Understanding the development of children with Down syndrome

An overview of the development of children with Down syndrome (5-11 years)

Sue Buckley1 and Ben Sacks2

1 Emeritus Professor of Developmental Disability, Psychology Department, University of Portsmouth, UK. Director of Research and Training, Down Syndrome Education International, UK.

2 Consultant Developmental Psychiatrist and Medical Advisor to Down Syndrome Education International, UK. Formerly Professor of Developmental Psychiatry, Charing Cross and Westminster Medical Schools, The University of London, London, UK.

Summary - Children with Down syndrome usually make significant progress during their primary school years. In this module, the goals for their development are discussed in the context of the progress in social, academic and personal independence skills that is seen in typically developing children during the period from 5 to 11 years. It is argued that the goals for children with Down syndrome should be age-appropriate and therefore many of the goals should be the same as for other children, although the child with Down syndrome may not achieve quite the same levels of competence in each area. This age period begins with settling into full-time school and it is the start of increasing independence from the family and moving away from the high levels of individual support that preschool children receive, to being able to be part of a group and to cope in the larger social world of the school and community. There is consistent evidence that children with Down syndrome gain significantly from full inclusion in mainstream schools. In particular, they show significant gains in spoken language, reading, writing and arithmetic. These gains will enhance their adult lives - especially the ability to communicate more effectively. However, the special educational needs of the children should be met wherever they are receiving their education and the key needs and appropriate adaptations for teaching and learning are described. Children with Down syndrome will benefit from a partnership between parents, teachers and therapists and specific goals are provided for parents, teachers, speech and language therapists and physical therapists, based on the research into the children’s specific profile of needs and into effective interventions.

Introduction

The aim of this module is to provide an overview of development during the period from five to eleven years and to assist the reader in integrating the material covered in the other DSii modules for this age group. These modules each cover specific aspects of development, i.e. motor, social and behavioural, speech and language, working memory, reading and numeracy, and family issues, in detail.

This module is intended to help parents, families, teachers, speech and language therapists, and other practitioners who work with children in this age range. In our experience, they all wish to know what developmental and educational progress to expect for a child with Down syndrome and how to help the child progress as fast as possible.

These two main questions are addressed, with a discussion of the wide range of individual differences in rates of development of children with Down syndrome. In order to answer the question of how to help children to progress, the reasons for their developmental profile are outlined as far as they are known, as this information should help to identify effective therapy and teaching strategies. The question of how to help also leads to a discussion of the importance of balancing family needs with the needs of the child with Down syndrome.

In our view, in order to understand the ways in which having Down syndrome affects children’s development it is necessary to consider what is known about the development of typically developing children. In the last thirty years, there have been considerable advances in our understanding of the processes of development, particularly in the areas of social learning, cognition and language. The greater our understanding of typical development, the easier it is to begin to understand the effects of a disabling condition such as Down syndrome on the processes of development. As we identify the specific effects of Down syndrome on development, we are in a better position to develop effective interventions and teaching strategies.

This is the approach taken throughout the DSii modules. We draw on research into the development of typically developing children, the specific research into the development of children with Down syndrome and research that has evaluated effective interventions and teaching strategies, where it exists.

Age-appropriate goals

In addition to using this information to understand the children’s developmental and learning difficulties, we assess the major developmental achievements of typically developing children over the age span and consider the relevance of these for setting targets for children with Down syndrome.

If individuals with Down syndrome are to be fully included in the community, as children and as adults, it is important to recognise that their place in society will be linked to their chronological age. For example, the child with Down syndrome will join a school at 5 years of age in the UK and move through school with their peers. This means that, despite significant delays in some aspects of development, such as speech and language and motor skills, the child with Down syndrome needs to be able to cope with the social demands of the classroom, for example, to sit still, follow instructions and learn the school routines. Targets for social and behavioural development therefore need to be age-appropriate and the task for the child is to manage in an age-appropriate social world, with limited language and delayed motor skills. Similarly, if a child with Down syndrome wishes to join a club such as Brownies or Scouts, they will do so at the appropriate chronological age and, again, will need to be able to cope socially.

As teenagers, children with Down syndrome reach puberty at the same age as other children. As teenagers they develop typical leisure interests and awareness of sexuality. TODO: references 4 The point being stressed is that we believe that it is important to recognise that the social and emotional needs of children, teenagers and adults are age-appropriate. Their status in the community and their role in life is mainly determined by their chronological age. Moving through school to college, work and independent living will happen on the basis of their chronological age.

Therefore the goals for education and social development need to be considered within this framework, and take account of the child’s place in society, based on their age. In the next section, the developmental achievements for typically developing children from 5 - 11 years are discussed to provide a baseline for setting targets for children with Down syndrome across this life stage.

I. Development from five to eleven years

What typically developing children achieve

Between 5 and 11 years, typically developing children make significant progress in all areas of their development and we have tried to illustrate the significant changes that take place in Figure 1. There is a wide range of individual differences in achievements in all these areas, with some children being more socially confident than others, some children making faster academic progress than others and some children having more difficulty with behaviour control than others.

! An overview of developmental progress from five to eleven years in typically developing children

###### Figure 1. An overview of developmental progress from five to eleven years in typically developing children

Perhaps the biggest challenge for children at the start of this age period is settling into school. Children start full-time school at different ages in different countries but most children will be spending part or all of each day in a kindergarten or school class by 5 years of age. In the UK, full-time school starts in the year a child reaches their fifth birthday and formal instruction in reading, writing and number begins in that school year (Reception class).

Childhood can be seen as a period of preparation for adult life. In preschool years, children are largely sheltered in their family world and parents determine friendships and social experiences. Joining full-time school is a big step into the wider community and it provides important opportunities for children to learn about the wider community, and to mix with a wider variety of children and adults. Children’s strengths and weaknesses will become more apparent as they leave the support of their families and have to cope in these larger communities. If children are going to have some difficulties socially, emotionally or academically, these will become apparent when they meet the challenges of the typical school environment.

Going to school - social and academic demands

The classroom environment places demands on children’s social skills as well as their language and learning abilities.

The children are part of a large group of children in the classroom, and will have to be able to cope socially with a range of different children. They have to communicate with one another in the classroom and the playground. They are beginning to learn about how to get on with others and how to make and keep friends. These are very important skills for teenage and adult life. They will meet children whose behaviour and attitudes are different from their own as they experience a wide range of children in their community from different backgrounds and social circumstances. These experiences are very important for helping children to develop their social skills.

During the preschool years, children will have had quite a high level of individual support for learning in their families but in the full-time school classroom they have to be able to be part of a large group and to follow the teacher’s instructions to the whole group. They may also have many opportunities for small group work and activities, but they will not be able to have much one-to-one support for their learning in most typical school classrooms. In their first years in the school system, children are expected to learn to read, write and count, and some will find this easier than others. The abilities and the aptitudes of children will begin to become apparent to their parents, teachers and themselves. Children will also develop their learning skills and become increasingly independent learners.

Speech and language skills

The school environment places a heavy demand on children’s speech, language and communication abilities. Most of the classroom and school instruction is through spoken language. When typically developing 5 year olds enter school they do not all have the same spoken language skills. Some children will be much more competent talkers than others, with larger vocabularies and more advanced grammar and some will be more able listeners than others. Some children will have difficulty listening to and remembering all the spoken instructions and information given by their teachers. Studies show that children with delayed speech and language for their age have difficulty in the classroom and tend to fall behind with their academic progress. TODO: references 1 Children’s language abilities and learning abilities are also influenced by their working memory skills. Recent research has shown that children with poorer working memory skills when they enter school have difficulty keeping up with the academic work. TODO: references 2

Children’s spoken language skills develop a great deal during the period from 5 to 11 years. Most typically developing 5 year olds come to school talking in sentences. They have several thousand words in their vocabulary and they have mastered most of the grammar of their native language. They do learn some more complex and formal grammar during these school years and they learn a great deal of new vocabulary (as many as 3000 new words each year from 7 to 16 years). This progress in vocabulary and grammar has been shown to be influenced by the children’s reading progress. Children who have reading difficulties, and fall behind their peers on reading progress, do not make typical progress on language or working memory measures. Children also develop their ability to enter into conversations, to initiate conversations with other children and adults and to tell stories or describe events they have taken part in, during this period.

Academic progress

During the primary school years, typically developing children are expected to achieve at least a basic level of competence in reading, writing and mathematics. Most 5 year olds are beginners, usually starting school unable to read though they may know some sight words and some letter names and sounds, and they may be able to write their name and to count to 20. Most children make steady progress over the next six years and by the age of 11 years can write and spell well enough to record classroom work and to write a short story. Most will be able to read books for information and for pleasure. Most will be able to calculate using numbers at least to one thousand, tell the time and calculate money accurately. Most children will be able to weigh and measure adequately for everyday applications. Some typically developing children will be more advanced than this, but a significant number of typically developing children (20-25%) will not have achieved these levels by 11 years of age. TODO: references 3

Children will be studying a wide range of subjects on the school curriculum such as science, geography and history but their literacy skills will influence their progress across all aspects of the curriculum. Children will also be enjoying music and creative arts, and these areas, along with sport and music, will not be as influenced by success with literacy. Musical and sporting abilities will be developing and some children will discover that they have particular abilities in these areas or in creative arts, including painting, dance and drama.

Out of school - social and practical independence

In the years from 5 to 11, children will be experiencing a wider range of leisure and community activities. They will be able to join clubs and activities for their age group and they will become more involved in family tasks such as shopping. Children will begin to establish a wider range of friendships and visit with friends at their homes to play. They will be invited to parties and outings with friends and their families.

By the end of this age period, children may be walking to school without parent support and walking to local shops or to friend’s homes. They may also be learning to use buses independently or with friends by 11 years of age. They will have watches and be able to tell the time. They will know their names and addresses and know what to do if they have a difficulty when out on their own. They will be able to use the telephone.

