Showing papers in "Journal of Intellectual Disability Research in 2005"

Preschool Children with Intellectual Disability: Syndrome Specificity, Behaviour Problems, and Maternal Well-Being

Abstract: Summary Background Children with intellectual disability (ID) are at heightened risk for behaviour problems and diagnosed mental disorder. Likewise, mothers of children with ID are more stressed than mothers of typically developing children. Research on behavioural phenotypes suggests that different syndromes of ID may be associated with distinct child behavioural risks and maternal well-being risks. In the present study, maternal reports of child behaviour problems and maternal well-being were examined for syndrome-specific differences. Methods The present authors studied the early manifestation and continuity of syndrome-specific behaviour problems in 215 preschool children belonging to 5 groups (typically developing, undifferentiated developmental delays, Down syndrome, autism, cerebral palsy) as well as the relation of syndrome group to maternal well-being. Results At age 3, children with autism and cerebral palsy showed the highest levels of behaviour problems, and children with Down syndrome and typically developing children showed the lowest levels. Mothers of children with autism reported more parenting stress than all other groups. These syndrome-specific patterns of behaviour and maternal stress were stable across ages 3, 4 and 5 years, except for relative increases in behaviour problems and maternal stress in the Down syndrome and cerebral palsy groups. Child syndrome contributed to maternal stress even after accounting for differences in behaviour problems and cognitive level. Conclusions These results, although based on small syndrome groups, suggest that phenotypic expressions of behaviour problems are manifested as early as age 3. These behavioural differences were paralleled by differences in maternal stress, such that mothers of children with autism are at elevated risk for high stress. In addition, there appear to be other unexamined characteristics of these syndromes, beyond behaviour problems, which also contribute to maternal stress.

Parenting stress in mothers of children with an intellectual disability: the effects of parental cognitions in relation to child characteristics and family support

Abstract: Background Recent theories of stress and coping in parents of children with intellectual disabilities (ID) emphasize the importance of cognitive appraisals in influencing parents’ levels of stress and their adaptations to difficulties presented by the children. This study investigated the relationships between parental cognitions, child characteristics, family support and parenting stress. The aspects of cognitions studied were: parenting self-esteem (including efficacy and satisfaction) and parental locus of control. Methods The group studied consisted of  mothers of children with ID. The Vineland Adaptive Behavior Scales and Maladaptive Behavior Domain were administered by interview. Mothers also completed four questionnaires: the Family Support Scale, the Parenting Sense of Competence Scale, a shortened form of the Parental Locus of Control Scale and the Parenting Stress Index (Short Form). Results Data were analysed using Pearson’s correlation coefficients, partial correlations and a regression analysis. The results indicated that most of the variance in parenting stress was explained by parental locus of control, parenting satisfaction and child behaviour difficulties. Whilst there was also a strong correlation between family support and parenting stress, this was mediated by parental locus of control. Conclusions The results demonstrate the potential importance of parental cognitions in influencing parental stress levels. It is argued that these results have implications for clinical interventions for promoting parents’ coping strategies in managing children with ID and behavioural difficulties.

Moving from the quality of life concept to a theory.

Abstract: Background The Special Interest Quality of Life Group has updated its set of statements defining the quality of life (QOL) construct to reflect emerging areas of agreement and the framework for understanding better the QOL construct. Method This article examines the major areas currently under discussion involving the objective–subjective dichotomy, needs, and core domains. Results It is concluded that while the new statements constitute a significant advance, further progress requires testable theory. In order to facilitate such future research, a conceptual model is proposed that distinguishes causal and indicator variables within the framework of a homeostatic management system. Conclusion Several lines of empirical investigation are suggested to test this and similar theoretical models with a view to taking our conceptualization of QOL to the next level.

Quality of life and its measurement: important principles and guidelines.

Abstract: Este articulo discute los principios que deben guiar el proceso de medicion de la calidad de vida (QOL) , los principales interrogantes (quien, que, cuando, donde, por que y como), resultados y procedimientos en la variante cultural de la medicion de QOL y los usos de los datos de QOL.

Preschool children with and without developmental delay: behaviour problems, parents' optimism and well-being.

