Date of Award

2011

Document Type

Thesis

Publisher

Edith Cowan University

Degree Name

Doctor of Philosophy

School

School of Exercise, Biomedical and Health Sciences

Faculty

Faculty of Computing, Health and Science

First Supervisor

Dr Peter Roberts

Abstract

The research project undertaken as part of this thesis was designed to assess family stress levels and the use of coping strategies among the carers of people with an intellectual disability caused by Angelman syndrome (AS) or Prader-Willi syndrome (PWS). Both syndromes are genomic imprinting disorders that arise from disruptions in genes located within human chromosome 15q11-q13. Although the disease phenotypes are quite distinct, the genetic mechanisms involved are common to both syndromes but involve paternally-derived mutations in PWS as opposed to maternal mutations in AS. Previous investigations in Western Australia (WA) indicated that people with AS and PWS experience substantial ill health over the life course, and require more frequent hospital-based care than their typically-developing peers (Thomson, 2005; Thomson, et al., 2006a; b; Thomson, et al., 2007). A high proportion of the people identified in the earlier study were resident in the family home and many relied on family carers for assistance with activities of daily living. Studies of the families of people with intellectual and developmental disabilities (IDD) from other causes have shown that family carers often experience considerable stress through the caring role, especially as their offspring reach adulthood and beyond (e.g., Baxter, et al., 2000; Benson & Karlof, 2009; Glidden & Natcher, 2009). Information regarding the family carers of people with AS and PWS is limited and therefore this study examines the family aspects of caring for individuals with these disorders. Participants were recruited by means of invitation letters sent by staff of Disability Services WA and Genetic Services WA, and by presentations by the candidate to the Western Australian branches of the Angelman Syndrome Association and the Prader-Willi Association. Data were collected using postal questionnaires and by face-to-face interview with family carers. The detailed information collected at individual level on people with AS (n = 11) or PWS (n = 5) included demographic data, the nature and extent of their care needs, and their clinical and behavioural profiles. Family carers (n = 21) also provided personal demographic information, rated their own life satisfaction and health levels, and participated in the Family Stress and Coping Interview (FSCI).

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