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The central aspect of this study is a set of reflections on the efficacy of soft operational research techniques in understanding the dynamics of a complex system such as intellectual disability (ID) care providers. Organizations providing services to ID patients are complex and have many interacting stakeholders with often different and competing interests. Understanding the causes for failures in complex systems is crucial for appreciating the multiple perspectives of the key stakeholders of the system. Knowing the factors that adversely affect delivery of a patient-centred care by ID provider organizations offers the potential for identifying more effective resource-allocation solutions. The authors suggest cognitive mapping as a starting point for system dynamics modelling of optimal resource-allocation projects in ID care. The application of the method is illustrated via a case study in one of the ID care providers in the Netherlands. The paper discusses some of the practical implications of applying problem-structuring methods that support gathering feedback from vulnerable service users and front-line workers. The authors concluded that cognitive mapping technique can assist the management of healthcare organizations in strategic decision-making., (Copyright © 2013 John Wiley & Sons, Ltd.)

Sex education for individuals with intellectual disabilities is important. However, our knowledge about effective methods for teaching sex education to this population is limited. We report the results of a systematic review identifying methods for sex education programs aimed at individuals with intellectual disabilities. In all, 20 articles were included that met the criteria set in terms of topic--the effectiveness of sex education programs--and population of interest--individuals with intellectual disabilities. In these articles, methods for increasing knowledge and for improving skills and attitudes were reported. However, the studies revealed that generalization of skills to real-life situations was often not achieved. There are indications that the maintenance of knowledge and skills still needs extra attention. Moreover, detailed descriptions of the program materials, program goals, and methods used in the programs were often lacking in the reports. Although there is some evidence for methods that may improve knowledge, attitudes, and skills with regard to sex education aimed at individuals with intellectual disabilities, due to the lack of detailed descriptions provided it is unclear under which conditions these methods work. We therefore suggest that authors provide additional detail about methods in future publications or in online supplements.

Frequency and type of incontinence and variables associated with incontinence were assessed in individuals with Angelman syndrome (AS; n=71) and in a matched control group (n=69) consisting of individuals with non-specific intellectual disability (ID). A Dutch version of the "Parental Questionnaire: Enuresis/Urinary Incontinence" (Beetz, von Gontard, & Lettgen, 1994) was administered and information on primary caretakers' perspectives regarding each individual's incontinence was gathered. Results show that diurnal incontinence and fecal incontinence during the day more frequently occurred in the control group than in the AS group. In both groups, nocturnal enuresis was the most common form of incontinence. More incontinence was seen in individuals with AS who were younger, had a lower level of adaptive functioning and/or had epilepsy. Individuals with AS were able to stay dry for longer periods of time than the controls and often showed both in-toilet urination and urinary accidents during the day, whereas accidents and correct voids during the day were more set apart in the control group. Also, persons with AS had a lower micturition frequency implying possible voiding postponement. Both groups showed high rates of LUTS (lower urinary tract symptoms) possibly indicative of functional bladder disorders such as voiding postponement, dysfunctional voiding, or even an underactive bladder. In general, most primary caretakers reported severe intellectual disability as the main cause for urinary incontinence. Based on these results incontinence does not appear to be part of the behavioral phenotype of Angelman syndrome. Therefore, pediatric or urologic diagnostics and treatment are recommended for all persons with incontinence and intellectual disability. Further implications for practice and research are given., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background: Not much is known about the process of end-of-life decision-making for people with intellectual disabilities., Aim: To clarify the process of end-of-life decision-making for people with intellectual disabilities from the perspective of patient representatives., Design: A qualitative study based on semi-structured interviews, recorded digitally and transcribed verbatim. Data were analysed using Grounded Theory procedures., Participants: We interviewed 16 patient representatives after the deaths of 10 people with intellectual disabilities in the Netherlands., Results: The core category 'Deciding for someone else' describes the context in which patient representatives took end-of-life decisions. The patient representatives felt highly responsible for the outcomes. They had not involved the patients in the end-of-life decision-making process, nor any professionals other than the doctor. The categories of 'Motives' and 'Support' were connected to the core category of 'Deciding for someone else'. 'Motives' refers to the patient representatives' ideas about quality of life, prevention from suffering, patients who cannot understand the burden of interventions and emotional reasons reported by patient representatives. 'Support' refers to the support that patient representatives wanted the doctors to give to them in the decision-making process., Conclusions: From the perspective of the patient representatives, the process of end-of-life decision-making can be improved by ensuring clear roles and an explicit description of the tasks and responsibilities of all participants. Regular discussion between everyone involved including people with intellectual disabilities themselves can improve knowledge about each other's motives for end-of-decisions and can clarify expectations towards each other.

