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ADULTS WITH WILLIAMS SYNDROMEGUIDELINES FOR FAMILIES & PROFESSIONALSbyDr. Orlee Udwin, Dr. Patricia Howlin and Mark Davies
Williams Syndrome (WS) is a rare condition, occurring in approximately 1 in 20,000 live births. As a result, most people will not have encountered an individual with WS before, and they will understandably know little, if anything, about the characteristics and difficulties associated with this condition. The purpose of this booklet is to provide information about this rare disorder, and to outline the difficulties that may be experienced by adults with WS. Of course every individual is different, and not everyone with WS shows all of the characteristics and difficulties described below. The contents of this booklet are based on research undertaken over the last few years, and, in particular, on a study of the daily lives and adjustment difficulties of 70 adults with Williams Syndrome carried out in 1994/5. This research demonstrates that WS is associated with a number of distinctive behavioural and psychological features, which differentiate individuals with this condition from other groups of adults with learning difficulties. These characteristics, and their implications for the supported management of adults with WS, are discussed in detail throughout this booklet. It is hoped that the advice and suggestions that follow will be of benefit to those who live and work with adults with WS. A separate information sheet for employers and work supervisors can be obtained from the WS Foundation, and families may choose to forward that document to potential employers and supervisors who may not have time to read this more detailed booklet. GENETIC BASIS OF WILLIAMS SYNDROME Williams Syndrome is a genetically determined intellectually disabling condition. Although most cases occur sporadically,identical twins have been reported, and there are a few accounts of parent to child transmission. A microdeletion on chromosome 7 has been identified in affected individuals, resulting in disruption to the elastin gene. Elastin is an important constituent of connective tissue in the body (including blood vessels, muscles, ligaments, skin), and reduced or abnormal elastin may explain some of the physical and medical characteristics, as well as the distinctivefacial features (see photographs), associated with WS.
PHYSICAL AND MEDICAL FEATURES OF WILLIAMS SYNDROME IN ADULTHOOD Individuals with WS are often of short stature: the average adult height is 5ft for females and 5ft 6in for males. Although many children with WS are of slight build, there is a tendency for some individuals to put on weight as they get older. Many adults also appear to age prematurely: greying hair and a coarse facial appearance are common even in the early to mid-twenties. Medical characteristics specifically associated with WS include the following: (i) Cardiac Problems: Cardiovascular abnormalities characteristic of WS include narrowing of the walls of the main blood vessel carrying blood from the heart (supravalvular aortic stenosis), and peripheral pulmonary artery stenosis. The severity of the cardiac anomalies varies from individual to individual, although it seems that more serious problems, requiring regular medication, affect only a small minority. However, it is strongly recommended that GPs are made aware of any history of cardiac problems, and that all adults with WS receive regular and routine check-ups. Carers frequently report that affected individuals have less physical vigour and tire more easily as they get older. This may be associated with the progression of congenital heart defects. (ii) Hypertension: High blood pressure is frequently reported in the medical literature on WS, although it may go undetected in affected individuals. Again it is recommended that regular and routine checks are carried out by GPs, since hypertension increases the likelihood of a stroke and of other heart problems as individuals get older. Therefore, early diagnosis and treatment are important should any problems occur. (iii) Renal Problems: Renal artery stenosis and renal hypertension are occasionally associated with WS; this should be borne in mind in cases where affected individuals are found to have hypertension. Investigation of the renal tract, for example by renal ultrasound, is advisable given the possibility of renal anomalies. Urinary tract infections and cystitis have been reported in quite a number of cases. Some adults with WS report bladder and urinary tract problems such as excessive urination and difficulty with bladder control - which can be a significant problem on long journeys to and from school, college or day centre. Many individuals urinate frequently, and unless they are clearly trying to gain attention or avoid a task, their requests for the toilet are usually valid. Difficulties with toileting during the day or at night (wetting or soiling) may persist from childhood or appear as a new problem in adulthood. These problems may be exacerbated by anxiety or excitement. Carers may wish to enlist specialist help from psychologists or other professionals in Adult Learning Difficulty Teams in order to introduce a systematic toileting programme to address these problems. (iv) Bowel and Gastrointestinal Problems: Although constipation is a major problem in WS children, this tends to improve somewhat in adulthood. Prolapse of the rectum, diverticulitis (sometimes caused by poor diet), and haemorrhoids can also be prominent. Compared to early childhood, hernias are comparatively rare in adulthood. (v) Joint Problems: Commonly found skeletal abnormalities include limited rotation of the fore-arms (radioulnar synostosis), curvature of the spine (scoliosis) and contractures. In some cases these may progressively interfere with daily activities over time, and curvature of the spine may become more prominent with age. Many adults with WS complain of aches in the limbs, and joint dislocation (particularly in the knees) sometimes occurs. (vi) Menstrual Problems: Early onset of menstruation is well documented in females with WS. As they get older, many women experience difficulties coping with the effects of the menstrual cycle. Premenstrual tension (PMT) is commonly reported, and individuals can become extremely irritable or tearful, sometimes with accompanying outbursts of anger. There is some evidence to suggest that periods may become more irregular with age, although premature onset of the menopause has not as yet been reported to be a significant feature. (vii) Hypercalcaemia: A proportion of infants with WS are found to have high levels of calcium in the blood. These levels return to normal with dietary treatment, or simply with the passage of time. High calcium is found almost exclusively in infancy, and regular checks of blood calcium later in life are unnecessary. (viii) Hypersensitivity to Sounds: Hyperacusis refers to over-sensitivity to particular sounds that would not cause discomfort in most people. In a recent survey, we found that over 90% of adults with WS show at least some hypersensitivity to sounds. These seem to fall into two categories: sudden ‘explosive’ sounds (such as fire-works, balloons bursting, thunder, objects falling, hand clapping), and mechanical or electrical sounds (such as vacuum cleaners, drills, food processors, washing machines, vehicles reversing). In some cases even people’s voices or laughter may cause individuals to become anxious or upset. The basis for hyperacusis is not clear. In many cases it appears to improve with age, but about 50% of adults continue to experience significant problems with hypersensitivity to noise. Individuals tend to cover their ears or try to avoid distressing sounds by leaving the room or switching off the radio or television. Panic attacks and aggressive outbursts have also been noted in some individuals , apparently in response to the discomfort caused by sounds. Many find noisy rooms or rowdy environments distressing, and in these cases it is advisable to reduce the level of noise in the work place or home setting as much as possible.
