GUIDELINES FOR TEACHERS
by
Dr Orlee Udwin and Professor William Yule
Williams Syndrome (WS) is a rare condition, occurring in approximately 1 in 20,000 live births. As a result, most professionals will not have encountered a child with WS before, and they will understandably know little, if anything, about the characteristics and difficulties associated with the condition. The purpose of this booklet is to provide teachers with information about this rare disorder, and to elaborate on the special learning difficulties and needs of affected children.
The contents are based on research undertaken by the authors and others over the last decade, which demonstrates that WS is associated with a number of distinctive behavioural and psychological features which differentiate children with this condition from others with learning difficulties. Of course every child is different, and not everyone with WS will show all of the characteristics and difficulties described here. Nevertheless, it is hoped that this information and the advice and suggestions that follow will be of benefit to teachers, learning support assistants and others who work with children with the condition. A separate information booklet for parents is available from the Williams Syndrome Foundation.
We adopt the practice of referring to the child with WS as "he/him" and "she/her' in alternate sections.
Williams Syndrome (WS) is a genetically determined, intellectually disabling condition. Most affected children develop severe feeding difficulties in the first year of life, including vomiting, constipation and refusal to feed, and they may be irritable and cry excessively. A proportion of the children are found, on testing, to have raised levels of calcium in their blood. This subgroup with Infantile Hypercalcaemia is generally treated with a low-calcium and vitamin D-restricted diet and the feeding difficulties then improve, either rapidly or, in some cases, more gradually.
Many of the children also have a distinctive facial appearance, and renal and cardiac problems (a heart murmur, narrowing of the main arteries leading from the heart), which may be severe or quite mild. They may also have dental abnormalities, back and joint problems, raised blood pressure and a delayed rate of growth, including low stature and a slight build. The average adult height is 5 ft for females and 5ft 6in for males.
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ABILITIES OF CHILDREN WITH WILLIAMS SYNDROME
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Missing material (a microdeletion) on chromosome 7 has been identified in affected individuals, resulting in disruption to the elastin gene. Elastin is the structural protein in our body that gives elasticity to our tissues and organs. It is found predominantly in the walls of the arteries, in the lungs, intestines and skin; and reduced or abnormal elastin may explain some of the physical and medical characteristics, as well as the distinctive facial features, associated with WS. Most cases occur sporadically, and the risk to parents of having another child with WS is no higher than the original risk. Brothers and sisters of WS individuals, too, are not at any increased risk of having a child with WS. However, people with WS themselves have a 50% chance of transmitting the condition to their offspring.
Children with WS tend to be delayed in their development and they show a range of learning difficulties, varying from mild to severe mental handicap. About 50% have severe learning difficulties, while the remainder have mild to moderate learning difficulties . Overall the children's verbal abilities are markedly superior to their visuo-spatial and motor skills. Furthermore, they show a distinctive pattern of abilities and particular behavioural and personality characteristics which are common to most individuals with this syndrome, and which set them apart from other children with learning disabilities.
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BEHAVIOUR OF CHILDREN WITH WILLIAMS SYNDROME |
| Overactive | Excessive anxiety & worry |
| Limited concentration span | Preoccupation with objects/topics |
| Sociable & outgoing | Hypersensitive to sounds |
| Overfriendly to adults | Fearful of heights/uneven surfaces |
Children with WS tend to be very outgoing and sociable, and by school age most develop fluent and articulate spoken language. They love talking and interacting with adults and are mostly helpful, co-operative and eager to please. Many chatter incessantly but at a superficial level, and often in a formal, adult-like style, with frequent use of sophisticated vocabulary, stereotyped phrases and clichés. Taking turns and keeping to the point in conversation may be especially difficult for them. In many cases their understanding of speech is not as good as their expression, so the level of spoken language and highly developed sociability may be deceptive in suggesting that a child is more able than he actually is. In contrast to their relatively good speech, many children with WS have visuo-spatial problems and difficulties with gross and fine motor co-ordination. So they may be slower than usual in learning to sit and to walk, and they may find tasks like riding a bicycle, doing up buttons, cutting with scissors and holding a pencil particularly difficult to master. They tend to have poor posture, an awkward gait and limitations of joint movements. Because of their motor and perceptual problems they may also be fearful of heights and of negotiating stairs and uneven surfaces such as grass, gravel or sand.
