Motivational Interviewing, 2nd ednWilliam Miller and Stephen RollnickThe Guilford Press. ISBN: 1‐572‐30563‐0, 2002, 428 pp with index, £26.95 (hbk)

More people with intellectual disability are living independent lives. They can and do experiment with substances that the wider community try, such as alcohol and drugs (both legal and illicit). Unfortunately for some, they develop problems related to their use of these substances. Face‐to‐face, semi‐structured interviews were conducted with 13 professionals who work in Intellectual Disability Services and Alcohol &; Drug Services to discover their experiences of caring for people with intellectual disabilities who hazardously use substances. Although small numbers of people presented to these services, many more people with intellectual disabilities used Intellectual Disability Services for support, rather than their local Alcohol &; Drug Services. While the numbers may be relatively small, the challenges this client group pose are very perturbing in relation to their physical, emotional and social health. The professionals reported a lack of education in working with this doubly disadvantaged population. Moreover, policies were absent to guide staff to work collaboratively with this often‐ignored population. These findings are discussed in light of the innovative practices that are occurring in other parts of the UK regarding the recognition, assessment treatment and long‐term management of this population. Intellectual Disability Services and Alcohol &; Drug Services need to work more closely together if the needs of this population are to be effectively met.     

Discapacidad intelectual en niños y adolescentes: influencia en la familia y la salud familiar. Revisión sistemática

Objective

To examine the influence of a child or adolescent with intellectual disabilities on the family unit.

Cross-cultural adaptation to Brazilian Portuguese of the Activity Measure for Post-Acute Care (AM-PAC) short forms for outpatients in rehabilitation

Background

The Activity Measure for Post-Acute Care was developed to evaluate the limitations of activities of adult individuals with different health conditions.

Psychometric properties of the International Classification of Functioning,Disability and Health set for spinal cord injury nursing based on Rasch analysis

Objective: To examine the psychometric properties of the International Classification of Functioning, Disability and Health (ICF) set for spinal cord injury nursing (ICF-SCIN) using Rasch analysis.

Methods: A total of 140 spinal cord injury patients were recruited between December 2013 and March 2014 through convenience sampling. Nurses used the components body functions (BF), body structures (BS), and activities and participation (AP) of the ICF-SCIN to rate the patients’ functioning. Rasch analysis was performed using RUMM 2030 software.

Results: In each component, categories were rescored from 01234 to 01112 because of reversed thresholds. Nine testlets were created to overcome local dependency. Four categories which fit to the Rasch model poorly were deleted. After modification, the components BF, BS, and AP showed good fit to the Rasch model with a Bonferroni-adjusted significant level (χ^2?=^?86.29, p?=?0.006; χ^2?=^?22.44, p?=?0.130; χ^2?=^?39.92, p?= 0.159). The person separation indices (PSIs) for the three components were 0.80, 0.54, and 0.97, respectively. No differential item functioning (DIF) was detected across age, gender, or educational level.

Conclusions: The fit properties of the ICF set were satisfactory after modifications. The ICF-SCIN has the potential as a nursing assessment instrument for measuring the functioning of patients with spinal cord injury.

• Implications for rehabilitation

• The International Classification of Functioning, Disability and Health (ICF) set for spinal cord injury nursing contains a group of categories which can reflect the functioning of spinal cord injury patients from the perspective of nurses.

• The components body functions (BF), body structures (BS), and activities and participation (AP) of the ICF set for spinal cord injury achieved the fit to the Rasch model through rescoring, generating testlets, and deleting categories with poor fit.

• The ICF set for spinal cord injury nursing (ICF-SCIN) has the potential to be used as a clinical nursing assessment tool in measuring the functioning of patients with spinal cord injury.

     

Job satisfaction of people with intellectual disabilities: the role of basic psychological need fulfillment and workplace participation

Purpose: Knowledge on what contributes to job satisfaction of people with intellectual disabilities is limited. Using self-determination theory, we investigated whether fulfillment of basic psychological needs (i.e., autonomy, relatedness, competence) affected job satisfaction, and explored associations between workplace participation, need fulfillment and job satisfaction.

Method: A total of 117 persons with intellectual disabilities, recruited from a Dutch care organization, were interviewed on need fulfillment at work and job satisfaction. Data on workplace participation was obtained from staff. Questionnaires were based on well-established instruments.

Results: Basic psychological need fulfillment predicted higher levels of job satisfaction. Level of workplace participation was not associated with need fulfillment or job satisfaction.

Conclusions: Allowing workers with intellectual disabilities to act with a sense of volition, feel effective, able to meet challenges, and connected to others is essential and contributes to job satisfaction. It is needed to pay attention to this, both in selection and design of workplaces and in support style.

• Implications for rehabilitation

• Knowledge on factors that contribute to job satisfaction is necessary to improve employment situations and employment success of people with intellectual disabilities.

• In order to achieve job satisfaction, it is essential that workplaces allow for fulfillment of the basic psychological needs for autonomy, relatedness, and competence of people with intellectual disabilities.

• People with intellectual disabilities are able to report on their needs and satisfaction, and it is important that their own perspective is taken into account in decisions regarding their employment situation.

     

Effects of extended-release quetiapine fumarate on long-term functioning and sleep quality in patients with Generalized Anxiety Disorder (GAD): Data from a randomized-withdrawal,placebo-controlled maintenance study

Background

This analysis evaluated effects of quetiapine XR maintenance treatment on functioning and sleep in patients with GAD.