Once individuals reach adulthood they no longer qualify for paediatric care and check-ups. Although some continue to see the paediatrician well into their twenties, most will depend largely on their GP for medical care in adult life. Since GPs are unlikely to have encountered anyone with WS before, it is extremely important that they are fully informed about the individual’s medical history and about the medical problems generally associated with WS in adulthood. An Information and Health Surveillance pamphlet designed for GPs is available from the Williams Syndrome Foundation, which provides useful information about the potential health risks associated with the condition. It is recommended that renal function, cardiac function and blood pressure are checked routinely, at least once a year. ABILITIES OF ADULTS WITH WILLIAMS SYNDROME People with WS have learning difficulties, although these vary in severity. About half have severe learning difficulties, while the remainder have mild or moderate learning difficulties. Most also show a distinctive pattern of abilities, often with relatively good spoken language and large vocabularies, but poorer perceptual and motor skills, and concentration difficulties.
Language and Communication The language and communication skills of adults with WS are often relatively good, and quite out of keeping with their general level of learning disability. On the surface at least, most appear fluent and articulate, and have sophisticated and wide ranging vocabularies. They tend to use complex and grammatically correct sentences, incorporating long words, clichés and sophisticated phrases which they have heard other people use. However, their speech can also be inappropriate and repetitive, especially when talking about topics of particular interest to themselves. In addition, they tend to exaggerate and over-dramatize events in conversation with others. For example they may describe minor mishaps as major tragedies. In many cases their understanding of language is more limited than their expressive language would suggest, and inappropriate language may result from their use of words without fully understanding their meaning. For example, they may be able to recite instructions or directions word for word, yet have difficulty understanding the most basic concepts. In conversation, many adults lack the intellectual ability to imagine what other people may be thinking or to see somebody else’s point of view. Many find it difficult to build a conversation based on the dialogue of others; consequently their speech tends to be one-sided, which may well antagonize or irritate others. People with WS will often use frequent chatter and incessant questions to initiate and maintain social contact with other people. They are very adept at using their superior verbal skills to get attention (occasionally this might include telling the most elaborate and untrue stories to elicit the attention or sympathy of others!). It is important to encourage individuals to answer questions relevantly and to engage in appropriate conversation with people they meet; it is also important to discourage them from chatting too much, asking irrelevant or inappropriate questions or engaging in conversation with strangers.
Many adults with WS talk to themselves at times, and this can occur in public as well as in private. This behaviour is more evident when an individual is anxious or annoyed, and can also serve as a distraction in stressful situations. It is unlikely to be a symptom of psychiatric disturbance, but should be discouraged.
Perceptual and Motor Abilities Many adults with WS have particular difficulties with gross and fine motor coordination; with visual discrimination tasks, with orienting themselves and objects in space, and with judging distances, directions and depth. Even something as apparently simple as walking down stairs or on an uneven surface like grass or gravel, may prove extremely challenging. A fear of heights and unsteady surfaces is common, and crossing the road can be a particular problem, because individuals often find it difficult to judge accurately the speed and distance of cars. Difficulty with fine-motor coordination may make individuals appear clumsy or slow when performing delicate or intricate tasks, such as doing up buttons or threading a needle. Some individuals have a marked hand or arm tremor when performing such tasks. Handwriting is also affected by these difficulties.
Concentration and Attention Span Poor concentration and overactivity are common in children with WS. Overactivity tends to diminish in adolescence, but distractibility and limited attention span can remain a problem, and adults with WS often find it difficult to concentrate on tasks for long periods. Their anxiety and hypersensitivity to noise can exacerbate this distractibility.
The abilities of adults with WS vary considerably; about 50% do learn to read, at least at a basic level. Individuals tend to have relatively good verbal skills, including a very good memory for sounds and words, and excellent auditory sequencing skills. These strengths are useful for acquiring reading skills. Many individuals with WS reach their ceiling of reading ability in their mid-teens, and often do not progress beyond a reading age of 8 or 9 years; however this level provides a good basic reading vocabulary.
