L.M.G. Curfs

Impact of environmental factors on community participation of persons with an intellectual disability: a systematic review

STUDY DESIGN A systematic review of the literature. OBJECTIVES To describe which environmental factors have an impact on community participation of persons with an intellectual disability. METHODS A systematic literature search was conducted for the period of 1996-2006 in Pubmed, CINAHL and PSYCINFO. Search terms were derived from the International Classification of Functioning, Disability and Health. Three investigators assessed the relevance of the studies identified using predefined selection criteria. Aspects of community participation included were: domestic life; interpersonal interactions and relationships; major life areas; community, civic and social life. Environmental factors included were: products and technology; natural environment and human-made changes to environment; support and relationships; attitudes; services, systems and policies. RESULTS Out of 236 initial hits, 9 quantitative studies and 2 qualitative studies met the predefined selection criteria and were included in the study. Various research instruments were used in the studies and only one study used a conceptual framework. The review allowed the identification of a number of environmental factors positively affecting participation: opportunities to make choices; variety and stimulation of the environment of facilities; opportunities for resident involvement in policy making; small residential facilities; opportunities for autonomy; vocational services; social support; family involvement; assistive technology; and positive staff attitudes. A number of identified environmental factors negatively affecting participation are: lack of transport and not feeling accepted. DISCUSSION It can be concluded that little has been published about the impact of environmental factors on community participation. Many studies do not clearly define the concept of community participation. Research on the impact of environmental factors on community participation so far seems not to be based on a theoretical framework. Most studies focused on the impact of services on community participation in general.

End-of-life decisions: an important theme in the care for people with intellectual disabilities

BACKGROUND While end-of-life decisions in the general population have received attention in several countries, not much is known about this in people with intellectual disabilities (IDs). Therefore, the prevalence and nature of end-of-life decisions were investigated in a Dutch centre providing residential care for 335 people with IDs. METHOD A retrospective study of medical files of people who died between January 2002 and July 2007. RESULTS One or more end-of-life decisions were taken in 27 out of 47 cases. A non-treatment decision was taken for seven residents, possibly shortening life expectancy in some cases. The family was involved in decision making in half of the 27 cases. No information was found about the process of end-of-life decision making. There was no evidence in the notes that any of the people with IDs was asked for his or her own opinion in taking an end-of-life decision. CONCLUSION This study demonstrates that medical end-of-life decisions played a part in significant numbers of people with IDs who have died within this centre, but further studies are needed to establish decision-making processes.

Melatonin treatment in individuals with intellectual disability and chronic insomnia: a randomized placebo-controlled study

BACKGROUND While several small-number or open-label studies suggest that melatonin improves sleep in individuals with intellectual disabilities (ID) with chronic sleep disturbance, a larger randomized control trial is necessary to validate these promising results. METHODS The effectiveness of melatonin for the treatment of chronic sleep disturbance was assessed in a randomized double-blind placebo-controlled trial with 51 individuals with ID. All of these individuals presented with chronic ideopatic sleep disturbance for more than 1 year. The study consisted of a 1-week baseline, followed by 4 weeks of treatment. Parents or other caregivers recorded lights off time, sleep onset time, night waking, wake up time and epileptic seizures. Endogenous melatonin cycle was measured in saliva before and after treatment. RESULTS Compared with placebo, melatonin significantly advanced mean sleep onset time by 34 min, decreased mean sleep latency by 29 min, increased mean total sleep time by 48 min, reduced the mean number of times the person awoke during the night by 0.4, decreased the mean duration of these night waking periods by 17 min and advanced endogenous melatonin onset at night by an average of 2.01 h. Lights off time, sleep offset time and the number of nights per week with night waking did not change. Only few minor or temporary adverse reactions and no changes in seizure frequency were reported. CONCLUSIONS Melatonin treatment improves some aspects of chronic sleep disturbance in individuals with ID.