At home - personal independence

At home children will begin to take care of their personal needs over this period. At 5 most children will still be helped to wash, bath and choose appropriate clothes for the day’s activities and weather. By 11 years, most children will be achieving independence in self-care, able to run a bath or shower at the right temperature, learning to wash their own hair and cut their own finger and toe nails. They will choose their own clothes. They will not yet be expected to do their own laundry, although they may be expected to keep their own rooms clean and tidy.

Most 11 year olds can make simple snacks such as toast and hot drinks, using kettles, toasters, microwave ovens and possibly cookers and ovens safely for simple meals. Many parents will be beginning to leave children of this age unsupervised at home for short periods and be confident that they know what to do in the event of an emergency i.e. they can use the phone and know when to call a neighbour or phone for a doctor, ambulance, fire engine or policeman.

In summary

Children progress significantly in all areas of their development during this period from 5 to 11 years and success in these years builds their self-esteem and confidence. Conversely, difficulties in any of the areas of progress discussed can have a significant negative effect on any child’s confidence and self-esteem.

Children’s progress varies widely, even within the range considered to be ‘typical development’ and these differences will be in part explained by inherited individual differences in abilities and aptitudes but it will also be powerfully influenced by the family support and educational opportunities that they receive.

Influences on development for all children

Development is not fixed by genes at birth

Development is a process, a dynamic, transactional and social process. Most children are born with the potential to make typical developmental progress in all areas of their development, but to do so they have to be able to interact with the world, to move, explore, and to have people around them who talk to them and react to their behaviours. At each step, the child has to be able to store the information that they are gaining from the world and to be able to practise new skills. Later steps in development are built on earlier ones. A child who is placed in a barren orphanage with no toys and little human contact will not sit up, smile or talk at the usual ages. At any age, therefore, the development of a particular child, including those with Down syndrome, is the result of an interaction between that child’s biology and innate learning potential and the social and learning opportunities that they have experienced. It is not fixed in any simple way by genes at birth.

Until recently, children with Down syndrome in all countries grew up in situations of social and educational deprivation. They were not fully included in the social life of the community. In particular, they were denied the usual opportunities to play with and learn with typically developing children. They did not receive any education in most countries until the last 30 years, and then the education offered was mainly in special schools and classes, in which all the other children were disabled.

If a child with no disability only had this kind of social and educational experience, they would certainly not show typical development year on year: they would show cognitive and social delay. The progress of individuals with Down syndrome over the past 20 years, if they have had good social and educational inclusion, illustrates that the same is true for them. Recent studies in Australia, the USA and the UK document these changes. The development of children with Down syndrome is as sensitive to the quality of family, social and educational environments as any other child. At present, children with Down syndrome are progressing faster and achieving much more than they did 25 years ago. TODO: references 4, TODO: references 5, TODO: references 6

In addition, research on inclusion in education for children with Down syndrome consistently reports significant speech, language and academic gains (reading, writing and number), with these teenagers 2 to 3 years ahead of their peers in special schools in all these areas, based on typical rates of development. TODO: references 7, TODO: references 8, TODO: references 9 These differences are dramatic, since children with Down syndrome usually only make about 6 months progress during one year on these measures. They are progressing, but more slowly than typically developing children who are expected to make 12 months progress in a year. Measures of expressive language skills of the included teenagers show that their speech intelligibility has improved significantly. Eight out of ten included teenagers are intelligible to people they meet in the community, for example in shops or on buses, compared to only four out of ten teenagers in special schools. TODO: references 9

Similarly, children with Down syndrome who have been fully included in social activities with typically developing peers have more age-appropriate and socially mature behaviour than those who have only had social experience with peers with disabilities, at school and in the community.

Development is a dynamic, interactive and social process

Further, within a typical social and educational environment, there is much evidence that development is influenced by the sensitivity and responsiveness of parents and teachers, and by the quality and range of the learning opportunities available to children. While parents and carers may not always be aware that they are teaching, they are often engaged in explaining the world to their children as they talk or read to them and scaffolding their learning as they play. Scaffolding means supporting a child to succeed at a task that they cannot yet complete on their own. This is often done when helping children to find out what a new toy or computer game can do, helping them to complete a jigsaw or to count, for example. The adult does not take over, they join in with the child, just supporting and demonstrating as necessary when the child is not sure what to do next, so that the child is able to see how to succeed. Therefore, learning is socially mediated - that is, children learn in social interactions with more competent others in their world, such as parents, brothers and sisters, grandparents, friends and teachers. A child with a learning disability will need more support and scaffolding for their learning and development than a typically developing child.

Curiosity, motivation and self-esteem

Children’s learning is also influenced by their curiosity and motivation. Children are usually active explorers of their world from the first months of life and in their play, they seek to find out what toys can do and explore imaginary roles.

As they develop spoken language, children learn by asking as well as investigating. Children’s learning is influenced by their self-confidence and self-esteem. Children who are confident explore and learn faster than those who lack confidence. Confidence may be influenced by success but it is also influenced by children’s sense of self-esteem (self-worth), which comes from being secure, loved and valued. Children’s self-esteem influences their social and academic progress in school.

Brain and behaviour

While brain development and brain function underpins all that children do, brain development is a highly dynamic and ongoing process from birth to adult life and it is influenced by input and activity. As children learn and develop, so the brain stores that information, and brain structure and function change as new learning takes place. This means that intervention should take account of normal developmental milestones and try to ensure that a child who cannot engage in some of the age-appropriate activities independently is being assisted to experience them and helped towards achieving them with support and practice. This will ensure that they are at least gaining some of the brain stimulation that would be typical for their age. It is also necessary to be very cautious when interpreting studies of brain structure and function in children and adults with Down syndrome. Any apparent abnormalities described could be the result of the extra chromosome material and the way that it has affected the brain’s development but they could also be the result of delayed and different progress in learning and mastering new skills.

Summary

For all children, including those with Down syndrome, their developmental progress at any age is influenced by their biological make-up and their opportunities to learn and develop throughout their lives. Throughout childhood, much learning is influenced by the social relationships experienced in families and by social learning and educational opportunities with other children and adults outside the family. It is also influenced by children’s self-confidence and self-esteem. Brain development is a process which continues through life and brain function and structure are influenced by learning and activity.

The development of children with Down syndrome

Children with Down syndrome make progress in all areas of development, but usually at a slower pace. Some areas of their development are usually more delayed than others, leading many researchers to now describe a specific profile of strengths and weaknesses. TODO: references 10, TODO: references 11

However, before describing this profile in more detail (see ’ A specific developmental profile‘), it is important to stress that any large group of children with Down syndrome will vary as widely in abilities, behaviour, personality characteristics, size and appearance as any group of ’typically developing’ children. Their development is influenced by their biology and by their social and learning opportunities, like all other children.

The effect of the extra chromosome on the development of babies with Down syndrome is not the same for all the infants. For example, nearly half are born with congenital heart defects but the other half have no heart abnormalities, and while some children have bowel abnormalities, most do not. It is clear that the effects on physical development vary, for reasons not yet fully understood, and it can be predicted that the effects on brain development and learning abilities also vary between children. Some of the individual differences in rates of progress are therefore due to biological differences at birth. Some children with Down syndrome will have a greater degree of disability than others, however good their family care and stimulation, their therapy and school services. It is important to stress this point, as many parents wrongly blame themselves when their child makes slow progress.

Children with Down syndrome are individuals

Children with Down syndrome are all individuals. The conventional stereotypes are inaccurate and unhelpful. In physical appearance, they tend to look like their parents and brothers and sisters, just like all other children. They do have some physical characteristics as a result of having Down syndrome but they do not all look alike. Similarly, children with Down syndrome vary widely in personality, from being extroverted, friendly and sociable to being introverted and shy. Some children are mostly calm, while others are anxious. Some children are flexible and adaptable, others find change difficult and may have a tendency to be obsessional in their behaviours. Some children are easy to manage, are happy to be co-operative and to conform at home and in school, while other children are difficult to manage and like to have their own way, or to be in control, at home and at school.

Similarly, in all areas of development, children with Down syndrome vary in their progress. Some children will be fairly slow to achieve the motor milestones of reaching, sitting and walking while others will show little motor delay, and some children with Down syndrome will have more difficulty learning to talk than others.

The reasons for these differences will be partly influenced by genetic make-up and partly influenced by the way in which parents, carers and teachers have been able to help the child to adapt to the demands of growing up. If a child with Down syndrome is more severely delayed than is typical, it is particularly important that their parents have extra help and support from services and from parent support groups.

Each child with Down syndrome is an individual and they have the same needs as any other child as well as some specific needs, and it is important that everyone concerned with a child with Down syndrome remembers this. It is helpful to know about the specific needs that are usually associated with Down syndrome and these are described in the next section.

Additional difficulties

It is also important to remember that a child with Down syndrome may have additional difficulties, like any other child. A small number of children with Down syndrome have additional medical complications, like seizures or other illnesses, which may affect their development. Similarly, a small number of children have autistic spectrum disorders, attention deficit or hyperactivity disorder. These additional difficulties affect less than 10% of children with Down syndrome but they should be recognised and treated in their own right when they do occur. These difficulties are discussed in a little more detail in the ’ Children with more severe developmental delays’ section.

Healthcare needs

Children with Down syndrome are at greater risk for some illnesses and for hearing and visual difficulties. Any child’s developmental progress will be influenced by illness or sensory difficulties, so that it is important that all healthcare issues are understood and addressed. The Health section of this module provides a guide to the healthcare needs of children with Down syndrome, explains assessments and treatments and gives references to further reading. It is perhaps worth noting that some illnesses will present with obvious symptoms but some may not. For example, a child with limited language who has an ear ache or a tummy ache may not be able to explain that they are in pain, except by being unhappy and, maybe, irritable or difficult. Therefore it is always important to consider and rule out illness if a child’s mood changes, they stop making progress or they are difficult, before assuming they are simply being unco-operative or naughty.

