This is not the document you are looking for? Use the search form below to find more!

Report home > Psychology

WILLIAMS SYNDROME GUIDELINES FOR TEACHERS

0.00 (0 votes)
Document Description
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.
File Details
  • Added: April, 28th 2011
  • Reads: 365
  • Downloads: 3
  • File size: 222.41kb
  • Pages: 24
  • Tags: williams syndrome, teachers, guidelines
  • content preview
Submitter
  • Name: kenjirou
Embed Code:

Add New Comment




Related Documents

WILLIAMS SYNDROME GUIDELINES FOR PARENTS

by: liesje, 28 pages

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, ...

Evidence for superior parietal impairment in Williams syndrome

by: etta, 2 pages

Parietal lobe impairment is hypothesized to contribute to the dramatic visual-spatial deficits in Williams syndrome (WS). The authors examined the superior and inferior parietal lobule in 17 patients ...

New height, weight and head circumference charts for British children with Williams syndrome

by: patrick, 5 pages

This paper aims to produce a growth reference for British children with Williams syndrome

A review of various abilities improved after the basic Tomatis Method Program for autistic, Williams syndrome, and ADHD children

by: pamela, 1 pages

Utilizing an 'Abilities to Be Improved' and an 'Abilities Improved' Form pre and post the basic Tomatis Program of 60 hours of listening, parents of 100 autistic children, 5 Williams syndrome ...

Past tense formation in Williams syndrome

by: petra, 34 pages

It has been claimed that in the language systems of people with Williams syndrome (WS) , syntax is intact but lexical memory is impaired. Evidence has come from past tense elicitation tasks wit ha ...

The Williams Syndrome Cognitive Profile

by: terttu, 25 pages

Williams syndrome is a rare neurodevelopmental disorder caused by a hemizy-gousdeletion of approximately 1.5 mega bases on chromosome 7q11.23. In this article, we outline a Williams Syndrome ...

Williams Syndrome: Pediatric, Neurologic, and Cognitive Development

by: roberto, 7 pages

This study examines the developmental history of 32 Williams syndrome patients, positive to the fluorescence insitu hybridization (FISH) test. The information is intended to provide help for early ...

Abnormal Cortical Complexity and Thickness Profiles Mapped in Williams Syndrome

by: liang, 13 pages

We identified and mapped an anatomically localized failure of cortical maturation in Williams syndrome (WS), agenetic condition associated with deletion of 20 contiguous gene son chromosome7.Detailed ...

Implicit Learning in Children and Adults With Williams Syndrome

by: valerio, 25 pages

In comparison to explicit learning, implicit learning is hypothesized to be a phylogenetically older form of learning that is important in early developmental processes (e.g., natural language ...

Affective prosody in children with Williams syndrome

by: kenjirou, 14 pages

The aim of the current study was to investigate expressive affect in children with Williams syndrome (WS) in comparison to typically developing children in an experimental task and in spontaneous ...

Content Preview



WILLIAMS SYNDROME
GUIDELINES FOR TEACHERS


by

Orlee Udwin, William Yule & Patricia Howlin






FOREWORD


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, 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.
Illustrations by Cynthia Cooper.
First published in 1991.
Revised version published in 2007
Copyright 1991, 1998, 2007

Published by:
The Williams Syndrome Foundation
161 High Street
Tonbridge, Kent TN9 1BX





CONTENTS

Description of the Syndrome

Type of Schooling

Speech and Language Skills

Perceptual and Motor Difficulties

Reading

Writing and Spelling

Numeracy

Concentration Difficulties and Over activity

Coping with Behavioural and Emotional Difficulties

1. Anxiety
2. Preoccupations, Obsessions and Stereotyped Behaviours
3. Temper Tantrums and Moodiness
4. Feeding Difficulties Further Reading
5. Hypersensitivity to Sounds

Social Relationships

Teaching Self Help and Independence Skills

1. Toilet Training
2. Dressing
3. Independent Travel

On Leaving School

Further Reading

Acknowledgements







DESCRIPTION OF THE SYNDROME

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.

GENETIC CHARACTERISTICS

Williams syndrome is a rare genetic disorder, with a prevalence of between 1 in 7,500 and 1 in
20,000. It is caused by a deletion of approximately 21 genes on one copy of chromosome 7. This
is sometimes knows as the Williams syndrome critical region (WSCR). Our understanding of the
consequences of the deletion of these genes is very limited. However, research is beginning to
identify the effects of some of the commonly deleted genes. The first of the deleted genes
identified was the elastin gene. Deletion of this is associated with issues regarding connective
tissue, such as heart disease, premature ageing of the skin and hoarse voice. The deletion of the
elastin gene is extremely common in WS, occurring in 95-99% of individuals. As such, it is often
used as a genetic marker for WS, with the deletion typically being assessed using Fluorescent in-
situ Hybridisation (FISH). Other deleted genes have been hypothesized to have roles in visuo-
spatial difficulties (LIMK1), level of intellectual disability (GTF21), facial characteristics
(GTF2IRDI), differences in brain structure (CYLN2), hypercalcaemia (BAZ1B) and diabetes
(STX1A). Overall, it appears that while some genes may have a direct effect, many genes may
interact with other genes at various stages throughout life to produce the characteristics of 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 children with WS. However, people with WS themselves have a 50% chance of
transmitting the condition to their children.