Abstract: Background Children with intellectual disability are at heightened risk for behaviour problems, and these are known to increase parenting stress. This study explored the relation of behaviour problems to less child-related domains of parent well-being (depression and marital adjustment), as well as the moderating effect of a personality trait, dispositional optimism. Method Participating children ( N =  ) were classified as developmentally delayed, borderline, or nondelayed. Mothers’ and fathers’ well-being and child behaviour problems were assessed at child ages  and  years. Results Parents of delayed and nondelayed preschoolers generally did not differ on depression or marital adjustment, but child behaviour problems were strongly related to scores on both measures. Optimism moderated this relationship, primarily for mothers. When child behaviour problems were high, mothers who were less optimistic reported lower scores on measures of well-being than did mothers who were more optimistic. Conclusions Interventions for parents that aim to enhance both parenting skills and psychological well-being should be available in preschool. It may be beneficial for such programmes to focus not only on behaviour management strategies aimed at child behaviour change, but also on parents’ belief systems, with the aim of increasing dispositional optimism.

The relationship between quality of life and self-determination: an international study.

Abstract: Background The aim of this study was to evaluate the relationship between self-determination and quality of life (QOL) of persons with intellectual disabilities (ID) living in four countries (Canada, United States, Belgium and France). Method Participants were 182 adults with mild ID living in community settings (with families, living independently or in supported living environments). QOL was measured with the Quality of Life Questionnaire. Self-determination was measured using the Adult version of The Arc's Self-Determination Scale. Discriminant function and correlational analyses were conducted. Results Discriminant function analysis indicated that essential characteristics of self-determination predicted membership in the high QOL group and that overall self-determination and QOL were significantly correlated, as were sub-scale scores. Conclusions The study replicates findings from a previous study with an international sample and confirms the importance of self-determination to enhance QOL. Subsequent research should examine the direction of the relationship between self-determination and QOL and examine the relationship of essential characteristics of self-determined behaviour and core domains of QOL in greater detail.

Conceptualizing and measuring family quality of life.

Abstract: Background Increasing emphasis on family-centred approaches to services and supports for families of children with disabilities has surfaced the issue of accountability for family outcomes. We present a review of literature about the impacts of children with disabilities on families as a backdrop to proposing family quality of life as a concept that encompasses impacts of disability and one that can be used to assess the impact of supports and services on families. Method We briefly introduce the Beach Center Family Quality of Life Scale, providing information about its factor structure, reliability and convergent validity. Results The Beach Center Family Quality of Life Scale contains 25 items assessing family ratings of importance and satisfaction with five domains: Family interaction, Parenting, Emotional well-being, Physical/material well-being and Disability-related supports. Conclusion We present a framework for utilizing a measure of family quality of life as a long-term outcome in concert with other short-term measures of service outcomes for families.

Underweight, obesity and exercise among adults with intellectual disabilities in supported accommodation in Northern England

Abstract: Background Significant deviation from normal weight (obesity and underweight) and lack of physical exercise have been identified as three of the most significant global behavioural risks to health. Methods Body mass index (BMI) and levels of physical activity were measured in a sample of  adults with intellectual disabilities (ID) receiving supported accommodation in nine geographical localities in Northern England. Comparative population data were extracted from the Health Survey for England  and . Results Men and women with ID living in supported accommodation are at increased risk of being significantly underweight and physically inactive. Women with ID living in supported accommodation are at increased risk of obesity. Within the population of people with ID living in supported accommodation increased behavioural health risks are associated with gender, severity of ID, age and location. Conclusion Significant deviation from normal weight and lack of physical exercise are significant behavioural risks to health among people with ID.

A survey of sleep problems in autism, Asperger's disorder and typically developing children.