Writing and Spelling The majority of adults with WS attain at least some basic writing ability, such as writing simple sentences. However many find writing more difficult than reading because of the additional visual and fine motor skills required. Many people have problems holding a pen or pencil, and forming the letters, spacing out words, and aligning words. Again, only limited progress in spelling ability is found in the period from adolescence to adulthood.
SOCIAL RELATIONSHIPS Over-friendliness Almost all adults with WS find it difficult to establish and maintain friendships with people of their own age, especially as they tend to be reluctant to socialize with other people with disabilities. On the other hand, they love company, are eager to please and will often seek out people and engage them in conversation. Unfortunately, more able adults of their own age may lack patience, and quickly tire of their repetitive chatter and limited conversation. Individuals with WS may approach strangers in an over-friendly and over-familiar manner, and will often tag along with them. This can be a major worry for parents and carers, who fear that they are too trusting and could be taken advantage of if not supervised sufficiently. Individuals with WS may occasionally seek affection and physical contact in ways that would be acceptable in a child but which are inappropriate in an adult. Again, such behaviours usually illustrate poor understanding of the boundaries of different types of relationships. Whereas hugging, kissing and touching are acceptable in children, this type of over-demonstrative behaviour may be a real barrier to acceptance in the ordinary community. Understanding Other People As already noted, adults with WS often possess good verbal skills which enable them to initiate superficial social contacts. However, they often lack understanding of the underlying, ‘unwritten’ rules governing all types of social interaction. They are often too open, direct or personal in their communication with others, without recognizing the social constraints that would be apparent to other people in the same situation. In other cases their social naiveté and lack of inhibition can lead them to tell tales or to say things that might hurt or embarrass other people. Such behaviour is rarely intentional or malicious, but occurs because individuals do not understand the social implications of their utterances. Similarly they will not hesitate to try and gain other peoples’ attention with their comments or questions, or to reprimand others. They may even try and imitate the manner of their supervisor when bossing or telling somebody off! Consequently they may give the impression of being rude, bossy or attention seeking, which again may antagonize others, if they are not forewarned. Inability to fully understand the subtleties of social interactions, and a tendency to take language very literally, may result in individuals becoming easily offended or upset; even mild teasing or ‘leg-pulling’ can cause great distress. Many adults with WS believe, quite incorrectly, that they are being ‘picked on’, and are extremely sensitive to the perceived disapproval of others. Concern for the physical and emotional well-being of others can be one of the most endearing aspects of the personality of adults with WS. However, whereas they seem to know when people are upset they do not always understand why people feel that way, and some individuals are over-sensitive, believing that they have caused upset in others without understanding the true cause.
Emotional Attachments Many individuals with WS do have boyfriends or girlfriends. Opportunities to meet members of the opposite sex may come at college, work or in social situations (e.g. Gateway Club). As these situations are often supervised, the resulting relationships are frequently rather ‘superficial’, although they may bring considerable satisfaction to the individuals concerned. Occasionally adults with WS may focus their affection on ‘pop-stars’, television/film personalities or someone they know like a neighbour or carer; where such emotional attachments become intense and obsessive, they can be extremely disruptive.
Sexual Relationships As is the case for many people with learning difficulties, adults with WS are often socially vulnerable, and their friendly and trusting nature may place them at particular risk of exploitation. Sex education at an appropriate level for the individual concerned, is crucial from an early age. Some adults may display inappropriate sexual behaviours such as masturbating in public. The best way to deal with such behaviour is to teach the individual to identify when and where masturbation is appropriate (e.g. in the bedroom with the door closed) and where it is not (e.g. in public places or in the presence of others), rather than trying to stop it altogether. Teach that masturbating is not forbidden altogether, but is acceptable in private and at certain times. The Association to Aid the Sexual and Personal Relationships of People with a Disability (S.P.O.D. - address at the back of this booklet) provides advice and counselling to families or individuals with special needs who are experiencing sexual or relationship problems. The subject of contraception raises a number of problems for adults with WS. Mechanical barriers are often impractical and ineffective. Given the risks associated with congenital heart disease, which is present in many cases, the advice of a cardiologist should be sought with respect to other forms of contraception. Where the oral contraceptive is contemplated for a woman with WS a low dose oestrogen should be used. Other alternatives include