Achievements at 5 years of age for children with Down syndrome

Most children with Down syndrome can achieve a number of the same developmental goals at 5 years of age as other children. Most five-year-olds are walking, toilet trained, able to feed themselves and put on at least some of their own clothes.

Most 5-year-olds are able to be part of an age-appropriate group and can conform to the main social expectations in the classroom. They are able to sit at a table, listen to the story and follow the teacher’s instruction - with some needing no help to do this and others needing some support. Most children can control their own behaviour and are not anti-social. They have appropriate perception of the emotions of others, for example, they are able to understand when others are happy, sad or hurt.

Motor skills, social progress and behaviour are strengths. However, most 5-year-olds with Down syndrome will have significant delays in spoken language - typically talking in 2 or 3-word phrases, and the words may be difficult to understand. Some children will have a knowledge of the early mathematics concepts needed in the classroom, and be starting to count, despite general language delay. Some children will be reading a sight word vocabulary and know their letter names and sounds, despite having general language delay.

While many children with Down syndrome will have achieved the skills described above, there will also be many who have not yet reached these and will need help to do so in their first years in full-time school. All children move forward through the same steps in each area of development, but some go at a slower pace for a variety of reasons, some of which have already been discussed.

In the practical modules for 5-11 year olds we have tried to take account of this wide range of development and some of the targets in the 0-5 modules are repeated in the 5-11 modules to ensure that these cover the needs of most children with Down syndrome as they start school, whatever their pace of development.

Achievements during primary school years

The school curriculum for children with Down syndrome may vary according to their school placement. Children in special education classes or special schools may not receive the same level of teaching input for literacy and numeracy as the children included in mainstream classrooms. Similarly the social demands and the social learning opportunities will not be the same in special education classrooms and schools as in mainstream schools. The speech and language learning environment may also be different, if the peer group in special education also have significant speech and language delays.

However, the goals for the children with Down syndrome should be the same in all educational settings although they may be more difficult to achieve without the support of a typically developing peer group. TODO: references 6, TODO: references 7, TODO: references 8

! An overview of developmental progress from five to eleven years in children with Down syndrome

###### Figure 2. An overview of developmental progress from five to eleven years in children with Down syndrome

Social skills and behaviour

Children with Down syndrome should be expected to behave in an age-appropriate and socially acceptable way at home and at school, and they can achieve this regardless of their level of cognitive and communication skills. At 5 years of age, a child should be able to sit still and be quiet in the classroom, to follow the school and classroom rules for playtimes, lunchtimes and assembly, with some support as necessary but with the same expectations for their behaviour as for the other children. It is particularly important that children with Down syndrome are not ‘babied’ by other children or by adults. This is a very real risk because they are often small for their age and have delayed spoken language, and so seem like younger children.

Similarly at home, children with Down syndrome should be able to behave appropriately at the meal table and in all family and social situations. They should be going to bed at an age-appropriate bedtime and sleeping through the night. Their behaviour out in the community, when shopping, going to the park or in church, for example, should be acceptable. Their social behaviour with other adults and children should also be socially appropriate.

The targets for the development of social skills and behaviour set out in Figure 1 are not unrealistic targets for children with Down syndrome, as is illustrated in Figure 2.

While children with Down syndrome should be expected to behave in a chronologically age-appropriate manner and are capable of doing so, it is still not always easy to achieve these goals with all children. Practical advice on achieving age-appropriate behaviour is provided in the social development modules.

Speech and language

Speech and language skills should show considerable progress over the period from 5 to 11 years, but as Figure 2 indicates, this is the most significant area of delay for children with Down syndrome. Most 5 year olds will begin to join more words together, although still using keyword phrases, and their speech will become a little more intelligible. Most 5 year olds will be using spoken words to communicate and may be using signs as well. During the primary years, speech should become the children’s main form of communication and they will steadily learn more vocabulary and more grammar. By 11 years, some children will be talking in grammatically correct sentences, with correct word endings and all the little grammatical words in place, while others will still be using keyword sentences and some will still be using only 2, 3 or 4 words together.

Almost all children with Down syndrome will understand more than they can say during these years. This can be very frustrating and lead to their ability to understand being underestimated.

Reading, writing and number

Academic progress will be very variable but all children should receive high quality instruction to progress their reading, writing and number skills from their first year in school. Some children with Down syndrome will make rapid progress and be able to write short stories, record class work and read for pleasure at 11 years, while many others will still need full support in the classroom to do this. These children may understand the main points of the story or the lesson but may need help to put their words into sentences and to write them down. Some children will be able to calculate with numbers to 100 or beyond but some will still be mastering counting with numbers to 20.

The important point is that all children progress through the steps of learning to read, write or calculate in the same order, so each child should be making progress through the same stages, but at different rates. Some children with Down syndrome will reach the same levels of competence in reading, writing and number at 16 years or 20 years that other children with Down syndrome reached at 11 years. It is the fact of making progress that matters, however slow, and all progress should be praised and encouraged.

Motor skills

Progress in motor skills and sports will vary between individuals, according to the interests and aptitudes of the children but will also be much influenced by the opportunities available. Children who join dancing, swimming or gymnastics groups, for example, can achieve a high level of competence. Progress in fine motor skills for handwriting and using the computer keyboard for example vary, but are also influenced by practice. Some children have significantly more motor skill difficulties than others.

Personal independence

Self-help skills and personal independence should progress steadily, at home and at school, and the targets for 11 year olds in Figure 1 will be reached by some children with Down syndrome by 11 years, for example, bathing without help and making tea and toast. With appropriate support and encouragement, most children with Down syndrome will largely achieve personal independence during their teenage years.

Social independence

Independence in the community will vary. Children with Down syndrome who have been educated in fairly large mainstream schools will have had the opportunity to learn to find their way around a large site, to use the canteen for lunch (involving choices and the use of money) and they may be walking to school with friends, crossing roads or using the bus. Children in special education may be part of much smaller communities (50 to 70 children rather than 300-400 children), with less opportunity to achieve these levels of independence. Out of school, children’s opportunities will vary according to the resources of the areas in which they live. Some children may walk to a local shop or club if they are very close to home, by the time they are 11 years old, but most children with Down syndrome will become more independent in their communities during their teenage years. Most children in the 5 to 11 age range will still be fully supervised outside the home.

Achievements at 11 years

In summary, many children with Down syndrome will be on the way to independence in personal care, able to choose their own clothes appropriately for the day, wash and bathe with minimum help, make a simple snack, answer the telephone and help with household tasks, but some will still need support for daily activities. Many children will be quite socially confident in school and at clubs and only need minimal support to function in these settings. However, some children will still need a high level of support with their personal care and in social situations.

Many children will be progressing with reading, writing and counting, with some children able to write short stories and record their work in lessons while other children still need full support to do so. Some children will be calculating with numbers to 100 or beyond but some will still be learning to count with numbers to 20. Children will be progressing with their understanding of time and money at varying rates. It is possible for children to benefit from access to the full school curriculum, differentiated to their needs, whatever their rate of progress, provided that there is adequate support in schools for this to be achieved.

Most children will be enjoying music, dance, drama, art and sporting activities and developing their skills at varying rates. In these activities, the enthusiasm and creativity of teachers will have considerable influence on the way in which children with Down syndrome progress. Participation in these activities is not dependent on good speech and language skills and individuals with Down syndrome can often show considerable talent if given the opportunity. Mime and dance activities, for example, allow children to express their understanding of emotions and behaviour in a way they cannot do in daily life.

Most children with Down syndrome can behave in an age-appropriate and socially acceptable manner, at home, at school and in the community, regardless of their level of ability, if they are expected to do so. This is a very important goal, as behaviour influences all aspects of children’s lives, and the lives of their families. During childhood, difficult behaviour causes stress in the family and reduces children’s learning and social opportunities. In adult life, a person who can behave in a socially acceptable manner can participate fully in community and social activities. In our experience, less cognitively able adults with good social behaviour will be working and leading more fulfilling lives than more cognitively able individuals with poor social behaviour.

Individual differences

Parents and teachers of typically developing children have some idea about expected rates of development, which they can use to decide whether their children are making appropriate progress or whether they should be worrying about their progress. For parents and teachers of children with Down syndrome, guidelines are equally important but more difficult to find and to interpret.

In each of the detailed modules, milestone data is included if available, for example, for speech and language, counting or reading. Children with Down syndrome vary widely in their rates of progress. Some children make much slower progress than others and it is not possible to predict or explain these differences fully at the present time.

Typically developing children also show a wide range of rates of progress, due to both different genetic make-ups and to social and educational learning opportunities and the range for some 85-90% of children with Down syndrome is probably due to exactly the same factors. However, the development of approximately 10% to 15% of children with Down syndrome is being affected by additional difficulties. This group of children show the greatest developmental delays and may be significantly more disabled as adults than the majority of individuals with Down syndrome. This is illustrated in Figure 3. The most common reasons for the more severe levels of developmental delay seem to be health issues, autism and attention deficit/hyperactivity disorder.

! The spectrum of abilities in individuals with Down syndrome

###### Figure 3. The spectrum of abilities in individuals with Down syndrome

Children with more severe developmental delays

Health problems

In a study by the authors of 90 teenagers with Down syndrome TODO: references 4 it was clear that one reason for more severe developmental delay was additional health difficulties such as seizures and neurological damage. However, for some children with severe developmental delay, the reasons cannot be identified. The prevalence of medical and psychiatric disorders in people with Down syndrome varies from those which have a lower incidence to those which are relatively common and some of these are described in the Health section of this module.

Autism

It was once thought that Autistic Spectrum Disorders (ASD) were not found in individuals with Down syndrome. This is now known to be incorrect, but the incidence of ASD in people with Down syndrome remains a matter of some debate. It has been estimated to affect some 3-7% of children and adults. The reason for this uncertainty is that there have been no large scale controlled studies of this issue and, more importantly, that the diagnosis of ASD is too uncertain to produce reliable data.