Abstract: BACKGROUND - Sleep problems are common in typically developing (TD) children and in children with autism, however, less is known about the sleep of children with Asperger's disorder (AD). The aim of this study was to compare sleep patterns of children with autism and AD to a TD group of children. METHODS - Sixty-six parents of TD children, 53 parents of children with autism, and.. parents of children with AD completed a survey on their child's sleep patterns, the nature and severity of any sleep problems and success of any treatment attempted. RESULTS - The results showed high prevalence of sleep problems with significantly more problems reported in the autism and AD groups (TD = .50%, autism = 73%, AD = 73%), with no significant differences between groups on severity or type of sleep problem. Children with AD were significantly more likely to be sluggish and disoriented after waking and had a higher Behavioral Evaluation of Disorders of Sleep (BEDS) total score compared to the other two groups. The autism and AD groups reported significantly better treatment success for medication compared to the TD group. The autism group reported significantly better success for behavioural treatment compared to the AD group. CONCLUSIONS - In conclusion, children with AD may have more symptoms of sleep disturbance, and different types of sleep problems than children with autism. As this is the first study to compare autism and AD and to survey treatment outcomes, further research is needed to validate these findings.

Obesity in adults with Down syndrome: a case-control study.

Abstract: Background Obesity has a negative impact upon mortality and morbidity. Studies report that obesity is more prevalent in individuals with Down syndrome than individuals with intellectual disabilities (ID) not associated with Down syndrome. However, there have been no studies using a methodology of matched comparison groups and findings from previous studies are contradictory. Methods A detailed method was used to identify all adults with ID in Leicestershire. Individuals were invited to participate in a medical examination – that included measurement of their height and weight, from which body mass index (BMI) was calculated. For each person with Down syndrome, an individual matched for gender, age and accommodation type was identified, from the Leicestershire ID database. Results The data for 247 matched pairs is reported. Women with Down syndrome had lower mean height and weight, but greater mean BMI than the matched pairs. Men with Down syndrome had a lower mean height and weight but there was no statistical difference in BMI compared to the matched pairs. Using World Health Organization categories of BMI, women with Down syndrome were more likely to be overweight or obese than their matched pairs (odds ratio = 2.17). Men with Down syndrome were more likely to be in the overweight category than their matched pairs but were less likely to be obese (odds ratio = 0.85). Conclusions This study demonstrates that, compared to a matched sample, there is a greater prevalence of obesity amongst women with Down syndrome but not men. As the impact on the health of people with Down syndrome of being overweight or obese is uncertain, this is an area that requires further study.

Beating the Barriers: Recruitment of people with intellectual disability to participate in research

Abstract: Background: The health status of people with intellectual disability is poor, yet very little research has been done on their health needs. There are several barriers to performing this research. Our aim was to examine the significant barriers encountered in recruiting adults with intellectual disability to participate in research. Method: Our project was a randomised controlled trial aiming to improve health advocacy of people with intellectual disability. We approached many organisations to recruit participants and kept records of our results. Recruitment was very low for the first four months. Then we adopted two new recruitment strategies - in-service telephone recruitment and meetings with prospective participants. We then monitored the subsequent recruitment rates. We also questioned participants about the difficulties they encountered when considering recruitment. Results: Initial recruitment of participants yielded less than one-third of the expected number. Additional strategies implemented were partially successful. Significant barriers to recruitment included several sectoral issues and the challenges arising from the research process. Conclusions: Whilst this population is a difficult one to reach for research, attempts to do so should not be abandoned, since the potential health benefits for this underserved group far outweigh the recruitment barriers experienced.

The effectiveness of psychotherapeutic interventions for people with learning disabilities: a critical overview.

Abstract: Historically, people with learning disabilities have had little or no access to psychotherapeutic interventions, although there are signs that, over the past decade, this situation has seen some gradual improvement. This paper provides an overview of the evidence for the effectiveness of psychodynamic, cognitive-behavioural and cognitive therapies in this client group. The available data support the position that all three approaches can be effective in people with mild learning disabilities and in a proportion of people with more severe conditions. However, the literature reporting outcomes of psychotherapeutic interventions in people with learning disabilities is extremely limited, and there is a conspicuous and unjustified poverty of randomized controlled trials. There is also very little evidence regarding either the importance of specific components of therapeutic packages, or the optimal manner of delivering these interventions to people with learning disabilities.