contraceptives given by injection or in capsules, which are slow releasing and long lasting. Seeing friends and siblings leading independent lives, having relationships and getting married can be a source of frustration for adults with WS, who often cannot understand why they have been left behind. The desire to get married and lead an ‘ordinary life’ can lead some individuals to get involved in relationships, and even get engaged, without having a true understanding of what this involves. It is important that carers try to teach individuals about the subtleties of relationships with the opposite sex, and help them to cope with the emotional difficulties that often occur. EMOTIONAL AND BEHAVIOURAL DIFFICULTIES Adults with WS are often described as being over-anxious and easily upset by criticism or frustration, or by events that other people regard as trivial. They may worry excessively about the well-being of others, their own health or about all kinds of imagined disasters. In some cases this might manifest itself as hypochondria and exaggerating everyday aches and pains into serious illness. Anxiety may result from many different situations, but three areas in particular tend to give rise to difficulties: (i) Excessive demands: The typically good verbal abilities of adults with WS can lead others to over-estimate their general ability, and this can result in them being exposed to situations in which they are unable to cope. These can include living arrangements without adequate support, or college/work centre activities which are too demanding. Inappropriate demands can cause individuals to worry excessively and to become anxious or upset, if only because they think that they might be upsetting or disappointing their parents, carers or supervisors. There are a number of indicators that suggest when someone is not coping: they may try to get out of doing things by complaining of headaches or tiredness; they may ‘escape’ stressful situations by having tantrums or being aggressive; they may repeatedly ask for reassurance from others, or simply walk out of the room. If the excessive demands persist, adults with WS may withdraw into themselves, lose interest in food, activities and/or relationships, and become depressed. (ii) Change and Uncertainty: Individuals can become anxious when anticipating something new or different from their normal routine (even a visit to the doctor). Changes of staff or in the tasks undertaken at work can be particularly disturbing. (iii) Threat: Many individuals with WS have difficulty understanding the complexities of social interactions, and although increasing integration into mainstream society is generally a very positive trend, it is important to be aware that the outside world can also be a hostile and frightening place. Adults with WS can become extremely anxious if they feel that they are being ‘picked on’ or if someone is behaving in a confusing or threatening way. They can also be very protective of the people they care for (family, supervisors, carers) and may become anxious if they think that someone or something is threatening them in some way. Anxiety can show itself in many different ways: moodiness or apathy; tantrums; eating difficulties (e.g. excessive or fastidious eating or going off food); sleeping difficulties (e.g. restlessness or nightmares); over-sensitivity to criticism or disapproval; sudden loss of continence; picking and scratching the skin (sometimes causing bleeding) and increased stereotyped movements (e.g. rocking, thigh rubbing). Of course, individuals may get anxious for many other reasons than those described above, and anxieties may manifest themselves in many different ways. Occasionally anticipatory anxiety can develop into a phobia - a persistent and intense fear of specific objects, situations or events, leading to avoidance of the feared object or situation. Phobias can develop from anxieties about noises (e.g. fear of balloons, dentist’s drill or thunder) or upsetting experiences (e.g. phobias of needles dating from childhood visits to hospital).
Repetitive Movements (Stereotypies) Some children and adults with WS display persistent, repetitive movements (stereotypies) such as rocking or hand flapping. These behaviours are often found in people with learning difficulties, and may be particularly prominent when the individual is tired, anxious or agitated. In some cases rocking may occur when someone is engrossed in a particular activity, and it may aid concentration. However such behaviours can also limit social acceptance. Generally it is advisable to make individuals aware of their rocking or hand-flapping as soon as it begins, and to ask them to stop.
Adults with WS often display intense fascinations and preoccupations with certain objects (e.g. cars, electrical appliances, animals, tools and machinery), particular topics (e.g. disasters and violence in the news, crime, their own health and the health of others, sports, future events such as birthdays or holidays) or an intense interest in particular people (pop-stars, television personalities, film stars, or people they know - such as a particular neighbour, carer or supervisor). They may spend a great deal of time absorbed with these topics and talk about them repetitively. Such preoccupations can bore or irritate other people and often serve to isolate the individual.
Moodiness and Aggressive Behaviour Frustration or being unable to get one’s own way can sometimes lead to excessive displays of annoyance, outbursts of anger and, at times, aggressive behaviour such as hitting out. Such displays can occur at home or in public places, and can represent a major management problem for parents and carers.