Methods

Analysis of patient-reported data from a randomized-withdrawal, double-blind, placebo-controlled study of quetiapine XR monotherapy in GAD. Following open-label run-in (4–8 weeks) and a 12–18-week stabilization phase (quetiapine XR 50, 150, or 300 mg/day), eligible patients were randomized to continue on quetiapine XR or receive placebo for up to 52 weeks. Primary variable was time to an anxiety event. Secondary variables included the Sheehan Disability Scale (SDS) and Pittsburgh Sleep Quality Index (PSQI).

Results

In total, 432 patients were randomized (quetiapine XR, N=216; placebo, N=216). The risk of an anxiety event was significantly reduced for quetiapine XR vs. placebo (HR 0.19; 95% CI 0.12, 0.31; p<0.001). Quetiapine XR was more effective than placebo at maintaining SDS total scores (LSM change: −0.19 vs. 1.01; p=0.017) and non-work-related SDS domain score ‘family life/home responsibilities’ (−0.13 vs. 0.32; p=0.011), but not ‘social life’ (0.05 vs. 0.34; p=0.114). Quetiapine XR was more effective than placebo at maintaining the work-related SDS domain score ‘days lost’ (−0.05 vs. 0.11; p=0.027), but not ‘work/school’ (−0.10 vs. 0.29; p=0.051) or ‘days underproductive’ (0.06 vs. 0.13; p=0.619). PSQI global scores were reduced from randomization with quetiapine XR vs. placebo (0.39 vs. 1.60; p<0.001).

Limitations

Lack of active-comparator arm, exclusion of patients with comorbid depression.

Conclusions

In patients with GAD, long-term treatment with quetiapine XR (50–300 mg/day) monotherapy was effective at maintaining improvements in functioning and sleep quality.     

Ketogenic diet for refractory epilepsy with MEHMO syndrome: Caution for acute necrotizing pancreatitis

BackgroundThe MEHMO (mental retardation, epileptic seizures, hypogonadism and hypogenitalism, microcephaly, and obesity) syndrome, which is caused by a hemizygous variant in the EIF2S3 gene on chromosome Xp22, is associated with significant morbidity and mortality. Refractory epileptic seizures and glucose dysregulation are characteristic manifestations of the MEHMO syndrome, which can often diminish patients’ quality of life.CaseA 5-year-old boy was referred to our hospital because of profound intellectual disability, micropenis, cryptorchidism, central hypothyroidism, and microcephaly. He had neonatal hypoglycemia at birth and later experienced refractory epileptic seizures and developed obesity and insulin-dependent diabetes. A diagnosis of MEHMO syndrome was established on the basis of the patient’s clinical manifestations and de novo novel missense variant in the EIF2S3 gene (NM_001415.3:c.805 T > G) that was detected through whole-exome analysis. Although the patient’s refractory seizures and diabetes had been well controlled with a combination of ketogenic diet (KD) therapy and insulin therapy, acute fatal necrotizing pancreatitis occurred at the age of 68 months. Moreover, despite intensive care, his condition rapidly deteriorated to multiple organ failure and acute respiratory distress syndrome, resulting in death.ConclusionThe pathophysiology of glucose intolerance in MEHMO syndrome remains to be elucidated; however, recent studies have suggested that EIF2S3 gene variants could lead to glucose dysregulation and β-cell damage in the pancreas. We suspect that in the present case, KD therapy led to an abnormal load on the beta cells that were damaged owing to eIF2γ dysfunction. Therefore, the adverse effects of KD in patients with MEHMO syndrome should be considered.     

Sleep difficulties and related behavioral problems in Korean preschool children

ObjectivesSleep difficulties are associated with various behavioral problems and cognitive development, but the association has not been thoroughly evaluated in preschoolers with and without developmental disability (DD). The aim of this study was to compare the behavioral and cognitive characteristics of poor and good sleepers with and without DD in a community-based sample of preschool children.MethodsWe recruited 196 children aged 34–77 months from community-based daycare centers, kindergartens, and special education centers. Children were classified as poor sleepers with DD (n = 23), poor sleepers without DD (n = 23), good sleepers with DD (n = 64), and good sleepers without DD (n = 94). Poor sleepers were defined based on Children's Sleep Habits Questionnaire Total Sleep Disturbance Score (CSHQ TSDS) cutoff of >52.ResultsWhen compared to good sleepers with DD, poor sleepers with DD scored significantly higher on the Attention Problems (p = 0.005) and Aggressive Behaviors (p = 0.001) subscales of the CBCL, indicating greater difficulty. Caregivers of the poor sleepers with DD reported more depressive symptoms (p = 0.028) and more stress related to the child (p = 0.002) and the parent (p = 0.045) than caregivers of the good sleepers with DD. On the other hand, when compared to good sleepers without DD, poor sleepers without DD scored significantly higher only on the Anxious/Depressed subscale (p = 0.045) of the CBCL. Cognitive development and adaptive functioning were not significantly different between poor and good sleepers with and without DD. Among the subscales of the CSHQ, the Sleep Onset Delay showed strongest correlation with behavioral measures of the children and distress of the caregivers.ConclusionExternalizing problems such as aggressive behaviors, rather than cognitive development, are associated with sleep difficulties in preschool children with DD.