimination tasks, with orienting themselves and objects in space, and with judging distances, depths and directions. Even something as apparently simple as walking down stairs or on an uneven surface like grass or gravel, may prove extremely challenging. A fear of heights is common, and crossing the road can be a particular problem because individuals often find it difficult to judge accurately the speed and distance of cars. Difficulty with fine-motor coordination may make individuals appear clumsy and slow when performing delicate or intricate tasks, such as doing up buttons or threading a needle. Some individuals have a marked hand/arm tremor when performing such tasks. Handwriting is also affected by these difficulties.
Concentration and Attention Span:
Distractibility and limited attention span are significant problems, and adults with WS often find it difficult to concentrate on tasks for long periods.
Reading, Writing and Arithmetic:
The educational abilities of adults with WS vary considerably. Their relatively good verbal skills, including a very good memory for sounds and words, and excellent auditory sequencing skills, facilitate the acquisition of reading, and about 50% do learn to read, ill least at a basic level. Writing is more difficult to master because of the additional visual and fine motor skills required, but many individuals with WS attain at least some basic writing ability, and can write simple sentences. Because of their fine motor and visuo-spatial difficulties many have problems holding a pen or pencil; forming letters, spacing out words, and aligning words can also be difficult. Deficits in number work and arithmetic are common, and this makes coping with money a particular obstacle.
Anxiety:
People with WS tend to be over-anxious and may be easily upset by criticism or frustration, or by events that other people regard as trivial. In some cases anxiety might manifest itself as hypochondria - a tendency to exaggerate everyday aches and pains into serious illness. Anxiety may result from many different situations, but three areas in particular tend to give rise to difficulties:
(i) Excessive demands: The typically good verbal abilities of adults with WS may lead others to over-estimate their general ability, which can result in them being placed in situations that are too demanding, for example living arrangements or work settings that lack adequate support. Inappropriate demands can cause individuals to worry excessively and to become anxious or upset. As a result, they may try to get out of doing things by complaining of headaches or tiredness; they may 'escape' stressful situations by having tantrums or being aggressive; they may repeatedly ask for reassurance from others, or simply walk out of the room. If the excessive demands persist, the individual may become withdrawn, lose interest in food, activities and/or relationships, and become depressed.
(ii) Change and Uncertainty: Individuals with WS can become anxious when anticipating something new or different from their usual routine (e.g. a visit to the doctor). In the workplace, changes of staff or in the way work is undertaken can be particularly distressing.
(iii) Threat: Many individuals with WS have difficulty understanding the complexities of social interactions, and although increasing integration into mainstream society is generally a very positive trend, it is important to be aware that the outside world can also be a hostile and frightening place. Adults with WS can become extremely anxious if they feel that they are being 'picked on' or if someone is behaving in a confusing or threatening way. They can also be very protective of the people they care for (family, supervisors, friends) and can become anxious if they think that someone or something is threatening them in some way.