Many of the children are overactive and find it difficult to sit still and concentrate on particular tasks for any length of time. On the other hand, they can become fascinated and preoccupied by particular objects or topics and spend a great deal of time absorbed with these. While many are very friendly (and even over-friendly) to adults and often actively seek out adult company, they may have difficulty in making and keeping friends of their own age, often managing somehow to antagonise their peers.
Children with WS are often described as having anxious personalities and worrying excessively about themselves and others. Toileting, eating and sleeping problems (including settling down to sleep and waking in the night) are fairly common. Most are also hypersensitive to a variety of noises, such as loud bangs, clapping or laughter, and they may become tense and fearful when hearing or anticipating these sounds.
Teachers often report finding children with WS baffling and difficult to teach because their apparently superior verbal and communicative abilities mask significant difficulties in other areas, including comprehension of language, hyperactivity and attention deficits, and severe visuo-spatial difficulties. This can result in an overestimation of their overall abilities. Of course every child is different, and not all children with WS will show all of these characteristics and difficulties to the same extent. However, the research we have undertaken over the last decade indicates that these features are common to many of them, and that unless they are tackled early on they may well persist into adulthood. In the following pages we describe the particular learning and behaviour difficulties and needs of children with WS, and present suggestions and guidelines for their remediation. The methods and approaches we will describe are well tried and have been used successfully to cope with a wide variety of difficulties. They can be adapted for use with any individual child.
As already described, children with WS have an unusual pattern of abilities, often with relatively good spoken language but poorer perceptual and motor skills and concentration difficulties. As a result, they have special educational needs that are quite different from those of other children, and it can be quite difficult to find a school that will be exactly suited to their particular learning needs. Their verbal skills and highly developed sociability may suggest a relatively high level of ability, but if they are placed in an ordinary school or in a school for children with Moderate Learning Difficulties they are often unable to cope because of their lower abilities in non-verbal areas and their limited concentration. On the other hand, if they are placed in a school for children with more severe learning difficulties children with WS may well miss out on much of the verbal and social stimulation from which they could benefit if they were among more able children. There is no one type of school that is ideally suited to their needs. Finding the most appropriate school will depend on the individual child's level of ability (which can range from mild to severe learning difficulties), and also on the provision in the particular schools that are available locally.
Some of the children start off in mainstream schools and cope very well there at the primary level, though typically with additional help from learning support teachers and/or assistants for some part of the school day. At the secondary level some of these children move to special schools, where they can benefit from more individualised teaching and learn at a slower and less pressurised pace. A few WS children continue in mainstream at the secondary level, for example attending a remedial class for some lessons and their ordinary class for other lessons.
Mainstream schooling is by no means appropriate for all or even for the majority of children with WS. Many of the children need the slower pace and more individualised attention that are currently provided in special schools and units. Each child's needs will be different. In a study we undertook in the late 1980's we found that:
- 30% attended schools for children with Severe Learning Difficulties
- 30% attended schools for children with Moderate Learning Difficulties
- 30% attended schools for children with mixed handicaps
- 10% were in mainstream schools
With the greater emphasis that is now being placed on integration and inclusion, we are seeing increasing numbers of children with learning difficulties, and thus also more children with WS, being educated in mainstream schools, with varying amounts of additional support. We believe that for inclusion to work effectively for children with WS, classroom settings which offer substantial support and a great deal of flexibility are necessary. Typically this will require:
- extra adults in the classroom
- small classes
- intensive involvement of specialist teachers
- learning support assistants
- carefully structured and graded work plans
- teamwork between classroom teacher, assistant, specialist teachers and speech and occupational therapists
In the pre-school years children with WS may be particularly slow to develop speech, and at this time verbal stimulation will obviously be vital. Structured language teaching in the early years is also important, involving as it does the building up of vocabulary and comprehension of labels, verbs, instructions etc. Prompting and imitation are useful tools, as are praise and other rewards to encourage motivation. Children with WS will benefit from speech and language therapy at all stages of their development, and the therapist should also work closely with parents and teachers and provide suggestions on programmes and activities that can be carried out at home and at school in order to expand the child's spoken language and language comprehension.