The diagnosis of ASD is based on obtaining the developmental history of the individual and information about their behavioural patterns. At present there are no objective findings in ASD which are of diagnostic value. ASD is a spectrum disorder which covers a very wide range and there are many behavioural features which are associated with this group of conditions. However, many of these symptoms are non-specific in that they may be found in a large number of other disorders.

Some of the main characteristics of the condition are:-

• A lack of behaviours in which points of interest are shared with others.
• Inappropriate social behaviour which is not understood by other people.
• Abnormal interpersonal relationships, expressed as the inability to develop intimate, reciprocal communication with other people.
• Language usage which is not only delayed but abnormal in kind.
• Rigid, unimaginative style of thinking and behaviour.
• Play patterns which are repetitive, show a lack of understanding of the symbolic meaning of toys, and inability to pretend.
• Obsessional/repetitive behaviours and preoccupations.

A child displaying one of these characteristics, or even two or three, does not necessarily have autism. In fact, a number of these characteristics are very common in children with Down syndrome, reflecting slow development of speech, language and play skills or differences in temperament. For a diagnosis of autism to be appropriate, a majority of these symptoms need to be present.

Attention Deficit Hyperactivity Disorder (ADHD)

This condition is sometimes confused with ASD, especially if the child has severe learning disabilities. The level of intrinsic activity and liveliness in children varies enormously and the vast majority of children with Down syndrome are normal boisterous youngsters. However, a small number are so overactive that their entire pattern of behaviour is disorganised. They are characterised by their short attention span, impulsiveness, ‘silly’ social behaviour, clumsiness and constant movement which is little affected by environmental influences. The response of children with ADHD to the commonly used medications is interesting because they become calmer and more capable when prescribed drugs which are pharmacological stimulants.

The management of this condition consists of a combination of behaviour modification and drugs. If parents and/or carers observe behaviours in a child with Down syndrome which are similar to those described above it is important to obtain a diagnostic assessment from a specialist paediatrician or a child psychiatrist with experience of children with learning difficulties to find out if the child has additional problems.

Meeting the educational and developmental needs of children with Down syndrome

During the primary school years, children with Down syndrome usually make significant progress. They will start school and benefit from the quality of the teaching and educational environment. Their speech and language abilities and motor skills will be the areas of greatest delay and therefore, they will continue to benefit from speech and language therapy and from occupational and physiotherapy.

It is important that parents, teachers and therapists work together as a team if possible, with professionals recognising that the most important influences on any child’s development come from the family and respecting the fact that parents know their child better than anyone else. This partnership will enable children to make optimal progress and help to support families. Later in this section, specific goals have been identified for parents and for the key professionals, reflecting their different roles and expertise. Parents tend to be very knowledgeable about Down syndrome by the time their child is five years old.

This section begins with an overview of the health needs of children with Down syndrome and their specific profile of cognitive and developmental strengths and weaknesses, as an understanding of this profile is essential for planning effective teaching and therapy. This leads to a brief discussion of education research and goals for teachers, followed by goals for speech and language therapists, occupational therapists and physiotherapists. The final section discusses family perspectives and provides goals for families.

Health and sensory impairments

All developmental progress will be affected by illness or ongoing health problems. The common health issues for parents and teachers of children to be aware of are dealt with in the Health section. It is particularly important to be alert to hearing difficulties as they affect some 80% of preschoolers and even mild conductive losses can have a significant effect on the children’s development. Many school age children with Down syndrome have hearing losses and teachers need to be aware of this. Many children also have visual impairments, and clearly any hearing and visual difficulties will influence progress in the classroom. If children have hearing aids, it is important that they are properly maintained and adjusted. If children have glasses, they will need to be kept clean during the day.

A specific developmental profile

Over the last 15 to 20 years, researchers have made progress in understanding the effects of having Down syndrome on development, though there is still much more to learn in order to fully understand how to help the children. Research has identified a specific profile of developmental strengths and weaknesses. TODO: references 10, TODO: references 11 This profile is specific to children with Down syndrome, and makes their learning needs different from most other children with similar levels of cognitive abilities. However, while this profile is typically associated with Down syndrome, the degree to which any individual with Down syndrome shows this pattern will vary. It is helpful as a guide to understanding any child’s developmental needs and learning profile. He or she may have all or none of these characteristics and if they do have some, the degree to which they show any strength or weakness will need to be assessed in order to develop appropriate teaching programmes and therapy.

While children with Down syndrome experience some delays in all areas of development, the extent of the delay is not the same across all areas of development.

Social understanding and social interactive skills are a relative strength and less delayed than speech and language skills. Most children with Down syndrome make eye-contact, smile and interact by cooing and babbling from the first months of life, and show little delay in social interactive skills. They are socially sensitive and understand the non-verbal cues to emotions, such as facial expression, tones of voice and body postures, from the first year of life. By school age most children continue to show good empathy and good understanding of social behaviours, but they will not have the language abilities to explain how they feel or to negotiate social situations.

Motor skills, including reaching, sitting and walking, are delayed but the main milestones are steadily achieved and most children become mobile and independent in self-help skills, such as feeding and dressing, which require motor skills. By school age gross and fine motor skills will be delayed. This will affect taking part in games and physical education. It will also affect learning to write. The advice of an occupational therapist will be beneficial for most children. Fine and gross motor skills will steadily improve with practice, and most children can use the mouse to operate a computer at 5 years of age.

The authors encourage all parents to find activities for children in the community for sports such as swimming, gymnastics, horse-riding, dancing or football. These sporting activities contribute to health and motor skills development - and a sporting skill developed in childhood provides teenagers and adults with increased leisure activities and social opportunities.

Speech and language skills are more delayed than the children’s non-verbal understanding and reasoning abilities. This is therefore a pattern of specific language impairment. Most children with Down syndrome understand more language than they can use as a result of specific speech production difficulties. For this reason, learning to sign will help the majority of children to communicate, to show their understanding and reduce their frustration. Hearing difficulties, usually due to ‘glue ear’ are common (see ’ Hearing disorders’ section) and contribute to speech and language difficulties.

For most children with Down syndrome, the most serious delay that they experience is in learning to talk. This is not only frustrating but it has serious consequences for all other aspects of their social and cognitive development.

As children learn to talk, each new word that they learn is a new concept or piece of information about their world. Once they can string words together, speech becomes a powerful tool for learning and for communicating with everyone in their world, and it also becomes a tool for thinking, remembering and reasoning. We carry out these mental activities using silent speech in our minds. While we can also use visual imagery to imagine and recall events, reasoning with the use of language is considerably more powerful. It follows that a serious delay in learning to understand and to use language will lead to delay in all aspects of mental or cognitive development. Conversely, if we can improve the rate at which children learn language, this should benefit all areas of their social and cognitive development.

Children of 5 to 11 years should be receiving regular speech and language therapy targeting phonology and articulation work as well as vocabulary and grammar development. However, learning to talk is a daily activity and is mostly learned with parents. The speech and language modules and checklists are designed to be used by parents, ideally with the support of a therapist, but on their own if necessary.

Working memory development, particularly verbal short-term memory, seems to be specifically impaired - again not progressing as fast as would be expected for non-verbal abilities - and this has consequences for the children’s ability to learn a spoken language and to process information. All teaching in the classroom needs to take account of the children’s working memory difficulties.

Visual memory and visual processing are relative strengths, while auditory processing and auditory memory are more impaired. This means that children with Down syndrome should be thought of as visual learners and all teaching supported with visual materials.

Reading ability is often a strength from as early as two years of age, perhaps because it builds on visual memory skills, and reading activities can be used to teach spoken language from this time. During the primary school years the teaching of reading should be a daily priority, with teachers aware that reading activities can be designed to improve the children’s spoken language. Recent research studies have demonstrated that reading activities may be the most effective way to improve the spoken language of children with Down syndrome and the most effective way to improve their working memories. TODO: references 21, TODO: references 22

Number seems to be relatively more difficult for children with Down syndrome and their number skills are often delayed relative to reading skills. Current research indicates that for children with Down syndrome, their early number abilities keep up with their non-verbal mental abilities. Teaching materials should make maximum use of visual supports and materials such as Numicon. TODO: references 20

Social behaviour is a strength as children with Down syndrome are less likely to develop difficult behaviours than other children with similar levels of cognitive delay. However, children with Down syndrome are, as a group, more likely to develop difficult behaviours than non-disabled children of their age.

Most children with Down syndrome are socially sensitive and understand the non-verbal cues to emotions, such as facial expression, tones of voice and body postures, from the first year of life. They can behave appropriately in showing concern, or becoming upset. However, this sensitivity can also make the children vulnerable as they will quickly pick up on negative emotions such as anger, dislike or rejection. As they are usually not able to explain how they feel, their distress will be expressed in behaviour. Some children with Down syndrome can be quite naughty and difficult to manage at times, at home or at school, and they may use their understanding of people’s behaviour to be unco-operative and the centre of attention. It is always possible to change behaviour and to encourage and teach age appropriate behaviour. However, it is not easy to change difficult behaviours that have become habits, and most difficult behaviours can be avoided with calm routines and good control from infancy.

In order to provide detailed overviews of the research and practical advice relevant to the development of children with Down syndrome in this modular series, individual modules address social development and behaviour, and motor development, and four cognitive areas - 1) speech, language and communication, 2) reading and writing, 3) number and 4) working memory skills. This division is somewhat arbitrary and reflects the focus of the team of authors. Self-help skills are covered in social development and behaviour.

Education

It can be argued that the most important influence on a child’s progress between 5 and 11 years is their school experience and this is equally true for children with Down syndrome. Finding the right school place will usually be more difficult for parents of a child with Down syndrome than for parents of typically developing children.