INDEPENDENCE AND SELF CARE SKILLS The development of independence skills becomes increasingly important in adulthood -both for the WS individuals and for their families. Parents, carers and supervisors should provide opportunities for individuals to maximize their potential, but in a safe environment which does not place excessive demands on them. The particular characteristics associated with WS can both limit and facilitate independence. For instance, an adult may be able to communicate with others, which obviously facilitates independence, but still display an over-friendly or anxious personality, both of which limit the amount of social independence that can be safely allowed. Such patterns of relative strengths and weaknesses are characteristic of individuals with WS, and can make it difficult for parents and professionals to judge how much independence is suitable in each case. Living Arrangements In a recent survey of adults with WS aged 19 to 39 years, we found that over two thirds still lived with their parents, and one quarter lived in sheltered accommodation, group homes, residential communities (e.g. Camp Hill, Home Farm Trust) or residential colleges. Only one in twenty lived independently. These figures indicate that most adults with WS continue to need at least some supervision and support in their daily lives. Even the people who lived independently needed regular help from relatives to cope with housework, budgeting and self care. Although the choice of living arrangements is still somewhat limited, there are a number of charitable and other organizations which offer a range of different provision. Home Farm Trust provides supervised accommodation in residential communities catering for adults with learning disabilities. The MENCAP Homes Foundation offers sheltered accommodation integrated into the mainstream community (addresses at the back of this booklet). The Learning Disabilities Team at local Social Services Departments will provide information about sheltered and community based accommodation available locally. Regional MENCAP organizations are another extremely useful source of advice. Moving away from home can be a difficult as well as an exciting time for young adults with WS. Because of their over-anxious personalities and dependence on their families, they may find a change of home environment particularly worrying, and even traumatic. This does not mean that such a move should be avoided, but rather that it should be carefully planned, with support from the family to help the individual cope in the new environment. Many parents believe that it is better to help their son or daughter settle into alternative long term accommodation while they are still fit and well, rather than waiting until they are no longer able to care for them. Anthony Quinn’s "Guide to Families Wishing to Make Legal Provision for a Learning Disabled Member" (see below) provides helpful advice on planning for the future financial and legal welfare of a learning disabled son or daughter. Respite Care Respite care is short term residential care provided by local Social Services where individuals with special needs can spend a few weeks or days in a special unit or with another family. Respite care is helpful not only for giving parents and other carers well-deserved breaks at regular intervals, but also for enabling the individual to experience different environments, meet different people and gain independence skills. The standard and availability of respite care services is variable nationwide. Parents/carers can obtain information on what is available locally from social workers (Learning Disabilities Social Services), from special needs schools and colleges, and from other parents of individuals with disabilities. The National Association of Family Based Respite Care (Norah Fry Research Centre, University of Bristol, 32 Tyndalls Park Road, Bristol BS8 1PY) publishes an annual survey of family-based respite care schemes available in the UK. As individuals get older they are expected to learn a whole array of skills while moving towards independence: washing, toileting and hygiene, bathing, shaving, dealing with menstruation, dressing, cooking and kitchen skills, undertaking household chores, shopping and dealing with money, telephone skills, and travelling. Many adults with WS require help in developing these skills. They may have problems in physically carrying out the required task or in fully understanding and planning the task requirements. They may also need help in deciding when or how often a task should be performed - for example, should one have a bath or change of clothes every day?
Community Independence Adults with WS may be capable of learning to travel independently along familiar routes, or even further afield. Many parents and carers are reluctant to let individuals out alone because they are understandably worried about the possibility that the individual will wander off or approach strangers. This can be a real danger, and it is therefore important that parents and other carers start as early as possible, to teach independent travel and safety when out.
Numeracy and Coping with Money Many adults with WS find dealing with numbers particularly difficult, and this can make coping with money a particular obstacle. Individuals with WS often have a poor concept of the value of money, and budgeting skills are usually extremely limited. Any ideas of saving can easily be swamped by their interest in particular items. Likewise, many individuals are attracted by mechanical or electrical equipment, and the ‘charm’ of fruit machines can often be an expensive distraction. The caring nature of many WS adults and their naivetè can also make them vulnerable to financial exploitation. Because of this, most parents and carers tend to take charge of finances (including benefit payments), while giving the individual a daily allowance (weekly allowances are often ‘frittered away’ on the first day).
Coping with Time Problems with numbers and visuo-spatial difficulties also lead to problems understanding time. For people who have difficulty telling the time using analogue clocks, digital displays may be easier. It is also helpful to link the time on the clock to activities that take place at specific times. Many individuals also have problems with the concept of time itself, and find it extremely difficult to judge approximately how long a minute, hour or afternoon is. Often they will use events, such as lunch or home-time, to organize their day, and they can become extremely anxious if things do not happen exactly as they are supposed to. They can also become upset for other apparently ‘trivial’ reasons, such as a bus being delayed or if they think they are late for an appointment. There may be particular difficulty with the concept of future time, and inability to plan or schedule their activities is a characteristic problem. Leisure Activities Adults with WS are naturally sociable and can gain a considerable amount of enjoyment and useful experience from leisure pursuits. However they typically need help to organize their leisure activities, because they tend not to show initiative in choosing or persisting with activities themselves. Many carers find that if activities or outings are not organized by someone else, the individual will simply stay at home and engage in passive activities such as watching television or listening to music. If encouraged, however, adults do enjoy attending clubs designed for individuals with learning difficulties (e.g. Gateway or PHAB clubs), and some also belong to special interest clubs (e.g. train spotting or car clubs), which are related to their specific interests or preoccupations. In this way they can be introduced to new environments and social groups. Many individuals with WS have considerable aptitude for music, and some play instruments, such as