Williams Syndrome (WS)

Williams syndrome (WS) is a genetic condition that is present at birth and can affect anyone. It is characterized by medical problems, including cardiovascular disease, developmental delays, and learning disabilities. These often occur side by side with striking verbal abilities, highly social personalities and often an affinity for music.

WS affects 1 in 10,000 people worldwide - It is known to occur equally in both males and females and in every culture. Unlike disorders that can make connecting with your child difficult, children with Williams syndrome tend to be social, friendly and endearing. Parents often say the joy and perspective a child with WS brings into their lives had been unimaginable.

But there are major struggles including life-threatening cardiovascular problems. Children with WS need ongoing medical care and early interventions, such as speech or occupational therapy. As they grow, they struggle with things like spatial relations, numbers, and abstract reasoning, which can make daily tasks a challenge. As adults, most people with Williams syndrome will need supportive housing to live to their fullest potential. Many adults with WS contribute to their communities as volunteers or paid employees and at The Collett School, enjoy promoting our school and the positivity of life to others.

Just as important are opportunities for social interaction. People with Williams syndrome often don’t process nuanced social cues and this can make relationships difficult.

Supportive Strategies With Williams Syndrome

Short attention span and distractibility:
* Attention difficulties often lead to associated difficulties such as impulsivity which can result in the child not following directions well, getting out of their seat, etc.
  • Flexibility in requirements for time spent working
  • Frequent 'breaks' in work time
  • A "high success," high motivation curriculum
  • Minimal distractions; auditory as well as visual when working
  • Rewards for attending behaviors and, when possible, redirection around 'off task' behaviors or ignoring same
  • allowing some degree of choice for the child in terms of activity
  • Small groups

Difficulty modulating emotions
*Extreme excitement when happy
*Tearfulness in response to apparently mild distress
*Terror in response to apparently mildly frightening events
  • Decide when this is a problem. For example, expressing enthusiastic excitement, albeit impulsively or without raising a hand, may be beneficial to the motivation of the class as a whole, whereas frequent tears and a high degree of anxiety is problematic for the child with Williams syndrome as well as the other children.
  • Help the child to develop increasingly effective internal controls to modulate emotions while adapting the environment to minimise situations of extreme anxiety and frustration. Anticipate beginning buildup of frustration. Help the child to remove himself from the frustrating situation and find a different activity before the frustration escalates
  • Minimise unexpected changes in schedule, plans, etc.
  • Use stories and role play/pretend play to act out various anxiety provoking situations with the child
  • Heightened sensitivity to sounds (hyperacusis)
  • This characteristic in combination with a tendency toward anxiety sometimes causes behavior problems around noise related activities such as fire drills, vacuum cleaners, ceiling fans, heating or plumbing systems, and school bells.

Some children may become distracted, overly excited or fearful with events.
  • Provide warning just before predictable noises when possible (fire drills, hourly bells etc.)
  • allow the child to view and possibly initiate the source of bothersome noises (e.g. turn the fan on and off, see where the fire alarm is turned on)
  • Make tape recordings of the sounds and encourage the child to experiment with the recording (playing it louder/softer etc.)
  • Perseverating on certain "favorite" conversational topics
  • Some children with Williams syndrome have "favorite" topics that they want to talk about more often than is socially appropriate. Sometimes these favorite topics have to do with things that make them anxious such as fire trucks, trains or lawnmowers. Other children may show overwhelming fascination with, or interest in bones or other topics related to the body. Some fascination with things that are scary is quite normal in people generally (hence our interest in horror movies or 'thrillers') although this tendency can be particularly acute in children with Williams syndrome. Sometimes favorite topics are simply areas the child is confident discussing, and the child may be relying on that topic to ensure that he/she will be a competent participant in the conversation.

  • include social skills teaching as part of the IEP. Use role play, stories, discussion and small group experiences to teach alternative appropriate topics, and expand the child's repertoire
  • When the favorite topic involves repetitious asking of the same question (e.g. which day are we having a fire drill) first respond sufficiently to make sure the child has understood the requested information. (you can check this by asking the child the same question) Then ignore the subsequent repetitions, while offering other topics and activities. Avoid a discussion of whether or not the topic will continue to be discussed as this prolongs the perseveration
  • Provide some time for discussion of the child's favourite topic
  • Capitalise on the favourite interest as a curriculum topic. The child will approach curriculum based on favourite topics with a high level of motivation