Aetiology of autism: findings and questions*

Abstract: Background Although there is good evidence that autism is a multifactorial disorder, an adequate understanding of the genetic and non-genetic causes has yet to be achieved. Methods Empirical research findings and conceptual reviews are reviewed with respect to evidence on possible causal influences. Results Much the strongest evidence concerns the importance of susceptibility genes, but such genes have yet to be identified. Specific somatic conditions (such as tuberous sclerosis and the fragile X anomaly) account for a small proportion of cases. Over recent decades there has been a major rise in the rate of diagnosed autism. The main explanation for this rise is to be found in better ascertainment and a broadening of the diagnostic concept. Nevertheless, some degree of true rise cannot be firmly excluded. However, the epidemiological evidence on the main hypothesized environmental explanation, namely the measles-mumps-rubella vaccine, is consistently negative. Conclusion Progress on the elucidation of the causes of autism will be crucially dependent on the combination of epidemiology with more basic science laboratory studies.

The effect of different types of employment on quality of life.

Abstract: Background Despite research that has investigated whether the financial benefits of open employment exceed the costs, there has been scant research as to the effect sheltered and open employment have upon the quality of life of participants. The importance of this research is threefold: it investigates outcomes explicitly in terms of quality of life; the sample size is comparatively large; and it uses an established and validated questionnaire. Method One hundred and seventeen people with intellectual disability (ID) who were employed in either open or sheltered employment by disability employment agencies were interviewed. Quality of life was assessed using the Quality of Life Questionnaire. After making an initial assessment to see whether the outcomes achieved depended on type of employment, quality of life scores were analyzed controlling for participants’ level of functional work ability (assessed via the Functional Assessment Inventory). Results The results showed that participants placed in open employment reported statistically significant higher quality of life scores. When the sample was split based upon participants’ functional work ability, the type of employment had no effect on the reported quality of life for participants with a low functional work ability. However, for those participants with a high functional work ability, those in open employment reported statistically significantly higher quality of life. Conclusions The results of this study support the placement of people with ID with high functional work ability into open employment. However, a degree of caution needs to be taken in interpreting the results presented given the disparity in income levels between the two types of employment.

The early development of stereotypy and self-injury: a review of research methods.

Abstract: Background The origin and developmental course of stereotypic and self-injurious behaviour among individuals with developmental disabilities such as intellectual disability (ID) or pervasive development disorders such as autism is not well understood. Method Twelve studies designed to document the prevalence, nature, or development of stereotypic and/or self-injurious behaviour in children under 5 years of age and identified as at risk for developmental delay or disability were reviewed. Comparisons were made with similar studies with typically developing children. Results It appears that the onset of naturally occurring rhythmic motor stereotypies is delayed in young at-risk children, but that the sequencing may be similar. A very small database, differences in samples, measures, and designs limited the degree to which comparisons could be made across studies. Conclusion Future work is needed based on appropriately designed prospective comparison studies and uniform quantitative measures to provide an empirical basis for new knowledge about the early development of one of the most serious behaviour disorders afflicting children with ID and related problems of development.

Adaptive functioning and behaviour problems in relation to level of education in children and adolescents with intellectual disability.

Abstract: Background The interrelationship between adaptive functioning, behaviour problems and level of special education was studied in 186 children with IQs ranging from 61 to 70. The objective was to increase the insight into the contribution of adaptive functioning and general and autistic behaviour problems to the level of education in children with intellectual disability (ID). Methods Children from two levels of special education in the Netherlands were compared with respect to adaptive functioning [Vineland Adaptive Behavior Scales (VABS)], general behaviour problems [Child Behavior Checklist (CBCL)] and autistic behaviour problems [Autism Behavior Checklist (ABC)]. The effect of behaviour problems on adaptive functioning, and the causal relationships between behaviour problems, adaptive functioning and level of education were investigated. Results Children in schools for mild learning problems had higher VABS scores, and lower CBCL and ABC scores. The ABC had a significant effect on the total age equivalent of the VABS in schools for severe learning problems, the CBCL in schools for mild learning problems. A direct effect of the ABC and CBCL total scores on the VABS age equivalent was found, together with a direct effect of the VABS age equivalent on level of education and therefore an indirect effect of ABC and CBCL on level of education. Conclusions In the children with the highest level of mild ID, adaptive functioning seems to be the most important factor that directly influences the level of education that a child attends. Autistic and general behaviour problems directly influence the level of adaptive functioning. Especially, autistic problems seem to have such a restrictive effect on the level of adaptive functioning that children do not reach the level of education that would be expected based on IQ. Clinical implications are discussed.