percussion and keyboards. Even those who cannot actually play, are often interested in music, and may have a remarkable propensity for recognition of tunes and accompanying words. These abilities too can be harnessed in order to encourage leisure and club activities. Adults with WS are generally not very good at gross motor activities like ball games and other sports. However many enjoy swimming, which is a good way to encourage physical exercise. The lack of interest in sport may be attributed to the complexities of social interaction involved in group sports or to the increasing tiredness and lack of stamina reported in WS adults as they get older. A number of parents report that lack of exercise is a contributory factor to the weight gain observed in many individuals in adulthood. Information about local social and sports clubs for adults with a range of disabilities can be obtained from local colleges, training centres or from a social worker. Clubs often arrange supervised outings and other social activities, where people can meet together on a regular basis. A number of organizations (including the Williams Syndrome Foundation) arrange supervised holidays for disabled adults, and financial assistance for such holidays is often available. The question of what will happen after leaving school can be a major worry for families. Information about the various options available should be obtained well before school leaving age. Many Further Education and Technical Colleges integrate special needs courses into their prospectuses. Although the majority of families are satisfied with these further education courses, the quality can vary greatly from college to college. It is strongly recommended that parents find out as much as possible about the specific courses and colleges they are considering. Talking to the staff and the parents of students already undertaking particular courses is a good way to obtain information. Teachers in special schools are usually familiar with the quality of further education available locally, and school careers officers and social workers can also provide useful information. Residential colleges for young adults with special needs are another option. These are extremely useful for providing an introduction to living away from home, and they may also provide work placements in the local community. However Local Authorities are sometimes unwilling to fund a placement outside of their own region unless it can be proved that an ‘out of borough’ placement is more relevant to the needs of the adult than anything available locally. Careers Guidance Services will provide information about training centres and residential colleges locally and further afield. They should have a copy of a publication called ‘COPE’ published by Wiltshire Careers Guidance Service (address at the back of this booklet), which provides a comprehensive list of residential colleges and training centres catering for adults with learning and physical disabilities in Great Britain. It is generally advisable for further education courses to concentrate on helping adults with WS to acquire and improve their daily living skills and to concentrate on pre-vocational training, rather than spending a lot of time on academic skills. Young adults with WS may encounter a number of problems coping with further education courses. These include inability to cope with the demands of the course, and related problems such as punctuality, travelling and interpersonal difficulties. It is important that tutors and key-workers appreciate the pattern of strengths and deficits that characterize individuals with WS, and are aware of their particular behavioural and cognitive difficulties which may require particular support from staff (see section on Work-related Difficulties). Jobs and Sheltered Placements Most adults with WS attend Adult Training Centres or Day Centres, or remain at home with no daytime occupation. Only a minority work, and in all cases these are supervised work placements. When making decisions about training and occupations for individuals with WS, their profile of strengths and deficits needs to be fully understood. Jobs that are typically considered suitable for people with learning difficulties include routine manual tasks, such as stacking shelves, packing, or assembly line work. However, difficulties with visuo-spatial and motor skills, limited concentration span, and lack of physical stamina means that manual jobs such as these are often not suitable for people with WS. Individuals with WS also have marked difficulties coping with numbers, and jobs involving tills and dealing with money are also inappropriate. Adults with WS are usually extremely caring, and this concern for the physical and emotional well-being of others may be utilized in employment in homes for the elderly or disabled, with appropriate supervision. Helping jobs (e.g. being a ‘right-hand man’) or service jobs (e.g. dealing with and helping the public in coffee bars or libraries) are other possibilities. Jobs that relate directly to the individual’s special interests or preoccupations should also be considered. For example if the individual is very interested in cars then s/he may prefer to work in a garage, even if the actual job is in itself fairly routine or uninteresting. Parents and carers may have to do a considerable amount of ‘home-work’ in order to obtain adequate advice about employment. Information about what is available locally can be obtained from a Disability Employment Adviser (DEA), who can be contacted through larger job centres. The DEA can also help make contact with national organizations such as MENCAP Pathway Employment Service or the Shaw Trust (national addresses at the back of this booklet) - both of which strive to provide employment opportunities for adults with learning difficulties. DEAs are part of the Department of Employment’s Placing, Assessment and Counselling Teams (PACT) which can also provide advice about local schemes and organizations. Local Learning Disabilities Social Services Departments are also useful sources of advice about employment opportunities. Work Related
Difficulties and Suggestions In a recent survey of 70 adults with WS, we found that one third were currently involved in some kind of work experience. These placements included sheltered jobs, voluntary work and job placements organized by Further Education Colleges or Adult Training Centres. Only one person held an independent job on the open market. Half of those in work placements were considered by their employers to be having considerable problems in their job, and a further one third were considered to be having at least ‘some problems’. About one third of this group required ‘some extra supervision’ in the workplace, one third required ‘considerable extra supervision’ and the remainder required ‘constant supervision’. The problems typically encountered in the workplace are very similar to those faced in the home and in social settings. (i) Appropriate Demands in the Workplace: The superior speech and language abilities of adults with WS often disguise a much more limited level of intellectual functioning. Individuals often give the impression that they are more able than they actually are, and as a result, they may be placed in education and employment settings that place excessive demands on them. Equally, many individuals with WS themselves have little insight into their own limitations, and will often claim that they can carry out tasks far beyond their capability. Supervisors report that many adults with WS can only cope with simple instructions in the workplace, and some need help to understand even at the simplest level. Very few are capable of understanding ‘complex tasks’. Moreover, about half dislike changes in routine sufficiently to cause at least some disruption to their jobs. Many individuals with