Inclusive or special education

The significant benefits of inclusive schooling have been mentioned earlier in this module. The available studies all indicate very significant positive effects of inclusion in school on the spoken language development of children and teenagers and on their academic progress, particularly for reading, writing and arithmetic. TODO: references 7, TODO: references 8, TODO: references 9 The personal independence, social independence and behaviour of the included children have been reported to be either the same or better when compared with equally able peers in special education settings. These benefits result from being immersed in the typical social world and the normal spoken language world of the mainstream school as well as the increased support for learning and access to the same curriculum as the other children. The authors and their colleagues have been directly involved in the support of children with Down syndrome in mainstream schools for the past 13 years and they believe that the level of daily support for reading and writing and the access to the full curriculum, alongside non-disabled peers in the classroom, has played a significant part in the speech and language progress of the children. It is not possible to create the same learning environment in a special class or special school, however dedicated the teachers. The typically developing peer group is essential for an optimum educational environment.

At present, the social aspects of inclusion in the UK are not as good as they could be in two ways. Firstly, schools are not yet as confident in promoting social inclusion as they are in providing good access to the curriculum. Many schools have yet to recognise that the main resource that they have to support the social inclusion of children with disabilities is all the other children in school. The peer group could usually be much more actively involved in supporting other children, but this requires planning and support. All children need to have the opportunity to discuss disability issues and to be actively supported in learning how to support children with significant disabilities. North American schools tend to be further advanced on this front and therefore children with disabilities are more fully part of the school and the home communities.

Secondly, the child with a specific or significant disability may not have the benefit of a peer group of disabled children, and, in our view this peer group is important. It is good to have some opportunities to play with friends on the basis of equality of skills and understanding. Friendships with non-disabled peers are not quite the same and are often ‘helping’ in nature rather than fun and mutually supportive relationships. Identity issues become important towards the end of the primary school years and in adolescence. Successful adult adjustment, for everyone, requires a realistic appraisal of who we are and our strengths and weaknesses. It is important for children with disabilities to have friends with similar disabilities for mutual support and understanding. It is good to know that you are not the only one facing the world with your particular difficulties. It is important to know adults with similar disabilities to provide positive support and realistic role models for adult life. It is also important to be able to form close and mutually supportive friendships and partnerships and successful ones are usually based on similar abilities and interests.

These peer group issues would not exist in a fully inclusive school system, with all children with moderate or severe learning difficulties in neighbourhood schools, but at present, in the UK and in other countries, children with Down syndrome are likely to be included in mainstream schools while their learning disabled peer group are still in segregated schools or classes. Given the recorded benefits of inclusive education, everyone interested in the welfare of children with Down syndrome should be working towards ensuring that school systems become fully inclusive.

Choosing a mainstream placement will not be straightforward, as parents often have to battle for a place and then may be faced with a school situation where the teachers are not confident about meeting their child’s needs. There is still much education and training needed in most countries if teachers are to feel well equipped to teach in fully inclusive school systems. Parents of a child in a mainstream placement will also find that they have to be more involved and take more responsibility for supporting the education of their child, going to regular meetings and working closely with the teachers and Teaching Assistants. Teachers in mainstream schools will find that they need to develop a close partnership with parents when teaching a child with a disability such as Down syndrome.

Meeting the specific educational needs associated with Down syndrome in special or mainstream classes

The educational needs of children with Down syndrome are the same whether they are in mainstream or special classes. Their special educational needs should be met, taking account of priorities and adaptations to teaching methods in all classrooms. The key goals are set out in the list below and developed more fully in the specific topic modules for this age range.

Goals for teachers of 5-11 year olds with Down syndrome

• to involve the child in all aspects of school life and school routines
• to support social independence in school and the development of friendships with peers
• to support the development of play skills and inclusion with peers in break and lunchtimes
• to encourage, model and expect age-appropriate, socially acceptable behaviour at all times
• to be familiar with the research findings which demonstrate a specific cognitive profile associated with Down syndrome (see ’ A specific developmental profile’ section) and to adapt teaching methods appropriately
• to provide access to all areas of the school curriculum at a level appropriate for the individual child
• to recognise the importance of teaching reading and writing daily, to develop speech, language and working memory skills as well as literacy skills
• to have clear targets for speech and language work for each child, and identify how these can be absorbed into all aspects of the curriculum
• to facilitate independent learning and the ability to work and to learn as part of a group
• to make full use of computer aided learning, with appropriate software for individual and group work

Approaches to teaching

In addition to stressing these goals it may be useful to draw attention to teaching methods which will help children with Down syndrome and to the importance of practice and automatisation for their progress. All children with significant learning difficulties need tasks to be broken down into smaller steps, and each step mastered before attempting the next, in order to learn successfully. They may also need help with generalisation - that is, explicit demonstrations of how to apply the new skill in situations other than the one in which it was learned. In addition, as all children master new skills, practise until the skill is overlearned is very important. This leads to the skill becoming automatic (can be carried out without conscious control and effort). This will reduce the load on working memory and free up working memory capacity for other aspects of a complex task.

Structured teaching and learning

There are various ways in which children can be helped to learn and there are debates about the advantages and disadvantages of different approaches. Some observers stress that for most children, progress is by natural learning through play and everyday social experience and therefore structured teaching programmes may not be an appropriate way to help children.

However, children with learning difficulties and delayed development often do not have the skills that are needed to learn in the more natural ways - for example, the motor skills to grasp or turn parts of toys, or the spoken language skills to join in a game or a conversation - so their ability to create their own learning experiences is reduced. In addition, they may need more examples of an association (for example hearing ‘cat’ and seeing the cat) before they learn, or more practice at a task (for example drinking from a cup, posting shapes into a box) before they master it. These extra learning opportunities will only happen if they are planned. The ideal compromise is to try to provide the extra learning experiences within ordinary daily activities and play as much as possible. The authors contributing to these modules believe that, in order for children with Down syndrome to make progress as fast as they are able to, they need to experience good parenting and stimulating everyday lives and they need some special structured teaching sessions each day.

The structured teaching sessions should try to combine natural learning approaches with more structured ones. In the natural learning situations, parents, teachers, brothers and sisters and grandparents use modelling, imitation and scaffolding, to show children how to do things and all these strategies will help children with Down syndrome. In addition, structured teaching will provide simplified and repeated opportunities to learn new skills, which can then be practised and mastered when they occur in everyday contexts. The dividing line between everyday learning and structured teaching is a fine one. For example, reading books together provides an ideal opportunity to teach new words and concepts to children, and can be used deliberately to increase learning opportunities by choosing books for vocabulary teaching, or shapes, colour or number. In other words book reading is being developed more consciously as a structured learning opportunity but it is a natural one as well.

Structured teaching may be taken a step further when the task to be learned is broken down into small steps and an errorless learning approach is used. Errorless learning simply means that the teacher prompts the child at each step as necessary, so that they successfully complete the task. This allows the child to learn how to succeed by imitation and practice rather than by trial and error. The teacher waits to allow the child to do the steps that they can without help but offers support before the child makes a mistake - a rather more deliberate form of natural scaffolding of children’s learning. The advantages of errorless learning are that the child can succeed every time and be praised for this and the child is practising the correct steps to success each time. If left alone, the child may not be able to work out how to do the task and fail every time. The disadvantage of errorless learning is that the child does not learn how to solve the problem or how to experiment and try new strategies. In our view, teaching and learning should be seen as a process over time and the skill of the successful teacher is in knowing when to provide maximum help using errorless learning techniques (at the start of learning a new task), how to slowly reduce that help (when the child can complete the practised task unaided) and when to encourage some problem solving or ‘trial and error’ learning. The successful teacher will find similar tasks for the child to tackle, to generalise their learning to new situations and to ensure that the skill is practised, consolidated and understood.

Practice

Practice is an extremely important concept in children’s development. All learning involves being a novice and making mistakes, becoming successful at the task but only with thought and effort, and then mastering the task through practice until it becomes quick and easy to do successfully. At this stage the task has often become automated - that is, it can be carried out without the need to consciously think or plan the action. Practice also continues to increase the skill and efficiency with which the task can be carried out.

It is easy to see how this applies to learning and perfecting motor skills such as drinking from a cup, using a knife and fork, dressing, writing, dancing, playing a musical instrument, playing tennis or driving a car, but it also applies to mental skills such as thinking, talking, and reading. As children learn to talk, the words that they produce only approximate to the sounds of adult words. As they practice, the words become more and more quickly and accurately spoken. As children learn to read, word recognition is effortful and each word has to be thought about. As children practice - read the same words over and over - word recognition becomes quick and automatic. Therefore, as children progress year by year, the improvement that they show is affected by the amount of practice that they have in the ordinary use of the skill (as in talking) or in the teaching and learning situations in preschool or school classroom. The faster children learn at the early stages of mastering a new skill, the more practice they have over the next weeks and months. Children who learn to talk with ease and start early, chatter away everyday from 14 or 15 months of age - getting a great deal of practice to improve their speech clarity, to put their thoughts into words and to be communication partners. Children who learn to read with ease in the first months in school, read more books and get much more reading practice than children who are struggling, leading to even bigger gaps between the progress of the fast and slower learners. It is the slower learners who need more practice to succeed and the usual effect of their difficulties is that they get less practice.

Automatisation

Automatisation of skills through practice leads to them being established in the brain and easy to access. Brain imaging studies can track this progress from effortful to automatic processing and the areas of the brain controlling automatised tasks may be different from those involved when learning a new task. Automatisation is important as automatised skills become readily available to support more complex activities. For example, knowing number words by rote makes counting tasks easier, and knowing sight words well makes reading for meaning easier.

Speech and language therapy

At five years of age, most children with Down syndrome are able to use speech as their main form of communication and they should be encouraged to do so. The use of sign as a support will still be valuable but parents and teachers should be aware that sign can be used as a prompt for new words and to aid short-term memory but the focus must be on helping the child to develop clear spoken language. Too much use of signing in an unplanned way may actually delay the development of clear spoken language at this stage.