Adults with autism living at home or in non-family settings: positive and negative aspects of residential status

Abstract: Background Very little is known about the context of caregiving by parents of adults with autism or about the perceived impacts of continued patterns of co-residence vs. out-of-family living. In the present study, maternal assessments of residential status, involvement with adult children living in a non-family setting, and the impacts on mothers of their residential arrangements were examined. Methods Mothers from  families of adults (aged  years and older) with autism spectrum disorder (ASD) completed questionnaires as part of a longitudinal study on family caregiving. Mothers completed open-ended questions regarding the positive and negative aspects of their child’s residential arrangement. Mothers also completed scaled questions regarding their satisfaction with their child’s residential arrangement, the different ways in which people’s lives change after a relative moves from the home, and the frequency of contact with their son or

Cross-cultural study of person-centred quality of life domains and indicators: a replication

Abstract: Background The increased use of the quality of life (QOL) concept internationally suggests the need to evaluate its etic (universal) and emic (culture-bound) properties. This study replicated and expanded a previous cross-cultural study on QOL. Method The three respondent groups (consumers, parents and professionals; total n = 781) were from four European countries: France, Belgium, Italy and Poland. The Cross Cultural Survey of Quality of Life Indicators was used to assess the importance and use of eight core dimensions of QOL. Two hypotheses were tested: (1) the etic properties would be demonstrated if there were similar profiles for the respondent and geographical groups, and if indicators grouped into the proposed QOL domains; and (2) the emic properties would be demonstrated if there were significant differences on scores across groups. Results Results supported both hypotheses. Conclusion The present study replicated the findings of a large cross-cultural study that the QOL construct has both etic and emic properties.

Empowerment in parents of school-aged children with and without developmental disabilities.

Abstract: Background Despite the widespread use of the term ‘empowerment’ in clinical literature to describe both a desirable process and the outcome of service delivery, the term remains more of a theoretical than practical construct. This study examined the factors that contribute to empowerment in parents of school-aged children with and without developmental disabilities (DD) using the Double ABCX model of family adaptation contrasted with the linear ACBX model. Methods Parents of children with (n = 100, 97% mothers) and without (n = 100, 98% mothers) DD completed questionnaires relating to child behaviour problems, parent stress and well-being, and formal and informal support. Structural equation modelling was used Results Parents of children with DD reported more child behaviour problems, more stress, less well-being and more social support than parents of children without DD. Structural equation modelling supported the ACBX model for both groups. A linear relationship was found in which parent well-being and resources mediated the relationship between the stressor (child behaviour problems) and the outcome (empowerment). Conclusions The results of the current study support Hastings and Taunt's assertion in 2002, in that empowerment was adequately explained using a traditional model of family functioning. The significant prediction offered by the parent's resources points to the need to deliver services in a manner that is more family-centred. In the education system, this means providing parents with clear messages regarding the schools goals, clarifying the parent's rights and responsibilities, including the parent in planning and decision making, respecting their knowledge as caregivers and supporting their hopes for their child.

A review of the emotional, psychiatric and behavioural responses to bereavement in people with intellectual disabilities

Abstract: Background Our knowledge and understanding of grief and its consequences have grown rapidly in recent years There is a growing understanding that bereavement and loss represent a very significant event in the lives of people with intellectual disabilities (IDs) To date, there is no systematic review of the literature on the emotional, psychiatric and behavioural responses to bereavement in people with IDs Method This comprehensive literature review firstly examines the current opinion regarding the phenomenology of pathological grief in the general population Research examining the ability of people with IDs to understand the concept of death is explored In addition, a systematic review of the literature looking at the emotional, behavioural and psychiatric responses to bereavement is carried out Results Bereavement and loss have distinct effects on the mental health, behaviour and emotional lives of people with IDs Following a bereavement, symptoms of depression and anxiety increase, and general behaviour is altered Traumatic grief symptoms have not yet been specifically studied and quantified Conclusions Particular difficulties are associated with researching the effects of bereavement on people with IDs Further work needs to be done to more accurately describe the nature, time-scale, severity and frequency of the symptoms of traumatic grief in people with IDs, in order to improve assessment and treatment of affected individuals

Health co-morbidities in ageing persons with Down syndrome and Alzheimer's dementia.