By school age, most children with WS will have developed fluent, articulate speech with sophisticated, wide ranging vocabularies and complex and grammatically correct sentences, which are quite out of keeping with their general level of learning difficulty. They may use long words and unusual phrases which they have heard other people use and remembered, and they may chatter to anyone they meet, if given the chance. However, their speech can also be inappropriate and repetitive, especially when talking about topics of particular interest to themselves. Many use chatter and incessant questions to initiate and maintain social contact with other people, and they are adept at using their superior verbal skills to get attention. In addition, they are inclined to exaggerate and over-dramatise events in conversation with others. For example, they may describe minor mishaps as major tragedies.
WS children's understanding of language is in many cases more limited than their expressive language might indicate. They may, for example, be able to recite instructions or stories word for word, yet have difficulty understanding the most basic of concepts. Sometimes they echo or repeat phrases and sentences spoken by the person they are interacting with, often with little understanding of what the person is saying. If the echoing is caused by poor comprehension, it will be helpful to point this out to the relevant adults (parents, school staff etc.) and to suggest that they try to simplify their speech. Similarly, if the child responds to an adult's comments or questions inappropriately or irrelevantly, this may be because he does not fully understand them. Again, it may be necessary to simplify instructions and comments to make sure the child understands what you and other adults are saying. However, if the child's echoic or irrelevant speech is simply a habit he has developed, then ignoring his utterances and distracting him by moving on to another topic can be a useful method of discouraging such non-meaningful verbalisations.
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DISCOURAGING EXCESSIVE CHATTER AND INAPPROPRIATE SPEECH
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PERCEPTUAL AND MOTOR DIFFICULTIES
Many children with WS have particular difficulties with gross and fine motor activities, with eye-hand co-ordination, with orienting themselves and objects in space, judging distances and directions, and with visual processing tasks including discrimination, sequencing and visual memory. As a result, specific remedial attention will need to be given to these areas. It is of course known that tasks such as sorting and matching objects and shapes and being able to trace over lines, are important skills to master before the child can move on to the basics of reading and writing.
Such tasks may be more difficult for a child with WS because of her particular deficits in visual perception and motor co-ordination. She will therefore need additional help and opportunities to practice skills in these areas, and teachers may want to encourage parents to practice some of these exercises with the child at home as well. Thought should be given to the way work is presented to the child; books and programmes that use a lot of colour and pictures are not the best materials to use because of visual overstimulation. Instead, teachers should try and select books and worksheets that have relatively little information on each page.
The child's visual and motor problems may also mean that she is nervous of heights, and of such activities as climbing, going down stairs, walking along a raised bar in the gym etc. She may have particular difficulties with throwing and catching a ball, cutting with scissors and riding a bike. All children will improve on these tasks with time, provided that they are given reassurance, support and plenty of practice. Occupational therapists can be very helpful in all of the above areas. They can assess and provide exercises for the WS child in the areas of co-ordination, balance, gross and fine motor activities, and they can advise school staff on exercises to carry out with the child at school. Where necessary, parents might be encouraged to approach the child's Paediatrician or the GP for a referral to an occupational therapist.
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TEACHING
VISUO-SPATIAL AND MOTOR SKILLS :
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The abilities of WS children vary a lot, but many do learn to read at a basic or, in some cases, at a more advanced level. As already discussed, many WS children tend to have relatively good verbal skills, including a very good memory for sounds and words and good auditory sequencing skills. In view of this, approaches to teaching reading which rely on these auditory skills and which emphasize a PHONETIC approach, are likely to be more effective than approaches which rely on the child's visual memory of how a word looks (such as the 'Look Say' approach).
