title: Rigidity in routines and the development of resistance to change in individuals with Prader-Willi syndrome.
abstract: Individuals with Prader-Willi syndrome (PWS) commonly show debilitating resistance to change, which has been linked to cognitive deficits in task switching. Anecdotal reports suggest that exposure to flexibility in routines during development may be beneficial for limiting subsequent resistance to change in people with PWS, which is consistent with a beneficial role of such exposure on the development of task switching, highlighted in typical children. Here, we aim to investigate the development of resistance to change in individuals with PWS and hypothesise that exposure to increased rigidity in routines will be associated with increased subsequent resistance to change.
  
  
  
  An author-compiled informant report interview and two previously validated questionnaires were administered to the caregivers of 10 individuals with PWS (5-23 years). The interview examined rigidity in routines and resistance to change across life stages defined by easily distinguishable events (before school, during primary school, during secondary school, after school, currently), using open-ended and structured yes/no and 5-point Likert questions. Open-ended data were coded using an author-compiled system. Responses from two additional informants and data from the questionnaires were used to assess inter-informant reliability and concurrent validity of the structured questions.
  
  
  
  The validity of the interview was supported by acceptable inter-rater reliability of the open-ended coding system and inter-informant reliability, internal consistency and concurrent validity of structured questions. Descriptive analyses of ratings of behaviour change showed a pattern of increasing resistance to change over the life course for the four oldest individuals, who had all been exposed to substantial rigidity in routines before and during primary school. Furthermore, only one individual - currently in primary school - was exposed to very little rigidity in routines before and during primary school, and he had showed a decrease in resistance to change after entering primary school. Open-ended data showed that more individuals currently evidencing little resistance to change had been exposed to parent or self-imposed flexibility in routines, than those currently evidencing substantial resistance to change. However, correlational analyses on rigidity and resistance to change ratings highlighted the possibility that rigidity during primary school is most relevant for developing resistance to change. Finally, open-ended data emphasised an important beneficial role of rigidity in routines for limiting the current challenging behaviour of individuals with high resistance to change.
  
  
  
  Because task switching appears to evidence a period of high developmental sensitivity during early primary school years, we propose that this period may represent a critical time when increasing flexibility in the routines of children with PWS could limit the development of resistance to change. However, a careful balance would need to be struck, given the apparent benefit of rigid routines on current behaviour. Further work in this area is much needed.
authors.affiliation: School of Psychology, Queen's University Belfast, Belfast, UK.
authors.forename: E L
authors.initials: EL
authors.lastname: Haig
authors.affiliation: School of Psychology, Queen's University Belfast, Belfast, UK.
authors.forename: K A
authors.initials: KA
authors.lastname: Woodcock
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D056344:Executive Function / Q000502:physiology*; D006801:Humans; D007407:Interviews as Topic / Q000592:standards*; D011218:Prader-Willi Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D011594:Psychometrics / Q000295:instrumentation; D015203:Reproducibility of Results; D055815:Young Adult
keywords: Prader-Willi syndrome; autism spectrum disorder; behavioural flexibility; challenging behaviour; insistence on sameness; task switching