'I get by with a little help from my friends' : adults with intellectual disability discuss loneliness

Background This study explored 'loneliness' as experienced by adults with intellectual disability, with 'intermittent' to 'limited' support needs.

Method A measure of loneliness was piloted, and qualitative techniques used to develop a greater understanding of the participants' experience.

Results The Loneliness Scale proved valid and reliable and the participants reported loneliness in ways comparable with the general population.

Conclusions The findings demonstrate the effectiveness of combining quantitative and qualitative techniques to enhance understanding of people's perspective when developing support systems to promote their quality of life. Based on participant perspectives, recommendations are made concerning the issues and types of support families and professionals could consider when seeking to assist people with intellectual disability address loneliness. Further investigation of the effects of differing educational and vocational opportunities on people's post-school social networks appears warranted.

The utility of the personal wellbeing index intellectual disability scale in an Australian sample

Background Subjective wellbeing (SWB) in people with intellectual disabilities has been the focus of increased interest in the identification of support needs and as an outcome measure for interventions and service delivery evaluations. It is therefore important to conduct further research in this area, and to develop appropriate scales to measure SWB.

Methods A new scale, the Personal Wellbeing Index-Intellectual Disability (PWI-ID) was administered to 114 adults with mild (n = 82) or moderate (n = 32) level ID in Victoria, Australia.

Results The PWI-ID demonstrated good reliability and validity. A comparison of the findings with previous research indicates that participants' SWB levels are within the normative range, and are similar to those reported by the general population.

Conclusions The results support the notion that individuals with ID do not experience life quality lower than normal, which can be explained theoretically by the Theory of Subjective Wellbeing Homeostasis. The use of the PWI-ID may ultimately assist in ensuring that the needs of people with ID are being met and inform the planning and delivery of congruent resources and services.

Detecting and treating depression in people with mild intellectual disability : the views of key stakeholders

There is a pressing need for the development of programs for the early identification and treatment of depression in individuals with mild intellectual disability. The aim of this study was to ascertain the perspective of 64 key stakeholders (people with intellectual disability, healthcare professionals, family/caregivers & support workers) regarding the risk factors and characteristics of depression, the support and service needs of individuals with intellectual disability/depression and those who care for them. Findings enhance our understanding of depression and its risk factors in people with mild intellectual disability and endorse the need for the development of screening and intervention programs suitable for delivery by staff in agencies providing services to people with mild intellectual disability.

An investigation into the subjective wellbeing of people with an intellectual disability

This thesis investigated the theory of subjective wellbeing homeostatic control for a sample of New Zealand people with mild to moderate intellectual disability, compared to non-disabled people. This involved three linked quantitative studies that investigated the internal buffers of self-esteem, control and optimism.

The content validity of the behaviour support plan quality evaluation tool (BSP-QEII) and its potential application in accommodation and day-support services for adults with intellectual disability

Background The quality of support provided to people with disability who show challenging behaviour could be influenced by the quality of the behaviour support plans (BSPs) on which staff rely for direction. This study investigated the content validity of the Behaviour Support Plan Quality Evaluation tool (BSP-QEII), originally developed to guide the development of BSPs for children in school settings, and evaluated its application for use in accommodation and day-support services for adults with intellectual disability.

Method A three-round Delphi study involving a purposive sample of experienced behaviour support practitioners (n = 30) was conducted over an 8-week period. The analyses included deductive content analysis and descriptive statistics.

Results The 12 quality domains of the BSP-QEII were affirmed as valid for application in adult accommodation and day-support service settings. Two additional quality domains were suggested, relating to the provision of detailed background on the client and the need for plans to reflect contemporary service philosophy. Furthermore, the results suggest that some issues previously identified in the literature as being important for inclusion in BSPs might not currently be a priority for practitioners. These included: the importance of specifying replacement or alternative behaviours to be taught, descriptions of teaching strategies to be used, reinforcers, and the specification of objective goals against which to evaluate the success of the intervention programme.

Conclusions The BSP-QEII provides a potentially useful framework to guide and evaluate the development of BSPs in services for adults with intellectual disability. Further research is warranted to investigate why practitioners are potentially giving greater attention to some areas of intervention practice than others, even where research has demonstrated these others areas of practice could be important to achieving quality outcomes.

A mouse model for X-linked adrenoleukodystrophy

X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder with impaired β-oxidation of very long chain fatty acids (VLCFAs) and reduced function of peroxisomal very long chain fatty acyl-CoA synthetase (VLCS) that leads to severe and progressive neurological disability. The X-ALD gene, identified by positional cloning, encodes a peroxisomal membrane protein (adrenoleukodystrophy protein; ALDP) that belongs to the ATP binding cassette transporter protein superfamily. Mutational analyses and functional studies of the X-ALD gene confirm that it and not VLCS is the gene responsible for X-ALD. Its role in the β-oxidation of VLCFAs and its effect on the function of VLCS are unclear. The complex pathology of X-ALD and the extreme variability of its clinical phenotypes are also unexplained. To facilitate understanding of X-ALD pathophysiology, we developed an X-ALD mouse model by gene targeting. The X-ALD mouse exhibits reduced β-oxidation of VLCFAs, resulting in significantly elevated levels of saturated VLCFAs in total lipids from all tissues measured and in cholesterol esters from adrenal glands. Lipid cleft inclusions were observed in adrenocortical cells of X-ALD mice under the electron microscope. No neurological involvement has been detected in X-ALD mice up to 6 months. We conclude that X-ALD mice exhibit biochemical defects equivalent to those found in human X-ALD and thus provide an experimental system for testing therapeutic intervention.