The prevalence of sleep problems in individuals with intellectual disability (ID) seems to vary between genetic syndromes associated with ID. Different types of sleep disturbances may indicate underlying causes of sleep problems and these types of sleep disturbances may vary between different genetic syndromes. We examined and compared five types of sleep disturbance as well as severity of sleep problems in individuals with Cri du Chat syndrome (CDC), Down's syndrome (DS), Jacobsen syndrome (JS), and individuals with non-specific ID (NS). We used Simonds and Parraga's Sleep Questionnaire (1982) to assess prevalence of types of sleep disturbance and to explore differences in types of sleep disturbance and severity of sleep problems between the four diagnostic groups. In each group, mean scores for Snoring were significantly higher than those for Sleep apnea and Snoring was the most prevalent type of sleep disturbance in CDC, DS, and JS. The mean score on Complaints related to sleep was remarkably high in the JS group. There were no differences in severity of sleep problems between groups. These findings suggest that snoring is an important underlying cause of sleep problems in individuals with CDS, DS, and JS., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Improvements in client-caregiver relationships may lead to improvements in the quality of life of clients with intellectual disabilities (ID). For this reason, interventions aimed at influencing these relationships are important. To gain insight into the nature and intention of these relationships in the ID population, suitable measurement instruments are needed. This study examines the applicability of an existing relationship questionnaire designed for primary education, called the Student-Teacher Relationship Scale (STRS) on the basis of the following research questions: (1) What is the factor structure of the STRS? (2) Are there associations between STRS scales and other conceptually comparable instruments? (3) Is the STRS reliable? The participants in this study were 46 caregivers, who assessed 350 client-caregiver relationships. Psychometric research was conducted into the factor structure (n=350), construct validity (n=146), internal consistency (n=350) and test-retest reliability (n=177) of the STRS and the reliability of the individual scores (n=350) among a study population of people with moderate and severe ID. The three-factor model of the STRS as used in primary education (1. closeness, 2. conflict, 3. dependency) was, despite minor deviations, also found in the ID population. Research into the construct validity of the STRS showed statistically significant correlations with other scales with which similarities could be expected. The internal consistency and test-retest reliability of the STRS in the population studied were very good. The 95% confidence intervals of the means were small, and these measurements can be regarded as reliable., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