Most five year olds will be using 2 to 3 keywords joined together in their spoken communication and many single words, while understanding more vocabulary and grammar than this would indicate. Most children will still be difficult to understand and need help with developing clear speech. Ideally all children with Down syndrome should receive speech and language therapy but access to this service will vary considerably from place to place. There is a large research literature documenting the specific speech and language profile of children with Down syndrome and identifying their needs. There is also universal agreement among international experts on the speech and language therapy needs of children with Down syndrome. However, this information does not yet inform the provision of services everywhere and there is a need to encourage specialist training for speech and language therapist who work with children with Down syndrome.

Goals for speech and language therapists working with 5-11 year olds with Down syndrome

• to have up-to-date knowledge of the specific research literature on speech and language development, working memory and effective therapies for children with Down syndrome
• to understand the significance of the specific impairment in the phonological loop component of working memory for the speech and language profile associated with Down syndrome
• to understand the importance of reading work to support the development of vocabulary, grammar and speech clarity, using strengths in visual memory
• to understand the importance of auditory discrimination for speech sounds, phonics activities, phonological awareness training and speech work for improving working memory function
• to have clear targets for 4 areas of work, speech, vocabulary, grammar and communication skills and to keep detailed records of progress
• for vocabulary and grammar, to have separate targets for comprehension and for production, as comprehension in both domains is typically significantly ahead of production
• for speech work, separate targets may be needed for articulation, phonology and intelligibility (pacing, voice etc)
• to review oral-motor function, feeding, chewing and drinking patterns and advise accordingly
• all targets should be shared with parents, teachers and assistants
• children with Down syndrome should be seen at least monthly in school, targets reviewed and activities set for parents, teachers and assistants to include in their daily routines
• these activities should be modelled with the child by the speech and language therapist, if parents and assistants are expected to deliver therapy
• ideally, all children with Down syndrome of primary school age should have weekly individual or group sessions of speech and language therapy with a therapist who has specialist knowledge and the skills to address their profile of difficulties, particularly for speech and intelligibility work.

Reviews of the literature supporting these recommended goals TODO: references 12, TODO: references 13 and specialised workshops TODO: references 14 are available for speech and language therapists. Specialised accredited postgraduate training is being developed at the University of Portsmouth for speech and language therapists and for teachers to begin in autumn 2002.

Motor skills

While almost all children with Down syndrome will be walking, running and be reasonably mobile by 5 years of age, most will show delay in the control of gross motor skills and will have more difficulty learning to write, draw, paint and use scissors than other children of the same age. They will all benefit from the advice of an expert and this may be an occupational therapist, a physiotherapist or a specialist teacher for children with motor delays and difficulties.

Goals for occupational therapists and physiotherapists

• to provide programmes of activity to develop gross motor skills and spatial awareness
• to advise on adaptations and specific activities to enable children to participate in the school PE and games curriculum
• to advise parents on suitable sporting, dance or gymnastics activities and any adaptations necessary to enable their child to participate
• to advise teachers and parents on suitable seating and posture in the classroom and at home
• to provide activities and adaptations to develop fine motor skills, fine motor co-ordination and handwriting
• to review progress with independent drinking, feeding, toileting and dressing, if necessary, and advise on any adaptations needed

Family issues

There is now a growing body of research on the life of families with a member who has Down syndrome and this work is reviewed in detail in the module on the family. The research studies are largely reassuring, TODO: references 15, TODO: references 16 as they show that most families find the resources to cope with the special needs of the child with Down syndrome and still lead ordinary family lives. There is no evidence that brothers and sisters are negatively affected in the majority of families or that more marriages break down. This does not mean that there are not times of stress or difficulty, but most people do cope.

It helps to have friends, family and neighbours for emotional and practical help at times, so it is advantageous for parents to avoid social isolation. Many families report that they have found the support of other families with children with Down syndrome invaluable also, and joining parent groups a source of emotional and practical help. It also helps to have up-to-date and accurate information on the needs of the child and on the services available in the local community.

The available evidence on outcomes in adult life indicates that the most important developmental and learning experiences for a child with Down syndrome will come from being the much loved member of a happy, active family - and from doing all the things that the family does. It is, therefore, very important that parents recognise this and take care to balance the additional needs of the child with Down syndrome with the needs of themselves and the rest of the family. The therapies and the lessons are the icing on the cake, they are not the main influence on development even though they will help. They certainly should not be allowed to take time away from family life and activities.

Goals for parents of 5-11 year olds with Down syndrome

• to fully involve children in family life
• to involve children in a range of social activities in the community with typically developing peers
• to involve children in sporting activities to develop fitness, and leisure skills for teenage and adult inclusion
• to provide a stimulating speech and language environment by ensuring that children are listened to and fully involved in family talk
• to require socially acceptable and age-appropriate behaviour at home and in all social activities
• to plan, with help if necessary, to change any difficult behaviours that have developed
• to steadily encourage independence in personal care
• to encourage the development of reading, writing, number and money skills in partnership with school

In summary

In order to meet the developmental and educational needs of children with Down syndrome during their primary school years, it is important to take account of their health, social and behavioural development, their speech, language and communication needs, their educational needs and their motor skill development. In an ideal situation, this will happen as the result of parents, physicians, speech and language therapists, teachers and physical therapists sharing their knowledge and expertise and working together as a team. Many of the goals for children with Down syndrome should be age-appropriate, recognising that the children will move through life stages on the basis of chronological age and that successful inclusion in schools, communities and families, requires age-appropriate social behaviour.

Everyone involved with children with Down syndrome should have some understanding of their specific profile of strengths and weaknesses and understand the central importance of addressing their speech, language and working memory difficulties in order to advance their cognitive development. They should also be aware of the research evidence which indicates that daily involvement in reading instruction is one of the most effective ways of improving speech, language and working memory skills.

Education in fully inclusive school systems leads to significant gains in spoken language and academic skills, which will enhance the work opportunities and quality of life for the children when they reach adulthood. Children with Down syndrome should have the opportunity to have friends with Down syndrome and with other learning disabilities as well as being fully included by peers without disabilities, for the development of a positive self-identity and the opportunity to have close, reciprocal friendships based on mutual understanding and on similar abilities and interests.

II. Health

Children with Down syndrome, like typically developing children, may be affected by any type of illness. However, it is recommended that all children and adults with Down syndrome have regular health checks to detect certain disorders as soon as possible. These systematic health checks should be carried out because people with Down syndrome tend to be affected by a number of disorders more commonly than other people. Of those disorders which have a higher prevalence in people with Down syndrome there are several which are of particular importance because they are relatively easy to diagnose and treat.

These disorders are as follows:-

• Disorders of hearing
• Disorders of vision
• Infections
• Disorders of thyroid function
• Constipation and diarrhoea
• Congenital disorders of the heart
• Atlanto-axial instability
• Sleep disturbances

Hearing disorders

Children with Down syndrome tend to have a significantly higher incidence of hearing problems than other groups and almost all surveys conclude that about 80% of them will have some problem with hearing. TODO: references 17 The importance of hearing cannot be overemphasised. The vast majority of children acquire language primarily by hearing what is being said by those around them and good hearing is involved in the development of speech and language as well as socialisation. These, as well as other factors, have a profound effect upon the general intellectual development of the child. The early detection and treatment of hearing difficulties will be of great benefit to the child with Down syndrome. TODO: references 18

! The structure of the ear

###### Figure 4. The structure of the ear (diagram - not to scale)

Sounds, which are pressure waves in the air, travel along the external auditory canal and impinge upon the eardrum. This causes the eardrum to vibrate which, in turn produces movements in the bones or ossicles in the middle ear. These movements produce vibrations upon the oval window (a small membrane separating the middle ear from the inner ear) and, via the fluid in the inner ear, stimulate the hair cells of the cochlea differentially depending upon their various frequencies and pressures. It is at this stage that the information that is contained in these pressure changes is transformed (transduced) into nerve impulses which travel along the acoustic nerve via complex routes to the brainstem and brain where sound is perceived. Anything which interferes with any stage of this transfer chain will affect hearing.

The detection and diagnosis of hearing problems depends largely upon two factors:-

1. the routine screening of all young children as part of the prevailing public health program
2. the existence of a high level of suspicion on the part of carers that a hearing problem might be present.

Because of the high incidence of hearing problems in children with Down syndrome, and the fact that special testing techniques are sometimes needed, it is recommended that all of them should undergo appropriate hearing tests in a properly equipped and staffed audiology centre. The choice of which tests to use will depend upon circumstances and expert advice should be sought about this. The following descriptions are only a very short introduction to the subject.

Tympanometry (or impedance or compliance testing) is a commonly used test that records the manner in which the ear drum moves under the test conditions and this provides information about the function of the middle ear. It is a painless test but it does require a degree of co-operation from the child.

In the UK it is Government policy for all children to have their hearing evaluated shortly after birth with the Oto Acoustic Emission Test (OAE). This test is purely passive in that it does not require any active participation from the child and is, therefore, suitable for very young children. There are several versions of this test but they are all based on the detection and analysis of certain sounds which are produced by the inner ear. OAE is mainly useful for the detection of sensori-neural deafness, and other passive tests, which measure the way in which the eardrum moves, are used to detect middle ear problems.

Visual Response Audiometry, VRA, is now being used for older children. This test is one of a group of behavioural tests in which the child is conditioned to respond to sounds and is, therefore, useful for children who cannot be relied on to actively cooperate in responding to different types of sounds.

Where children are able to understand instructions to respond when they hear a sound, pure tone audiometry may be used. There are a number of versions of this technique, the principle of which is to test hearing by producing tones of known loudness and pitch in small increments. The child signals whenever he hears a specific tone and the responses are plotted on a chart - the audiogram.

The audiogram covers the range of frequencies (pitch) from 125 to 8000 Hz (cycles per second), and the range of loudness from zero to 100 dB (decibels).