Psychosocial problems, coping strategies, and the need for information of parents of children with Prader–Willi syndrome and Angelman syndrome

The aim of the present study was to identify the psychosocial problems of parents of a child with Prader-Willi syndrome or a child with Angelman syndrome. In addition, the strategies these parents apply to cope with these problems as well as their need for information are described. To assess these topics, parents filled in a self-report questionnaire. Both parent groups were found to have a high need for information, high feelings of loss of control, relatively high depressive feelings, particularly in mothers in both syndrome groups. Differences due to the type of syndrome were found on the fear factor. Parents of a child with Angelman syndrome had greater feelings of fear for the negative consequences for themselves, whereas parents of Prader-Willi children were more concerned about the consequences for the child. In general, coping strategies were not found to be different between the parent groups of children who had either type of syndrome.

The psychopathological phenotype of Velo-cardio-facial syndrome

Velo-cardio-facial syndrome (VCFS) is mostly associated with deletions of chromosome 22q11, and is thought to be characterized by an increased frequency of major psychiatric disorders. Sixteen patients adults with VCFS and psychiatric symptoms were evaluated using a semi-structured investigation of history, symptoms, signs and behaviour. All available data were used in consensus meetings to obtain a classifiable diagnostic category. In contrast to other reports, no categorical diagnosis could be established. Instead, a quite specific psychological, behavioural and psychopathological constellation emerged that should most adequately be denominated as a VCFS-psychiatric syndrome. It is concluded that VCFS is associated with a specific psychopathological syndrome.

Behavioural flexibility in individuals with Angelman syndrome, Down syndrome, non-specific intellectual disability and Autism spectrum disorder

BACKGROUND Little is known about behavioural flexibility in children and adults with Angelman syndrome and whether people with this syndrome have more or less problems in being behaviourally flexible as compared with other people. METHOD Behavioural flexibility scores were assessed in 129 individuals with Angelman syndrome using 11 items from the Behavioural Flexibility Rating Scale-Revised (Green et al. 2007). Level of behavioural flexibility scores in individuals with Angelman syndrome (N = 129) was compared with that of people with non-specific intellectual disability (ID) (N = 90), Down syndrome (N = 398) and Autism spectrum disorder (N = 235). RESULTS Comparative analyses show that individuals with Angelman syndrome were more flexible than those with non-specific ID (P < 0.001) and those with Autism spectrum disorder (P < 0.01). There were no differences in behavioural flexibility scores between individuals with Angelman syndrome and those with Down syndrome (P = 0.94). CONCLUSION It is concluded that individuals with Angelman syndrome are comparatively flexible in their behaviour.

Homosexuality among people with a mild intellectual disability: an explorative study on the lived experiences of homosexual people in the Netherlands with a mild intellectual disability

BACKGROUND Empirical research on homosexuality among people with an intellectual disability (ID) is limited and, to date, very little is known regarding the personal experiences of gay and lesbian people with an ID. This study set out to answer the question: What are the lived experiences of a specific cohort of homosexual people with an intellectual disability living in the Netherlands? METHOD To answer this question, a qualitative study was performed in which 21 people with a mild ID were interviewed via semi-structured interviews. In this study, 19 participants were men and two were women (average age = 40.5 years). RESULTS Participants reported positive and negative experiences, and talked about their gay or lesbian identity. Almost half of the participants (n = 10) reported that they had experienced sexual abuse including partner violence (n = 6). Additionally, they indicated that there was a lack of support for homosexual people with an ID. CONCLUSION Specific problems impact the lives of homosexual people with ID, namely the high prevalence of negative sexual experiences, the lack of support, training and sex education, and their search for a suitable partner.

Sleep disturbances and behavioural problems in adults with Prader-Willi syndrome.