We examined the psychometric properties of one part of the Sleep Questionnaire developed by Simonds and Parraga (SQ-SP; 1982), a questionnaire that is frequently used to explore sleep problems and behaviors related to sleep in individuals with intellectual disability (ID). The SQ-SP was completed for 345 individuals with ID (sleep clinic n = 146; control group n = 103; published studies n = 68; psychiatric clinic n = 28). Internal consistency was good (Cronbach's α = .80) and test-retest reliability for the total SQ-SP score was also good (Spearman's rank correlation = .83, p<.01). Convergent validity was adequate (r = .79, p<.001) and concurrent validity was satisfactory (r = .52, p<.001). Exploratory factor analysis suggested a 5-factor structure (Snoring, Daytime sleepiness, Complaints related to sleep, Sleep apnea and Anxiety related to sleep). Internal consistency of the five factors ranged from modest (Cronbach's α = .57) to good (Cronbach's α = .82). Confirmatory factor analysis corroborated the 5-factor structure. The Composite Sleep Index, the total SQ-SP score and the factor scores on Daytime Sleepiness and Complaints related to sleep were able to differentiate the control group from the sleep clinic group. The SQ-SP appears to be a reliable and valid tool in assessing sleep and different types of sleep disturbance in individuals with ID., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background: In some of our patients with intellectual disability (ID) and sleep problems, the initial good response to melatonin disappeared within a few weeks after starting treatment, while the good response returned only after considerable dose reduction. The cause for this loss of response to melatonin is yet unknown. We hypothesise that this loss of response is associated with slow melatonin metabolism., Method: In this study, we determined melatonin clearance in two female (aged 61 and 6 years) and one male (aged 3 years) patients who had chronic insomnia, late melatonin onset and mild ID, and whose sleep quality worsened a few weeks after initial good response to melatonin treatment, suggesting melatonin tolerance. After a 3-week washout period, patients received melatonin 1.0, 0.5 or 0.1 mg, respectively. Salivary melatonin level was measured just before melatonin administration, and 2 and 4 h thereafter. After this melatonin clearance test, melatonin treatment was resumed with a considerably lower dose., Results: In all patients melatonin concentrations remained >50 pg/mL at 2 and 4 h after melatonin administration. After resuming melatonin treatment sleep problems disappeared. The same procedure was followed in three patients who did not show loss of response to melatonin after 6 months of treatment. In all patients in the control group melatonin concentrations decreased between 2 and 4 h after melatonin administration with a mean of 83%., Conclusion: We hypothesise that loss of response to melatonin treatment can be caused by slow metabolisation of exogenous melatonin. As melatonin is metabolised in the liver almost exclusively by cytochrome P450 enzyme CYP1A2, this slow melatonin metabolism is probably due to decreased activity/inducibility of CYP1A2. In patients with loss of response to melatonin, a melatonin clearance test should be considered and a considerably dose reduction is advised.

Recent meta-analyses on melatonin has raised doubts as to whether melatonin is effective in treating sleep problems in people without intellectual disabilities. This is in contrast to results of several trials on melatonin in treating sleep problems in individuals with intellectual disabilities. To investigate the efficacy of melatonin in treating sleep problems in individuals with intellectual disabilities, we performed a meta-analysis of placebo-controlled randomized trials of melatonin in individuals with intellectual disabilities and sleep problems. Data were selected from articles published on PubMed, Medline, and Embase between January 1990 and July 2008. We examined the influence of melatonin on sleep latency, total sleep time, and number of wakes per night. Quality of trials was assessed using the Downs and Black checklist. Nine studies (including a total of 183 individuals with intellectual disabilities) showed that melatonin treatment decreased sleep latency by a mean of 34 minutes (p<0.001), increased total sleep time by a mean of 50 minutes (p<0.001), and significantly decreased the number of wakes per night (p<0.05). Melatonin decreases sleep latency and number of wakes per night, and increases total sleep time in individuals with intellectual disabilities.

Background: Few case controlled studies have been published on the behavioural phenotype of Angelman syndrome (AS). Little is yet known about preferences in individuals with AS., Method: Preferences for water-related items and non-water-related items were assessed in 27 individuals with AS and two matched groups of participants, one with Down syndrome (DS) and the other with non-specific intellectual disabilities (NS), using an adapted Dutch version of the Choice Assessment Scale (Matson et al., 1999)., Results: Individuals with AS showed a higher preference for water-related items compared to individuals in both comparison groups, substantiating previous reports which have suggested that individuals with AS are fascinated with water., Conclusions: Knowledge about preferences in individuals with AS, especially with regard to water-related objects and activities, is important in person-centred planning of daytime activities and training programs for this group.