Children with WS tend to be delayed in their development and they show a range of learning
difficulties, varying from mild to severe. 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.

ABILITIES OF CHILDREN WITH WILLIAMS SYNDROME

• Mild to moderate learning difficulties
• Relatively good spoken language, but poorer comprehension
• Fluent and articulate speech by school age, incessant chatter
• Marked gross and fine motor problems
• Marked visuo-spatial difficulties


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, cooperative 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 spoken
language and this together with their 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.

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


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 undertaken over the last 25 years 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.


TYPE OF SCHOOLING

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 complex special educational needs
• 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

SPEECH AND LANGUAGE SKILLS

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.

DISCOURAGING EXCESSIVE CHATTER
AND INNAPROPRIATE SPEECH

1. If a child has a favourite topic he talks about repetitively (e.g. trains, illness),
or a set of questions he always asks, it may be advisable for school staff to
ignore him when he launches into his 'favourite' topic. If they answer such
'pet' questions each time, the child will be encouraged to ask them again and
again!

2. Parents, teachers and other adults too could make a rule to answer the
child's questions ONCE but to IGNORE all repetitive questions and topics
thereafter, and either to turn away from the child or CHANGE THE
SUBJECT straight away and move on to topics that are more relevant.

3. Another useful strategy is to insist, when a child's question has been
answered and he asks it once again, that HE repeat the answer that has
already been given.
4. A CONSISTENT approach is important, so urge all adults to adopt exactly

the same strategy at home as well as at school. With such methods, socially
appropriate speech should increase rapidly and inappropriate speech will
steadily decline.
PERCEPTUAL AND MOTOR DIFFICULTIES

Many children with WS have particular difficulties with gross and fine motor activities, with eye-
hand coordination, 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.

TEACHING VISUO-SPACIAL AND MOTOR SKILLS: SUGGESTIONS FOR
CAPITALISING ON THE CHILDS STRENGTHS

1. Harness the child's superior spoken language abilities in training
perceptual and motor skills. It often helps if she can be encouraged to talk
through each step of an exercise while she is doing it and to speak out loud
what she is doing. This can help her to focus her attention on the task in
hand and also provides verbal reinforcement and support for the activity.

2. Introduce teaching materials using objects and themes that the child is
especially interested in or even obsessed with, in order to motivate her to
work on tasks in which she may not be directly interested. For example, if
cars fascinate her or machines get her to trace over, copy and draw
outlines of these. She will be more willing to practice pencil and paper
skills if she is asked to draw something she is interested in rather than any
other object or shape.


3. Try to incorporate music into appropriate gross and fine motor exercises
such as balancing, ball play, threading beads etc. Clapping to music and
banging musical instruments can also be used to encourage early
perceptual-motor development.

4. There are many enjoyable computer programmes available, which help
children to develop skills in matching, discrimination etc.
READING

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.
WRITING AND SPELLING

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.
CHILDREN WITH WILLIAMS SYNDROME MAY FIND IT
DIFFICULT TO HOLD A PENCIL OR PEN –
SOME SUGGESTIONS:

1. Encourage the child to try using a pencil grip. This device
is attached to a pencil or crayon and helps to develop an
appropriate tripod grip.
2. An elastic band wound tightly round a pencil or pen just
above the point will prevent the child from grasping the
pen/pencil too low down thereby obscuring what she has
just written.
3. If the child is unable to hold a pencil or pen sufficiently
tightly, or cannot exert sufficient pressure with the
pen/pencil, try giving her a fatter pen or a felt-tip pen.
Children may also enjoy trying out fluorescent pens.
4. The physical task of writing may be very tiring for WS
children. Avoid excessive fatigue by limiting the amount of
copying/writing required at any one time.

NUMERACY

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

Download
WILLIAMS SYNDROME GUIDELINES FOR TEACHERS

 

 

Your download will begin in a moment.
If it doesn't, click here to try again.

Share WILLIAMS SYNDROME GUIDELINES FOR TEACHERS to:

Insert your wordpress URL:

example:

http://myblog.wordpress.com/
or
http://myblog.com/

Share WILLIAMS SYNDROME GUIDELINES FOR TEACHERS as:

From:

To:

Share WILLIAMS SYNDROME GUIDELINES FOR TEACHERS.

Enter two words as shown below. If you cannot read the words, click the refresh icon.

loading

Share WILLIAMS SYNDROME GUIDELINES FOR TEACHERS as:

Copy html code above and paste to your web page.

loading