The decibel is the standard unit of measurement for the intensity of sound, and is recorded on a logarithmic scale on the audiogram, separately for each ear.

A person with normal hearing will have a line from the lowest frequency tested, 125 Hz (cycles per second), to the highest frequency tested, 8000 Hz, at the level of about zero decibels (see Figure 5).

! Normal right ear audiogram

###### Figure 5. Normal right ear audiogram

Figure 6 shows the audiogram from one ear of a person with a conductive hearing loss of about 35 dB.

The common causes of hearing difficulties in Down syndrome are:- wax in the external ear canal, ‘glue ear’, infection of the middle ear (otitis media) and sensori-neural hearing loss.

! Conductive hearing loss

###### Figure 6. Conductive hearing loss

Wax in the external ear canal, particularly if it is old and hard, may interfere with hearing and should always be removed. Wax-softening eardrops should be tried in the first instance but if this procedure is not successful the wax will have to be removed by syringing or with the use of an appropriate instrument. These procedures should be performed by experienced professionals since the external ear canal is usually narrower in individuals with Down syndrome and this may make the procedure more difficult to do.

Glue Ear is one of the commonest conditions involving the ear and it has a particularly high incidence in children with Down syndrome, causing conductive hearing loss. In this condition a mucoid secretion accumulates in the middle ear and has the effect of reducing hearing levels. The situation for children with Down syndrome is different because the ‘glue’ is stickier, is less likely to drain away, and is more likely to become infected. The Eustachian tubes, which connect the middle ear to the upper part of the throat, are often not effective in allowing drainage from the middle ear.

There are three main techniques which are used in the treatment of glue ear.

The insertion of grommets in the ear drum allows the fluid to drain out of the middle ear into the external auditory canal. Grommets are tiny tubes with flanges at each end which are inserted through the eardrum and allow the glue to drain out. Tonsillectomy and/or adenoidectomy may also be helpful. This form of treatment is effective as long as the grommets remain in position in the eardrum. They have a tendency to become extruded (pushed out), remaining in place for about three to twelve months, but they are almost always helpful in allowing drainage and therefore improving hearing.

The disadvantages of extrusion can be avoided by creating an artificial perforation in the eardrum with a laser apparatus. These laser-produced perforations tend to heal within a few weeks but many clinicians feel that the procedure is very worthwhile.

Microsuction is a technique in which the fluid in the middle ear is sucked out through a thin needle inserted through the eardrum. This procedure usually has to be repeated several times but can be very effective. It has the advantage that there is no discharge into the external auditory canal to be dealt with.

The likelihood of Middle Ear Infections in children with Down syndrome is considerably greater than in any comparable group of children. The reasons for this are the special problems of glue ear and the fact that individuals with Down syndrome are more susceptible to infections of all kinds. The treatment of middle ear infections usually involves the use of antibiotics and may also necessitate one of the interventions listed above for glue ear.

There is a view among some otologists that grommets should not be used because of the narrow external auditory canal found in children with Down syndrome, because they are often extruded and because continual replacement may scar the ear drum. The authors, and others, TODO: references 17 disagree with this view because they feel that the benefits of improved hearing upon language development and upon socialisation as well as on general intellectual development and self-confidence outweigh the possible difficulties associated with grommets. If grommets are not appropriate for a particular child, hearing aids may be offered for conductive loss.

! Hearing ranges for speech sounds

###### Figure 7. Hearing ranges for speech sounds

Sensori-neural hearing loss is a poorly understood set of conditions in which the inner ear or cochlea malfunctions. The phrase is sometimes used to include problems in other parts of the central nervous system as well. It may be constitutional or develop in later life and the higher tones are mostly affected. This type of hearing loss may have a serious effect on understanding since it is these frequencies which give speech most of its intelligibility. This type of hearing loss is often overlooked in the early stages because these children do not always behave as if they are deaf. They respond to sounds of many different kinds but what they hear is a type of low frequency rumble containing little real information. Those who can lip-read may sometimes be able to communicate to some extent.

There is no cure in this group of conditions and those who benefit from the use of hearing aids continue to depend on them indefinitely.

The suggested program for routine audiology is firstly at about nine months of age and annually until ten years of age. After the age of ten years testing every two years is considered sufficient.

There are a number of sophisticated techniques available which test different aspects of the hearing system but it is important to remember that the only way to be sure that a child has proper sound perception is by their behavioural response to purely aural information.

Hearing Aids may be needed in a proportion of people with Down syndrome and the situation has now improved in that hearing aids are now very much more sophisticated than they used to be.

The enormous processing power of digital chips is now being used in hearing aids so that the specific requirements of children with different types of hearing loss can be compensated for much more accurately.

Getting used to wearing an aid may be a difficult problem in some cases. Children need to learn that the aid is actually helpful and a period of training may be necessary. This is often best started in a quiet room with no distracting noises so that the child can appreciate the improvement in the understanding of speech. They may then be gradually introduced into more open environments. In more difficult cases a radio-link may be helpful. This consists of a combined aid and radio receiver for the child and a transmitter-microphone for the carer. This apparatus almost totally excludes extraneous noise and allows the child to appreciate the use of aiding more easily. It is obviously very important not to give up in the face of difficulties, but to be persistent in attaining success.

Although hearing losses of up to 25 dB are usually not considered to be serious in typically developing children, there is evidence to suggest that even mild hearing loss has a deleterious effect on educational, emotional and language development in children who have no disorders other than hearing loss. see 18

These data lend powerful support to the view that mild hearing loss is likely to have an even greater effect on children with Down syndrome.

Signing is almost always helpful in the particular situation where language development is being impeded by hearing difficulties. The advantage of this strategy is that language development can progress even in the presence of hearing difficulties.

Disorders of vision

There are a number of eye disorders which are of special relevance in Down syndrome but, apart from infections of the eyelid and conjunctiva, the commonest and most important are disorders which distort the image upon the retina. The majority of these are errors of refraction, i.e. short and long sightedness and astigmatism.

Because these errors of refraction are so common in individuals with Down syndrome it is important to have a high index of suspicion that they may be present. It is probable that children with Down syndrome rely on visual information to a relatively larger extent than other children. If this is so it means that vision is, relatively, even more important than it would normally be.

Testing of visual function should be carried out routinely every year in all children with Down syndrome until the age of ten years and every two years afterwards and as soon as possible if there is any clue in the behaviour of the child which could be accounted for by deteriorating vision. It is possible to test vision in virtually all children, even those who are very young and/or who cannot speak.

Fortunately almost all errors of refraction are treatable with the use of spectacles. New developments in lens manufacture have made lightweight plastic lenses available, and for those whose who have a very flat nasal bridge, double bridge pieces can be very helpful. Elasticised bands which attach to the ear pieces, such as are used by sportsmen can be useful in preventing spectacles from falling off. It is important that glasses be kept clean at all times, so that the child is always aware that they really do make the world around them easier to see.

A spare pair of glasses should always be available - it is not worth the disruption of waiting for new pair if the current one is lost or damaged.

The employment of behavioural techniques may be necessary if the child refuses to wear the spectacles. In any event it is useful to create situations where the child realises that vision is improved with use of spectacles.

Disorders of thyroid function

The thyroid gland is a shield-shaped gland situated at the base of the neck. It produces three main hormones; thyroxine, triiodothyronine and calcitonin. Disorders of thyroid function (excluding calcitonin, which will not be dealt with here) are amongst the commonest of all endocrine disorders. In the United Kingdom as a whole the prevalence of hypothyroidism is 1.4% in females and 0.1% in males. The prevalence in individuals with Down syndrome, however, is considerably higher. Thyroxine (T4) and triiodothyronine (T3) control many aspects of development and metabolism and are essential for proper development and function.

Of the many types of thyroid disorder which may occur, much the most frequent is hypothyroidism - underactivity of thyroid function - and the prevalence of this condition in people with Down syndrome is considerably higher than in the population as a whole.

The main clinical features of hypothyroidism (under-functioning of the thyroid gland), are slowness, both physical and mental, dry hair, thickening of the skin, deepening of the voice and weight gain. In addition the following symptoms are also found; intolerance of cold, slow pulse, constipation, slowed growth velocity, deteriorating performance at school, delayed or absent puberty, and a large variety of mental problems.

These signs and symptoms vary a great deal between individuals both in degree and in the particular combinations expressed. The situation is complicated by the fact that the signs of hypothyroidism, which are often insidious in their onset, are frequently attributed to Down syndrome itself.

The clinical features of hypothyroidism on their own are not sufficient to make the diagnosis; this can only be made with certainty by the laboratory measurement of thyroid stimulating hormone (TSH) and the thyroid hormones.

TSH is a hormone which is produced by the pituitary gland and which stimulates the thyroid gland to produce T4 and T3. This hormone is usually used as an index of thyroid function because its concentration increases when the level of T4 and T3 falls. However, because the level of TSH may be sometimes higher than normal in the presence of normal thyroid function in people with Down syndrome, it is considered advisable to measure T4, T3 as well as TSH whenever thyroid function tests (TFTs) are performed. Because of the great importance of not missing a diagnosis of hypothyroidism all screening protocols advise performing TFTs periodically throughout life. The recommended intervals vary from once every five years to annual testing, the most popular suggestion being two-yearly testing. Even if the symptoms of a disorder can be explained in other ways, it is still useful to perform TFTs as part of the general investigation of the problem because of its relative commonness and the possibility of multiple diagnosis.

Treatment

Once the diagnosis of hypothyroidism has been made, the treatment consists of the administration of tablets containing T4 by mouth. The thyroxine contained in these tablets is identical to the thyroxine produced by the human thyroid gland and if the dosage is properly monitored should not produce problems.

After the initiation of treatment, follow-up visits should be held at three-monthly intervals until the appropriate dose has been established and every six to twelve months thereafter. Growth and weight should be measured at regular intervals and some estimate of cognitive progress made. It is also useful to try to obtain some idea of the general state of well being of the patient.