Abstract: Background Consideration of the relationship between physical and mental health co-morbidities in ageing persons with Down syndrome (DS) and Alzheimer's dementia (AD) is of clinical importance both from a care and resource perspective. Aim To investigate and measure health co-morbidities in ageing persons with Down syndrome with and without AD. Methods Recorded physical and mental health needs were ascertained for 124 persons with DS > 35 years through a systematic and detailed search of individual medical and nursing case records. Differences in persons with and without AD were investigated, by stage of dementia and by level of intellectual disability (ID). A summed score for health co-morbidities was created and compared using t-tests. Results Persons with AD had significantly higher co-morbidity scores than persons without AD (t = −8.992, d.f. = 121, P < 0.0001). There was also a significant difference in summed co-morbidity scores for persons at end-stage vs. persons at mid-stage AD (t = −6.429, d.f. = 56, P < 0.0001). No differences were found by level of ID. Conclusions Increasing health co-morbidities in persons with DS and AD have important implications for care and resources. Appropriate environmental supports combined with competent skilled staff are crucial and will have an important impact on the quality of life for this increasingly at risk population.

The development of a new measure for the assessment of psychopathology in adults with intellectual disability

Abstract: Background People with intellectual disability (ID) and untreated psychiatric disorder lead unnecessarily difficult and unhappy lives. The prevalence of mental illness in children and adults with ID is greater than that found in the general population. A carer-completed checklist of psychopathology that could be used with both children and adults would help identify those individuals with ID most likely to have a mental health problem, help ensure that they receive the limited services that are usually available and also assist the process of clinical assessment, diagnosis and management. Method This research aimed to develop a reliable and valid carer-completed checklist of psychopathology for adults with ID by redeveloping an existing measure for children with ID, the Developmental Behaviour Checklist (DBC-P). The new checklist, The Developmental Behaviour Checklist for Adults (DBC-A) was devised by changing, deleting and adding to DBC-P items. Reliability studies were conducted with paid and family carers, and DBC-A scores were compared with the results from two other measures of psychopathology. Results One DBC-P item was deleted, seven items changed and 12 items added. The psychometric properties of this new checklist, the DBC for Adults with ID (DBC-A), were investigated and found to be satisfactory. Intraclass correlations for test-retest and inter-rater reliability ranged from 0.72 to 0.85, and concurrent validity with two measures of emotional and behavioural disturbance was satisfactory. Conclusions The carer-completed DBC-A provides a broad and comprehensive survey of the emotional and behavioural problems of adults with ID. It has satisfactory psychometric properties and therefore can be used with confidence in clinical, research and service settings, and its development allows continuous assessment of psychopathology across the lifespan for all people with ID.

The effect of support group on the mental health and quality of life for mothers with autistic children

Abstract: Background The purpose of this study was to explore the effect of support groups on the mental health and quality of life for mothers with autistic children. Method A quasi-experimental pre-post control group design was used in this study. The mothers had children without chronic diseases diagnosed as autistic based on Diagnostic and Statistical Manual of Mental Disorder-Fourth Edition (DSM-IV) and were their primary caregivers. The mothers were assigned to control or intervention groups based on their willingness. The 10 weeks of the support group programme served as an intervention. There were three waves of data collection: pre-test, post-test, and after 1 month follow-up. A total of 27 mothers with autistic children were assigned into experimental and control groups. In total, there were eight mothers in the experimental group and 19 mothers served as the control group. Results The results of this study showed that mental health had not significantly improved in the intervention group compared to those in the control group at the end of the first month during follow-up. However, the subjective well-being and employment status had an effect on their mental health. Only the subjective well-being had an effect on their quality of life. Conclusion According to the results of this study, recommendations included (1) regularly promulgating a caregiver support group, (2) the advancement of training skills within the group, and (3) upgrading and promoting nurses’ ability to lead support groups in the future.

The application of quality of life.