Even at the beginning stages of reading it is important to show the child that printed words are meaningful; they tell a story and impart information. Where children are preoccupied by particular objects or topics (e.g. trains, pop stars, foreign places), introducing reading materials on these topics will enhance their interest and motivation to read. Parents can also be encouraged to adopt some of the above strategies when reading to (and with) their children in the pre-school and school years.Whatever one is trying to teach, it is worth remembering that children with WS can be easily distracted by extraneous visual stimuli. Thus beginning reading books which contain many detailed pictures and colours on the same page as the printed word(s) are far from ideal. It is better to use books which have a few simple pictures or outline drawings which are less likely to distract the child.
For many children with WS attainment in writing and spelling tends to be much poorer than in reading, because of the additional visual and fine motor skill requirements of the former tasks.
When the child begins to learn to write, she will be helped to trace over and then copy the letters of the alphabet. Because of the WS child's visuo-spatial and motor difficulties, progress may be slow in this area and involve a lot of practice and repetition, including preliminary practice in pre-writing activities to improve tactile perception, motor control and eye-hand co-ordination.
Problems with forming the letters and spacing out and aligning words are common. The child may also have difficulty keeping her place, or she may skip sections. But with perseverance progress will be made.
Again, the child is more likely to co-operate if the exercises she is given incorporate materials that bear directly upon her special interests and preoccupations. For example, tracing or copying words of things the child is particularly interested in (e.g. the makes of cars or of electrical gadgets) is likely to enhance her motivation. When she is first learning to write the letters of the alphabet, it might also be helpful to encourage her to use speech to talk herself through the exercises. Try and use the same verbal cues in the same pattern each time (e.g. "b goes down, then back up and around"). In this way the child can learn the words and then prompt herself in the task. At later stages, for example when trying to write unfamiliar words, WS children might be encouraged to place greater reliance on the auditory channel, for example by sounding and then spelling out the word verbally, and only then translating each letter sound into a letter in print.
Like most children, children with WS love working on computers and can practise useful exercises on them. If they can write and spell but have difficulty with pencil control, they might be encouraged to use computers for free writing, thus by-passing the difficulties of writing by hand. For many children with WS writing is such a slow and laborious task that it can deter them from what might otherwise be an enjoyable exercise such as story-telling. Where typing itself is difficult, or the child cannot spell, she can be encouraged to dictate stories, homework etc. to parents or teachers or into a tape recorder, thereby still deriving pleasure from the creative process inherent in such activities.
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CHILDREN WITH WILLIAMS SYNDROME MAY FIND IT DIFFICULT TO HOLD A PENCIL OR PEN - SOME SUGGESTIONS:
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Our research suggests that arithmetic is a marked area of difficulty for many children with WS. Their perceptual, visual-spatial and motor problems may make it difficult for them to manipulate numbers and to carry out arithmetic operations, as well as to comprehend the principles behind the operations. It will be important to work at the child's pace, and with frequent repetition.
Initially, teaching should incorporate materials which can be touched and moved by the child, for example concrete tokens such as blocks. Getting the child to perform gross motor skills linked to numbers can also help to strengthen number concepts e.g. taking 5 steps, jumping 5 times. Only thereafter should more abstract items be introduced, such as pictures. Similarly, when moving on to addition and subtraction the use of concrete materials (blocks, rods etc.) is recommended until the notions are well established.
Because of their difficulties with writing and shaping numbers, it may be helpful at first to separate the task of writing numbers from the teaching of mathematical concepts and operations. Concentration on writing down numbers on the page will distract the child's attention from the mathematical operations he is trying to perform. Teachers point out that even older WS children have great difficulty writing down numbers correctly and aligning numbers on the page, for example when adding and subtracting. Using lined or squared paper may help to ensure that sums are correctly set out on the page.
Learning to tell the time can be a further area of difficulty. If the child makes little progress despite strenuous efforts, then reliance on digital watches may be helpful. Teachers and parents can always go back and make further attempts to teach 'time-telling' on standard watches and clocks at a later date. As with any child, it is important to link time on the watch or clock to routine activities that take place at set times.
When teaching about money it will be helpful to use real money (rather than tokens or pictures) in real life settings, for example by taking the child to the supermarket to buy food.