BACKGROUND Individuals with Prader-Willi syndrome (PWS) are at risk of sleep disturbances, such as excessive daytime sleepiness (EDS) and sleep apnoea, and behavioural problems. Sleep disturbances and their relationship with other variables had not been researched extensively in adults with PWS. METHOD Sleep disturbances and behavioural problems were investigated in adults with genetically confirmed PWS using standardised questionnaires. Results of adults with paternal deletion (n=45) were compared with those of adults with maternal uniparental disomy (n=33). RESULTS Eleven adults with PWS (i.e. 15%) had a current sleep problem, mostly night waking problems. Twenty-six adults with PWS (i.e. 33%) suffered from severe EDS. No differences in prevalence of sleep disturbances between genetic subtypes were found. Seventeen adults with deletion (i.e. 38%) and 17 adults with maternal uniparental disomy (i.e. 52%) had behavioural problems. No significant relationships were found between sleep disturbances and behavioural problems. CONCLUSIONS In adults with PWS, EDS is the most common type of sleep disturbance. Men and individuals with relative high body mass index are at increased risk for EDS. More research, aimed at developing a suitable screening instrument for sleep apnoea in adults with PWS, is necessary. Clinical implications of the findings are discussed.

Melatonin decreases daytime challenging behaviour in persons with intellectual disability and chronic insomnia

BACKGROUND Persons with intellectual disability (ID) and sleep problems exhibit more daytime challenging behaviours than persons with ID without sleep problems. Several anecdotal reports suggest that melatonin is not only effective in the treatment of insomnia, but also decreases daytime challenging behaviour. However, the effect of melatonin treatment on daytime challenging behaviour in persons with ID has not been investigated in a randomised controlled trial. METHOD We investigated the effects of melatonin on challenging behaviour using data from two randomised controlled trials on the efficacy of melatonin on sleep problems in 49 persons (25 men, 24 women; mean age 18.2 years, SD = 17.1) with ID and chronic insomnia. Participants received either melatonin 5 mg (<6 years 2.5 mg) or placebo during 4 weeks. Daytime challenging behaviour was measured by the Storend Gedragsschaal voor Zwakzinnigen - Maladaptive Behaviour Scale for the Mentally Retarded (SGZ; Kraijer & Kema, 1994) at baseline week and the end of the fourth treatment week. Salivary dim light melatonin onset (DLMO) was measured at baseline and the last day of the fourth treatment week. Sleep logs were used to gather information on sleep parameters. RESULTS Melatonin treatment significantly reduced SGZ scores, sleep latency, and number and duration of night wakes, and treatment increased total sleep time and advanced DLMO. However, after 4 weeks of treatment, change in SGZ scores did not significantly correlate with change in sleep parameters, nor with change in DLMO. Relatively strong correlations were found between change in SGZ scores, change in DLMO and number of night wakes. CONCLUSIONS Melatonin treatment in persons with ID and chronic insomnia decreases daytime challenging behaviour, probably by improving sleep maintenance or by improving circadian melatonin rhythmicity.

Personality profiles of children and adolescents with neurofibromatosis type 1

The personality profile of 44 youngsters (24 males, 20 females; mean age 11 years, 3 months) with neurofibromatosis type 1 (NF1) was compared with a group of 220 non‐NF1 control youngsters (matched on age and gender). Personality characteristics of each youngster were rated by both parents, using the California Child Q‐set (CCQ); [Block and Block, 1980]. The scores on eight personality dimensions were compared, i.e., Extraversion, Agreeableness, Conscientiousness, Emotional Stability, Openness, Motor Activity, Irritability, and Dependency. Moreover, personality of NF1 youngsters was related to IQ level, severity of medical problems, the presence or absence of visible cosmetic disfigurements, and de novo versus familial origin of NF1. The personality profile of NF1 youngsters was markedly different from the non‐NF1 youngsters. Compared to the 220 control children, they were equally agreeable, but less conscientious, less emotionally stable, less open for new experience, with less motor activity, and more extravert, more dependent, and more irritable. Personality characteristics were similar for children with maternally or paternally inherited NF1, or for children with a new mutation. There was no association with gender, the severity of medical and cosmetic problems, and IQ.