title: Direct Observation of the Behaviour of Females with Rett Syndrome.
abstract: The aim was to observe the behaviour of a sample of females with RTT and explore how it was organized in relation to environmental events. Ten participants, all with a less severe form of classic (n= 9) or atypical (n= 1) Rett syndrome (RTT), were filmed at home and at school or day centre. Analysis used real-time data capture software. Observational categories distinguished engagement in social and non-social pursuits, hand stereotypies, self-injury and the receipt of attention from a parent, teacher or carer. Associations between participant behaviour and intake variables and receipt of attention were explored. Concurrent and lagged conditional probabilities between behavioural categories and receipt of attention were calculated. Receipt of adult attention was high. Engagement in activity using the hands was associated with a less severe condition and greater developmental age. Engagement in activity, whether using the hands or not, and social engagement were positively associated with receipt of support. The extent of hand stereotypies varied greatly across participants but was independent of environmental events. Six participants self-injured. There was some evidence that self-injury was related to adult attention. Participants appeared to experience a carer and attention rich environment and their levels of engagement seemed high as a result. As in the more general literature, engagement in activity was related to personal development and to social support. Self-injury contrasted with hand stereotypies in having possible environmental function.
authors.affiliation: Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences, Cardiff University, 2nd floor Hadyn Ellis Building, Maindy Road, Cardiff, Wales CF24 4HQ UK.
authors.forename: Rina
authors.initials: R
authors.lastname: Cianfaglione
authors.affiliation: Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences, Cardiff University, 2nd floor Hadyn Ellis Building, Maindy Road, Cardiff, Wales CF24 4HQ UK.
authors.forename: Andrea
authors.initials: A
authors.lastname: Meek
authors.affiliation: Institute of Cancer & Genetics, Institute of Medical Genetics Building, Cardiff University, Heath Park, Cardiff, CF14 4XN UK.
authors.forename: Angus
authors.initials: A
authors.lastname: Clarke
authors.affiliation: Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences, Cardiff University, 2nd floor Hadyn Ellis Building, Maindy Road, Cardiff, Wales CF24 4HQ UK.
authors.forename: Michael
authors.initials: M
authors.lastname: Kerr
authors.affiliation: Centre for Educational Development Appraisal and Research, University of Warwick, Coventry, CV4 7AL UK.
authors.forename: Richard P
authors.initials: RP
authors.lastname: Hastings
authors.affiliation: Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences, Cardiff University, 2nd floor Hadyn Ellis Building, Maindy Road, Cardiff, Wales CF24 4HQ UK.
authors.forename: David
authors.initials: D
authors.lastname: Felce
keywords: Intellectual disabilities; Rett syndrome; activity; observation of behaviour; self-injury