WS lack strength and tend to tire more easily as they get older. Activities that are physically demanding or which involve a lot of standing can be extremely tiring. If an individual is complaining of fatigue or is obviously looking tired as the day or week wears on, it may be a good idea either to lessen the physical work-load or reduce the hours worked. (ii) Adequate Supervision: Adults with WS tend to be extremely distractible and restless, and as a result most require a high level of supervision in order to concentrate on a task. Many display intense preoccupations with particular subjects or people, which can also serve to distract them from their work. Supervision is needed to ensure that tasks are completed. Without sufficient supervision, people tend to give up work they are struggling with, without asking for assistance. Some become disruptive if they are unable to cope. In many cases too, work tends to be ‘slap-dash’, and only a small number of individuals are considered to be thorough in their work. Supervisors need to provide reassurance, simple explanations and demonstrations of what is required, prompts and reminders and help when trying new tasks or adapting old ones. Supervisors should also be prepared to give people with WS emotional support and reassurance should they become anxious or upset for any reason. Often adults with WS will only take instruction from someone they recognise as a supervisor. They tend to resent being told what to do by other people, which can cause problems with workmates. Having an approachable supervisor as a source of advice, reassurance and instruction can circumvent many of these difficulties. Rewards can be given for completing tasks appropriately. Similarly, developing self-instruction is often a useful strategy for improving concentration. The individual should be reminded to keep working on the task at hand, initially by thinking aloud and later by silent instruction to him/herself. So while carrying out a task, the adult is taught to tell himself at regular intervals to "keep working" and to "concentrate on what I’m doing". Breaking down tasks into short, simple steps with cues - such as pictures or written instructions - to indicate what needs to be completed at each stage can also be useful. (iii) Reducing Anxiety: Adults with WS tend to be particularly sensitive to the perceived criticism of others, and anxiety is a prominent personality characteristic. In the workplace, anxiety may result from excessive or unrealistic demands, or from uncertainty about the nature of the work. Individuals find it particularly difficult to adjust to changes in the way work is undertaken, as well as changes in personnel. Noisy and busy work environments may also result in increased distress due to their hypersensitivity to sound. Anxiety may manifest itself in the individual seeking constant reassurance from work mates and supervisors, increased stereotyped movements (eg. rocking, thigh rubbing, and hand rubbing), withdrawn behaviour or irritability and moodiness. Exposure to situations that are stressful or over which individuals have little or no control can lead to tantrums and aggressive outbursts. Fortunately displays of anger and violent outbursts are rare, but where they do occur frequently advice from a psychologist working with people with learning difficulties may be helpful. If it is felt that the individual is more nervous or anxious than usual, it will be important to examine both work and non-work environments to ensure that the demands made are not excessive. Stress and anticipatory anxiety can often be reduced by spending a short period ahead of time preparing the adult for the task or feared event, and by explaining in detail what can be expected and talking through possible outcomes. (iv) Friendly and Sociable Environment: Poor understanding of the ‘unwritten’ rules that govern social intercourse, of the boundaries of different types of relationships, and their keenness to please others, often result in adults with WS appearing to be over-friendly, inappropriately demonstrative and excessively chatty. Such behaviours can provoke irritation and even anger in colleagues and supervisors. Nevertheless most are accepted and well liked by their colleagues and supervisors. Because many adults with WS find noisy or busy environments distressing, a quiet work environment, which is as free from distractions as possible, is recommended. Individuals with WS often get particular pleasure from meeting and helping people, and often enjoy tasks such as running errands for others. Such tasks can be extremely rewarding and will also reduce boredom and distractibility. On the whole adults with WS do not work well in teams, and often resent receiving instructions or directions from others. It is generally best to give individuals a particular area of work or lists of simple tasks that are their sole responsibility. This can also enhance feelings of self-esteem. (v) Structured (but not repetitive) Work: Because of their distractibility and short attention spans, adults tend to get bored with repetitive work. Conversely they dislike change, and seem to work better when given a structured routine. More complex tasks can be coped with if individuals are provided with a written list or a sequence of pictures outlining task requirements.
Alternatives to Employment Paid employment is difficult to find, and may be beyond the capabilities of many adults with WS. Alternative daytime occupations include Day Centres and Adult Training Centres for adults with special needs, run by Local Authorities and Social Services. Adult Training Centres tend to offer more structured activities, often including pre-vocational training, whereas Day Centres offer more recreational activities. As with most services, the quality of these centres varies enormously, and parents and carers are advised to find out about the quality of the provision available locally. Information can be obtained from local Learning Disability Social Services Departments.
IMPLICATIONS FOR SIBLINGS OF ADULTS Living with a brother or sister who has WS can be rewarding and fun, but also confusing and stressful. The needs of individuals with WS can at times be overwhelming, and they often require a disproportionate amount of the family’s time and attention. As a result, brothers and sisters may get less attention from parents, grandparents and other relatives, and they may quite understandably feel resentful or angry. Parents need to try and ensure that they give their other children as much individual time and attention as possible. Siblings may feel embarrassed or resentful when, for example, they have to explain to their friends about their disabled brother or sister, or when having to keep an eye on him or her. At other times they may feel guilty that they themselves are not disabled. Typically they also feel a great sense of responsibility towards their brother or sister who has WS, and as they become adults they may worry about the future and who will look after their disabled sibling once their parents are no longer able to do so. Realistic planning for the future can help alleviate many of these justifiable concerns. Many siblings are poorly informed about WS, and as a result may harbour unnecessary worries about the possibility that they may be at risk of having a child with WS themselves. It is important for parents to talk with their unaffected children about WS, and to give them information about the cause of the condition, the negligible risk of recurrence in families, and its physical and behavioural characteristics. Siblings may also find it helpful to talk with the WS child or adult’s paediatrician, cardiologist or clinical geneticist. Siblings have as much need for information as do parents, and accurate information will serve to allay many of their worries and fears about the condition.