Abstract: Background Despite its popularity, to date little systematic work has been done in the application of the quality of life (QOL) concept to persons with intellectual disability (ID) and its impact on individuals and families. This article addresses that need. Method The article summarizes the four application strands suggested by the IASSID SIRG on Quality of Life regarding the application of the QOL concept and discusses critical aspects of each. Results Examples and guidelines regarding each strand are presented, along with the ongoing need to align conceptualization, application, and research efforts and integrate QOL principles into professional education and training programmes. Conclusions The QOL concept is now challenging some of the more traditional views and approaches to ID. These challenges are resulting in modifications and adaptations in current services and supports, along with the need to evaluate the outcomes from the application of QOL principles to persons with ID.

Staff in services for people with intellectual disabilities: the impact of stress on attributions of challenging behaviour.

Abstract: Background There is a lack of a conceptual framework as to how stress and attribution variables interact and influence staff behaviour in response to challenging behaviour. To address this, a model is tested examining the impact of stress on attributions of challenging behaviour within Weiner's model of helping. Method A total of 107 staff working in community homes for people with intellectual disabilities completed a self-report questionnaire that measured stress, burnout, attributions, emotions, optimism and helping behaviour in response to challenging behaviour. Results Partial support was found for the role of attributions and emotions. However, although staff reported high stress levels and moderate burnout, this did not appear to relate to their reporting of thoughts and feelings regarding challenging behaviour predicted by Weiner's helping model. It was not possible to fully test the helping model, as the ‘help’ variable was not normally distributed. Conclusions There was little evidence to suggest that stress has a primary role in determining staff responses when examined within Weiner's model of helping. Limited support in general was offered for Weiner's helping model. Potential conceptual difficulties and clinical implications are explored and alternative models for future research are discussed.

Cognitive, emotional, physical and social effects of growth hormone treatment in adults with Prader–Willi syndrome

Abstract: Background Prader–Willi syndrome (PWS) is a multisystem genetic disorder characterized by short stature, muscular hypotonia, hyperphagia, obesity, maladaptive behaviour, hypogonadism and partial growth hormone (GH) deficiency (GHD). Severe GHD of other aetiologies has been shown to affect mood and quality of life negatively, and there are reports of improvements with GH replacement. We have studied cognitive, emotional, physical and social parameters in PWS adults at baseline, during and after GH treatment. Patients and methods Nineteen patients, 9 females and 10 males, median age 25 years, mean BMI 35 kg/m2 participated in this study. Approximately half of the group had GHD. All patients fulfilled the clinical criteria for PWS and 13 had a positive genotype. The patients were randomized to 6 months of treatment with either GH [1.6 IU/day (0.53 mg/day)] or placebo, followed by 12 months of active GH treatment. Treatment was then stopped, and the patients were followed for an additional period of 6 months. A test battery for general cognitive evaluation and a computer-based measurement of reaction time, motor speed and fluency were employed at baseline, after 6 months and at the end of GH treatment. At the same time intervals, a self-evaluation questionnaire was answered at the end of each test session. Other questionnaires reflecting the patients’ cognitive, emotional, physical and social status were answered by relatives/caretakers at baseline and at 3 and 6 months following cessation of GH treatment. Results Baseline cognitive level was estimated to be moderately to mildly impaired; IQ range was 40–90. The results from some of the cognitive and the motor performance tests improved significantly after 6 and 18 months of GH treatment. According to the questionnaires, both the patients and the relatives/caretakers evaluated physical status rather negatively at baseline, but still, impairments in both physical and social status and overall functioning were observed when GH treatment was discontinued. The self-evaluation did not change in any aspect during GH treatment. Conclusions In this pilot study of an adult PWS cohort, we were able to document beneficial effects in mental speed and flexibility and in motor performance during GH treatment. Impairment was seen in physical and social status as well as overall functioning, when GH treatment stopped. Studies of larger cohorts are needed to further elucidate the role of GH treatment in this group of patients.