Sanfilippo type B is an autosomal recessive mucopolysaccharidosis (MPS IIIB) caused by deficiency of N-acetyl-alpha-D-glucosaminidase, a lysosomal enzyme involved in the degradation of heparan sulfate. It is characterized by neurologic degeneration, behavioral problems, and mental decline. Somatic features are relatively mild and patients with this disorder can reach late adulthood. It is the most common subtype of MPS in the Netherlands and probably underdiagnosed in adult persons with mental retardation (MR). In order to increase knowledge on the adult phenotype and natural history in Sanfilippo type B, we present the clinical data of 20 patients with this disorder. Sixteen of them were followed for one to three decades. Six died between 28 and 69 years of age, mainly from pneumonia and cachexia; the surviving patients were 18-63 years old. Apart from the youngest, they had lost mobility at 36-68 years. Most had developed physical problems, in particular in the 4th-6th decade of life: cardiac disease (cardiomyopathy, atrial fibrillations), arthritis, skin blistering, swallowing difficulties requiring feeding by a gastrostomy tube, and seizures. The course of the disease was dominated in most of them by challenging behavioral problems with restlessness, extreme screaming and hitting, difficult to prevent or to treat pharmaceutically. Even in absence of knowledge of the history of an elderly patient with MR, the presence of behavioral problems should prompt metabolic investigation for MPS., ((c) 2007 Wiley-Liss, Inc.)

Background Sex education programs have been developed with paid care staff as sex educators. However, no information is available about whether these programs are being delivered. Method The aim of this study was to investigate whether paid care staff working in an organisation specialised in the care of people with mild to moderate intellectual disability teach sex education or not. An online questionnaire was therefore constructed to assess the important factors associated with teaching sex education. Results Of the 163 staff members who completed the questionnaire, 39% provided sex education. Results show that it was mainly provided reactively. The main factor was the perceived social norm towards teaching sex education. Conclusions If we want paid care staff to teach sex education reactively, then we need to focus on changing the perceived social norm. However, if we want them to teach sex education proactively, a new needs assessment should be conducted in order to identify the important factors to motivate and enable them to provide sex education. [ABSTRACT FROM AUTHOR]

ABSTRACT Dutch adults from a nationwide Internet panel ( N = 426) were asked to imagine that their next-door neighbours would move out and that people with intellectual disability would move in. Severity of disability and group size were varied to manipulate intergroup threat. These two factors independently influenced social acceptance and a variety of emotional and behavioural measures. In particular, it was found that a large group with severe disability aroused the strongest negative response, whereas a small group with mild disability aroused the weakest negative response. Small groups with a severe disability and large groups with a mild disability aroused similar and intermediate negative responses. Results are discussed in terms of theories of intergroup threat and stigmatisation. Practical implications for predicting the success of de-institutionalisation and social integration of groups with special needs are addressed. Copyright © 2011 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Although people with intellectual disabilities (ID) are increasingly expected to relocate from traditional institutional care to ‘regular’ neighbourhood housing facilities and socially integrate in these neighbourhoods, little is known about how they are perceived and appreciated as neighbours. This paper reports on interviews carried out with 30 neighbours without ID who were neighbours of small-scale care facilities for people with ID. Interviews addressed the neighbours' everyday experiences of neighbouring in general, and neighbouring people with ID in particular. Neighbouring, for these informants, called for a fine balance between friendliness without over-involvement. While they were generally positive about their interactions with their neighbours with ID, it emerged that the formal nature of the care facility and the interaction style of some of the neighbours with ID often contravened informants' assumptions about neighbouring. Informants expressed concern about a possible lack of appropriate distance, reciprocity and accountability among their neighbours with ID. The nature of the care facility, with paid staff, often group activities, formal means of achieving the everyday small tasks which neighbours sometimes do for each other, and a high turnover of residents, all undermined the possibility of a typical neighbourly relationship. In conclusion, we suggest that integration of people with ID into everyday neighbouring relationships raises complex challenges for care organizations that need to find a balance between supporting the needs of people with ID they care for, adequate support and mediation for other neighbours when necessary, and all the while avoid becoming overly involved in neighbouring as a formal partner. Copyright © 2010 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]