Anxiety around unexpected changes in routine/schedule
  • Provide a predictable schedule and routine with specific warnings (e.g. a specific song a few minutes before cleanup time) marking daily transitions
  • Minimise unexpected changes
  • For preschool aged children: use of picture schedules for daily routines, and wall calendars with big squares on which special events can be sketched are helpful.
  • For older children: use digital watches and date books
  • Evaluation of other issues which might be making a child susceptible to feeling anxiety or a loss of control around changes
  • Capitalize on the child's orientation to a predictable schedule to work in less desirable but necessary activities at predictable times
  • Rocking, nail biting or skin picking
  • Usually these behaviors are fairly mild and may not pose a problem. It is important to realize that many of these behaviors may simply be outside the child's capacity to consistently control. Therefore, you should not dwell on them or continually remind the child not to do the behavior.
  • Ignoring the behaviors when possible while trying to lower environmental stress is usually sufficient to reduce them
  • If the behavior bothers the child or other children, sometimes occasional reminders in conjunction with behavioral techniques can be helpful (e.g. a sticker for each hour without nail biting)

Difficulty building friendships.
In spite of a tendency to have a very sociable nature, children with Williams syndrome often have difficulty building friendships. This is probably due to difficulties around sustaining attention, and impulsivity, as well as developmental and learning difficulties. Many of the children are, however, able to develop true friendships and this should be a goal included as part of the children's educational development. This may require extensive initial help from teachers.

Include social skills development as a "Goal" in the child's IEP
  • Work as a team with the child's parents regarding promoting a friendship with another likely friend. Encourage mutual visiting at homes
  • Facilitate social interaction during teaching activities (e.g. have the child with Williams syndrome and a likely friend pair up in working on a project or reading a story together)
  • Consider a variety of relationships for friendship building, including older or younger children and children with or without special needs

Our 'Wave One Strategies' for working with children with WS

Strengths of children with WS
Sociable nature, expressive vocabulary, long term memory for information, short term and long term auditory memory, hyperacusis (both positives and negatives - helps with phonics, hurts when child becomes excessively worried about noises).

Areas of Difficulty
Attending difficulties (often distractible & impulsive but not necessarily hyperactive), visual-spatial & visual-motor integration difficulties, visual memory deficits, difficulty with abstract concepts and abstract reasoning, perseveration on "favourite" topics.

Children with Williams syndrome have more difficulty processing nonverbal information than verbal information and there is usually a discrepancy between nonverbal ability and verbal ability in the Williams Syndrome cognitive profile. Children with WS share several key characteristics with children who have been diagnosed as having a nonverbal learning disorder. They both exhibit spatial relationship difficulties resulting, in the classroom, in handwriting problems. They share difficulty with maths, which also has spatial roots, as well as being associated with the abstract reasoning deficit. They do best at reading in general, which is linked to their verbal strengths. They do better at the lower level reading sub-skills such as word identification and phonics than they do at comprehension, which requires more abstract reasoning and, in general, the ability to go from parts to a whole. Comprehension also requires the reader to be able to understand implicit semantic relationships among words and be able to make inferences.

An area of difference appears to be in personalities. WS children, as we well know, are very sociable and outgoing. Children with nonverbal learning disorders tend to be shy and withdrawn, although they too are reported to be very verbal and friendly as very young children. Both, however, have some difficulty making and sustaining friendships. The common factor seems to be that both have difficulty reading nonverbal cues, and therefore are often inappropriate in their social interactions.

Nonverbal function, in a cognitive sense, involves visual processing and the harder to describe idea of perception. The affected individual does not form visual images easily and does not revisualise well (i.e. from memory). S/he also perceives the world differently than someone whose perceptual ability is seen to be intact. In a way, perception is the more spatial aspect of cognition, e.g. going from parts to whole, understanding cause and effect, etc. So a child with a deficit in this area would tend to focus on the details (and even perseverate on them) but fail to grasp the complete picture.

Direct instruction can be helpful. As the name implies, it involves directly teaching each aspect of a skill. It also engages the student orally, to ensure that they become an active part of the process. Direct instruction programs are very sequential in nature, progressing in a building block way until the target skills are acquired and mastered.

The most common element of all of the remedial interventions described in the literature on nonverbal learning disorders is the use of verbal mediation and verbal self-direction, both for analysing information and for organising to perform a task. This means the child must be taught, through direct instruction, how to talk him/herself through various steps, to successful completion of the process or task.

This concept can be used to improve verbal reasoning, vocabulary development, reading comprehension, and social skills. Because writing involves cognitively difficult processes requiring idea development, organisation, and the ability to go from parts to a whole, it lends itself well to a verbal mediation approach.