Behavioural, academic and neuropsychological profile of normally gifted Neurofibromatosis type 1 children

Abstract: UNLABELLED In the present study the neuropsychological, academic and social-emotional profiles were examined in Neurofibromatosis type 1 (NF1) children. SUBJECTS 17 NF1 children (ages 7-11) with NF1 without serious medical problems and with a full scale IQ (FSIQ) above 70. METHODS Wechsler Intelligence Scale for Children-Revised (WISC-R), academic tests and an exhaustive neuropsychological test battery were administered in all children. Parents and teachers filled out the Child Behavioural Checklist (CBCL) and Teacher Report Form (TRF), respectively, the NF1 children the Experienced Competence Scale for Children (ECSC). RESULTS AND DISCUSSION Nearly 50% (8/17) of the children showed learning disabilities, when corrected for IQ in the academic evaluations. Isolated impaired literacy skills, particularly spelling problems, were most frequent (4/8), whereas a pure arithmetic learning disability was rare (1/8). Three children presented both learning disabilities. Results on academic and neuropsychological tests did not fit the well-known types of learning disabilities -- nonverbal learning disability (NLD) and dyslexia. Nearly all NF1 children showed visual perceptual and executive dysfunctions. In this study, teachers more frequently reported behavioural problems in NF1 children than parents, as opposed to literature data in a general population. The correspondence of the perception of internalizing problems between the children and teachers was greater than between children and their parents. No correlation was found between the performances on the WISC-R, specific neuropsychological results, academic performances and behavioural problems. The Deficiency in Attention, Motor and Perception (DAMP) concept seems most appropriate in order to describe the neuropsychological deficits and their repercussions on behavioural and academic performances seen in NF1 children.

Expressive and receptive vocabulary in children with Williams and Down syndromes

Abstract: Background Williams (WS) and Down syndromes (DS) are two genetic disorders that involve intellectual disability (ID) and have been extensively studied over the past decades because of the unique linguistic profiles they exhibit. Recent investigations seek to explore the fractionation of linguistic components within the cognitive system using genetically based neurodevelopmental disorders such as WS and DS and to identify different profiles of linguistic function in these two groups of individuals. Method The ‘expressive vocabulary’, ‘receptive vocabulary’, ‘word opposites’ and ‘word definitions’ subtests (Level 1) of the Test of Word Knowledge (TOWK) were used to assess lexical skills in six children with WS and five children with DS. Results Our findings indicate that the two syndromes exhibit substantial differences on linguistic tasks with individuals with WS performing at a higher level compared to those with DS and producing atypical responses in word definitions. The pattern of errors for each syndrome is qualitatively different suggesting that their underlying linguistic mechanisms are distinctive even though ID is similar. Conclusions This study supports the differential outcome of two chromosomal disorders with similar ID. It also argues in favour of the large within group variability of the two syndromes that is not related to mental age but rather to different underlying mechanisms supporting language. These findings are discussed in the light of the current evidence concerning linguistic knowledge of neurodevelopmental and genetic disorders.

Family Quality of Life from the Perspective of Older Parents.

Abstract: Background Family quality of life is a relatively new field of study. Research has primarily concentrated on families of children and young adults with intellectual disability (ID). Method This project explored the concept of family quality of life from the perspective of older parents who had adult children with ID aged 40+. Focus groups, individual interviews and surveys investigated family issues related to maintaining or enhancing family quality of life. Results Parents reported positive aspects of lifelong caregiving and quality of family life. There were concerns for the health of all family members, the social lives of their adult child, long-term living arrangements and the role of siblings. Conclusion Current family and service issues that need to be addressed are related to funding and finances, staffing, and the need to adapt to age-related needs.

Conceptual principles of quality of life: an empirical exploration

Abstract: [Structured summary]: Background: Quality of life is a popular measure of outcomes and its widespread use has led to recent calls for a better understanding of the construct, emphasising the need to build a substantial body of knowledge around what determines perceptions of life quality. Some conceptual principles are examined in this study. Method: Self-ratings of life quality and three likely determinants at an individual level (stress), an interactional level (social support) and a community level (neighbourhood belonging) were used. Two groups of young adults from an urban community participated, one identified as having an intellectual disability. Results: Young adults with intellectual disability rated their satisfaction with health significantly higher and intimacy and community involvement lower than the comparison group. Social support emerged as the strongest predictor of life satisfaction across both groups. Conclusion: The conceptual principles of subjective quality of life provide a useful framework to discuss findings and to stimulate further research.