As children grow and their body weight increases the need for T4 will increase and it will be necessary to adjust the dose guided by the results of the TFTs.

Rarely it is found that the treatment is stopped, either because it is felt that the patient is ‘cured’ or for other reasons. It is, therefore, important to impress upon all those involved that the treatment of hypothyroidism is lifelong, that proper monitoring is necessary and supplies of T4 are always kept available.

The treatment of hypothyroidism is not difficult, is cheap, has no side effects and, because it is perfect replacement therapy, produces ideal results.

Infection in people with Down syndrome

The increased incidence of infections in people with Down syndrome is very well documented. Until the nineteen fifties it was the leading case of morbidity and mortality in Down syndrome.

Respiratory infections are particularly common, especially during the first five years of life, and infections of the skin and the bladder are also common.

The great increase in longevity in people with Down syndrome is primarily due to modern methods of treating infection. There is evidence that people with Down syndrome have this increased susceptibility to infection because their immune systems have some abnormalities. Fortunately this does not mean that they do not respond to immunisation procedures or antibiotics, but it does mean that they are prone to more frequent infections than control groups and that they are sometimes more difficult to treat.

The implications of this increased susceptibility is that antibiotics tend to be needed more frequently in people with Down syndrome and it is likely that they will need to be used earlier in the course of an infection as well.

Immunisations ought to be carried out in the normal way but it is sometimes necessary to ensure that the antibody response is adequate. Immunisation against hepatitis B is now commonly included with the usual list of childhood immunisations since the incidence seems to be higher in people with Down syndrome. This disease is highly infectious and a good case could be made for everyone to be immunised against it.

The presence of occult, or hidden infections should be suspected if a child with Down syndrome seems below par for no obvious reason. Common sites for such infections are in the bladder, the throat and tonsils, the teeth, the middle ear and the skin.

Generally, problems with infections tend to decrease as the person with Down syndrome grows older.

Gastro-intestinal system

People with Down syndrome are more likely to have more problems with the stomach and intestines than other comparable groups of people. One of the commonest of these is constipation. If the constipation is very serious the child should, preferably, be investigated by a paediatric gastroenterologist since there are some rather serious conditions of the gut which present in this way. If special investigations reveal no obvious reason for the constipation it may then be managed symptomatically.

Occasionally the lower part of the colon becomes greatly distended and chronically filled with faeces. This can be a difficult problem to deal with and emptying the lower colon can be difficult. The help of a physician experienced in techniques for emptying the colon may be needed.

Even if the child has a good mixed diet and has an adequate fluid intake, constipation may continue to be a problem and the management then usually consists of a combination of habit training and laxatives.

The use of laxatives should be closely monitored as to dosage and frequency since these details often determine the difference between success and failure.

Cardio-vascular system

Although incidence figures vary, it is generally accepted that about 50% of babies born with Down syndrome will have a disorder of the heart. These congenital cardiac disorders vary enormously in type and severity. Many of them are relatively mild and do not need surgical intervention, some are fairly easy to deal with, while a proportion are serious and necessitate complex surgery.

Because of the high incidence of congenital cardiac defects most paediatric departments have screening programs for newborn children with Down syndrome. The use of ultrasound in screening programs has made the detection of cardiac abnormalities easier and has had the effect of allowing earlier and more effective treatment.

The diagnosis and treatment of cardiac disorders is highly specialised and is the province of the paediatric cardiologist and paediatric cardiac surgeon.

Detailed information on the different types of cardiac disorders and their treatment as well as support are available from the Down’s Heart Group.

Atlanto-axial instability

Many parents and carers are advised not to allow children with Down syndrome to engage in certain sports such as trampolining and forward rolling. This advice is based on the view that people with Down syndrome are more likely to have difficulties in the top part of the spinal column.

Some understanding of the anatomy of the area may help to clarify some of the issues (see Figure 8 and Figure 9).

! Front view of cervical vertebrae 1 and 2, the odontoid process and the spinal cord

###### Figure 8. Front view of cervical vertebrae 1 and 2, the odontoid process and the spinal cord (diagram - not to scale)

The first vertebra of the spinal column is called the ‘atlas’ or C1 (1st cervical vertebra). It is a roughly circular bone with two areas on its upper surface which support the skull and the atlas, in turn, rests upon the second neck vertebra which is called the ‘axis’ or C2.

The axis has a projection, called the ‘odontoid process’, which projects upwards inside the circle of the atlas. This bony ring, therefore, contains, among other things, the odontoid process and the upper part of the spinal cord behind it.

If the anatomy of this area is altered so that the odontoid process is pushed backward it could then press upon the spinal cord and damage it.

! Side view of cervical vertebrae 1 and 2, the odontoid process and the spinal cord

###### Figure 9. Side view of cervical vertebrae 1 and 2, the odontoid process and the spinal cord (diagram - not to scale)

This occurrence is very rare in people with Down syndrome and the mechanisms are still not properly understood but, at present, some organisations such as the Special Olympic Committee require that people with Down syndrome have been tested for evidence of ‘atlanto-axial instability’ (a-a instability) as a condition for being accepted for the Special Olympics.

The test for ‘a-a instability’ consists of taking x-rays of the neck in several different positions and measuring the distances between various parts of the vertebrae and the spinal cord.

In 1986 the Department of Health of the United Kingdom recommended that people with Down syndrome should have their necks x-rayed before engaging in vigorous sporting activity.

However it later became evident that x-ray examination for ‘a-a instability’ was not a reliable way of predicting whether there was an increased likelihood of spinal damage in those people who were diagnosed as having ‘a-a instability’ and the original recommendations of the Department of Health that these x-rays should be carried out were withdrawn in 1995.

‘Atlanto-axial instability’ remains a controversial issue and although damage to the spinal cord is rare, it is important to point out that when such damage does occur it seldom does so without warning.

The signs of upper spinal cord compression usually start with weakness, new difficulties in walking, not lifting the feet properly and unsteadiness. Pain or discomfort in the neck may occur and sometimes the neck may be held in unusual positions.

Bladder and bowel function may be affected and problems with hand and arm function may be later signs.

When possible warning signs do appear it is essential that competent medical intervention is sought as soon as possible in order to establish a diagnosis and institute appropriate treatment.

It is sometimes suggested that a supportive neck collar be worn, especially by people with Down syndrome, if they have pain or discomfort in the neck. This may do more harm than good because supporting the head in this way relieves the neck muscles of the normal exercise they continually perform with the result that they become weak.

Virtually all joints need properly functioning musculature to ensure that they function well and it is, therefore, important that all muscles should maintain their tone and strength with adequate exercise.

The view of many Down syndrome medical advice groups is that there is no good evidence that any form of exercise carries an additional risk for people with Down syndrome.

Sleep and sleep disturbance

Sleep disturbances are common in children with Down syndrome and parents need to be alert to the different types of sleep difficulties, as some are of physical origin and some are behavioural. TODO: references 19 Most children with Down syndrome seem to be restless sleepers and move all over the cot or bed. The significance of this is not known. However, some children have restless or disturbed sleep as a result of obstructed or partially obstructed airways. Enlarged tonsils, adenoids and tongues may contribute to obstruction. If a child is a noisy breather, and restless or waking frequently, it is wise to get an assessment. Sleep apnoea - short periods when breathing temporarily stops - occurs for a minority of children. The whole area of breathing related sleep disturbance needs more research. There is the possibility that it affects development and daytime behaviour.

Children may have disturbed sleep for behavioural reasons. For example, reluctance to go to bed, night waking and early waking can be habits, and they will respond to behavioural management strategies.

It is important to resolve sleep difficulties as poor sleep has a debilitating effect on the child and the whole family.

Monitoring developmental progress

A number of Down syndrome medical interest groups as well as other organisations provide information and record charts which are particularly helpful in monitoring the health of children with Down syndrome.

These include charts illustrating the changes in height and weight with age, developmental milestones and record charts for health checks and their results. These charts help to ensure that health checks are carried out at the appropriate times. These pages are meant to be added to the existing records in the child’s Personal Child Health Record Book (PCHR).

In the UK these can be obtained from: Down’s Syndrome Medical Interest Group, Children’s Centre, City Hospital Campus, Hucknall Road, Nottingham, NG5 1PB. website: http://www.dsmig.org.uk

Excellent information, and videos on heart defects, can be obtained from: Down’s Heart Group, 17 Cantilupe Close, Eaton Bray, Dunstable, Bedfordshire, LU6 2EA. website: http://www.downs-heart.downsnet.org/

UK Guidelines for Paediatricians can be obtained from: Marder, E. and Dennis, J. (1997). Medical management of children with Down’s syndrome. Current Paediatrics, 7, 1-7.

USA Healthcare Guidelines for Individuals with Down Syndrome, edited by William I. Cohen, can be found in: Down Syndrome: A Promising Future Together, eds. Hassold, T. J. and Patterson, D. (1998). Wiley-Liss, with a 1999 Revision in Down Syndrome Quarterly, 4 (3), 1-16, available at: http://www.denison.edu/collaborations/dsq/health99.html

The most informative website providing information on health and medical issues is that provided by Len Leshin, a paediatrician and father of a child with Down syndrome, at: http://www.ds-health.com/

A detailed Down Syndrome Issues and Information series of Health modules to inform doctors and healthcare professionals as well as parents, will be available during 2003/4.

Acknowledgements

The authors would like to thank all the children, families and practitioners that they have been privileged to work with over many years. We hope that all we have learned from them is reflected in our writing. However, the opinions expressed and any errors remain the sole responsibility of the authors.

Terminology

The term ‘learning difficulty’ is used throughout this module as it is the term currently in common use in the United Kingdom. The terms ‘mental retardation’, ‘intellectual impairment’, and ‘developmental disability’ are equivalent terms, used in other parts of the world.

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