USEFUL ADDRESSES AND FURTHER READING: General: Mencap (Royal Society for Mentally Handicapped
Children and Adults) Provides information about residential homes and other accommodation for adults with disabilities. Also provides details about the national network of Gateway clubs, Pathway Employment Schemes, and legal, benefits and welfare advice.
Adult Learning Difficulties Teams - available in most NHS Trusts or NHS Community Units. They tend to be multi-disciplinary teams consisting of psychologists, occupational therapists, speech therapists, psychiatrists and community workers. In some cases, social workers are also members of such teams. These professionals can give valuable advice concerning abilities and behavioural and emotional difficulties of adults with WS.
Further Education Colleges and Training Centres: COPE (Compendium of Residential F.E. Colleges and Training Centres for Young Adults with Disabilities). 1996 Can be ordered through Mencap (address above) Published by:
Assisted Employment: The Shaw Trust: Thrale House, 44-46 Southwark Street, London SE1 1UN Similar to Pathway, in that it aims to provide sheltered employment and supported work experience for adults with disabilities. Can be contacted locally through the Disability Employment Adviser in local Job Centres, or directly by phone. Residential Accommodation: Mencap Homes Foundation (Mencap address above) Home Farm Trust Legal Provision Personal Relationships and Sexuality S.P.O.D. (Association to Aid the Sexual and Personal
Relationships of People with Disability) Provides advice on sexual and personal matters for people with physical and learning disabilities, and for their families or partners, and also runs a confidential telephone counselling line (Tel: 0171-607 8851).
Benefits and Entitlements: RADAR (Royal Association for Disability
and Rehabilitation) A campaigning and information-giving organization. Particularly useful for advice about benefits and entitlements. Disability Alliance Family Fund PO Box 50, York YO1 2ZX Produce a booklet called "After Age Sixteen, What Next?" which gives advice about changes in DSS benefits and entitlements when individuals reach adulthood. The booklet is free for parents of individuals with special needs, though professionals are charged £4.00 to obtain a copy. Independence Training Carr, J. & Collins, J. (1992) Working Towards Independence: A Practical Guide to Teaching People with Learning Disabilities. London: Jessica Kingsley Publishers. Yule, W and Carr, J (1987) Behaviour Modification for People with Mental Handicaps (2nd edition). London: Croom Helm.
Davies, M., Howlin, P. and Udwin, O. (1997) Independence and adaptive behaviour in adults with Williams Syndrome. American Journal of Medical Genetics, 70, 188-195 Davies, M., Udwin, O. and Howlin, P. (1998) A preliminary study of social, emotional and behavioural difficulties in adults with Williams syndrome. British Journal of Psychiatry. Morris, C.A., Demsey, S.A., Leonard, C.O., Dilts, C. and Blackburn, B.L. (1988) Natural History of Williams syndrome: Physical characteristics. The Journal of Paediatrics, 1988, 113, 318-326. Udwin, O (1990) A survey of adults with Williams Syndrome and Idiopathic Infantile Hypercalcaemia. Developmental Medicine and Child Neurology, 1990, 32, 129-141. Udwin, O., Davies, M. and Howlin, P. (1996) A longitudinal study of cognitive abilities and educational attainment in Williams syndrome. Developmental Medicine and Child Neurology, 38, 1020-1029 Udwin, O. and Yule, W. (1998) Williams Syndrome: Guidelines for Parents (Revised). Published by the Williams Syndrome Foundation (U.K.). Udwin, O. and Yule, W. (1998) Williams Syndrome: Guidelines for Teachers (Revised). Published by the Williams Syndrome Foundation (U.K.). Udwin, O., Yule, W. and Martin, N. (1987) Cognitive abilities and behavioural characteristics of children with Idiopathic Infantile Hypercalcaemia. Journal of Child Psychology and Psychiatry, 1987, 28, 2, 297-309. We would like to thank Sir George and Lady Cynthia Cooper and The Williams Syndrome Foundation for highlighting the need for an information booklet on adults with WS, and for funding the research upon which this booklet has been based. Alison Payne, Carol Mathurin, Cynthia and Ken Scott, Cynthia and Michael Glanville-Smith, Susan Golder, Christine Newman, Mike Adlam, Dr Mike Wolfman and Dr Neil Martin read and commented on earlier drafts of this booklet. Catherine Donnelly provided guidance concerning the vocational needs of adults with WS. We thank them all for their helpful suggestions. During the course of this research we have benefitted from insights and information provided by many parents, tutors, supervisors and key-workers of individuals with WS, and from meeting with and interviewing adults with WS themselves. We are grateful to them for what they have taught us about WS. Dr. Orlee Udwin, M.Phil., Ph.D., C
Psychol, FBPsS Dr. Patricia Howlin, M.Sc., Ph.D., C
Psychol, FBPsS and Mark Davies |
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