Thesis

EXTENSIVE SUMMARY FOR EDUCATIONAL PURPOSES 259 10 calculated with ICCAgreement. The ICCAgreement for stage 1 Mastery Score showed acceptable inter-rater reliability (0.646), where other aspects of the PRPP-Assessment had low ICCs. The low ICCs could be explained by a lack of variability within the sample and a rather good performance of most children. In addition, the standard deviation of the variance component of raters was lower than the variance of the children which indicates acceptable inter-rater reliability. Construct validity was assessed by comparing the PRPP-Assessment scores to the COPM scores (convergent validity) using Cohen’s kappa and descriptive statistics. PRPP-Assessments scores were evaluated with multi-faceted Rasch Analysis and compared with the information processing model and expert opinions (structural and known group validity). Six hypotheses were formulated a priori, fitting with the guidelines of the COSMIN47-50. Four out of six predefined hypotheses could be accepted, demonstrating good structural and known group validity. Convergent validity based on comparing COPM and PRPP could not be established. The low level of agreement between ratings on the COPM and the PRPP-Assessment was thought to be attributed to parents using a ‘normative’ frame of reference instead of the ‘criterion’ frame of reference that characterizes the PRPP-Assessment. Overall, Rasch analysis demonstrated sound goodness-of-fit, and supported the validity of the PRPP-Assessment in the heterogenic group of children with mitochondrial disorder. We concluded that the PRPP-Assessment showed sufficient to good reliability and validity in this heterogenic complex group of children. We suggested that when using the PRPP-Assessment with a heterogenic group of children, attention should be paid to a valid task selection and analysis (stage 1); the task should contain enough cognitive challenge, and should adhere to the checklist with criteria26. Chapter 8 – Potential of the PRPP-Assessment for measuring change in everyday activities by home -based videos in children with mitochondrial disorder In chapter 8 we describe a multiple case series which primarily focuses on exploring the responsiveness of the PRPP-Assessment and secondly describes the feasibility of the PRPP-Intervention in a home-based video coaching program in children with mitochondrial disorder. With this study we aimed to show the potential of the PRPP-Assessment as an outcome measure to use as a base for designing tailor made person-centered care. Out of 14 eligible children, three children completed the study in which they were measured pre and post intervention in a longitudinal multiple case series design. The children varied in age and functional abilities and had a genetically proven mitochondrial disorder.

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