RELIABILITY & VALIDITY PRPP-ASSESSMENT IN CHILDREN 157 7 Introduction Mitochondrial disorders are rare diseases that influence the cellular energy metabolism. These disorders are one of the most common inherited errors of metabolism that can be caused by mutations in more than 230 different genes [1, 2]. This genetic heterogeneity is also reflected in the large range of symptoms and impairments associated with mitochondrial disorders, varying frommotor impairments (such as muscle weakness or balance problems) to cognitive impairments (such as concentration problems and intellectual disability) [3, 4, 5, 6]. These impairments have a tremendous impact on daily functioning and influence participation in school, leisure activities, the neighborhood, and the community[6]. It is a challenge for clinicians to assess functioning of children with mitochondrial disorders with ‘standardized’ assessments due to the heterogenicity of the population. Best practice should include assessment of the child’s ability to perform meaningful activities and evaluate the specific motor and/or cognitive difficulties that impact on task performance. Ideally, assessments should inform how well children can participate according to their own needs, in line with personalized and value-based care[7]. Furthermore, norm-referenced tests do not provide the information needed for personalized and value-based care, and most existing tests are not applicable due to the heterogeneity of the group or that these children do not perform activities in the ‘standardized’ way[8]. In contrast, criterion-referenced measures focus on a more individualized outcome. Each child’s performance level is not compared to others[9], but is assessed against what is required for them to achieve their personalized goal. Criterion-based assessments are encouraged in pediatric occupational therapy as they can contribute to client centered practice and allow self-determination and self-definition[10, 11], and overcome difficulties in standardized testing of children with atypical development[12, 13, 14]. Hence, information gained from criterion-referenced assessments can guide personalized treatment, which makes the outcome more clinically relevant. Therefore, criterion-referenced tests in which children are compared to a personalized criterion seem more appropriate for the heterogenic group of children with mitochondrial disorders[9]. A suitable criterion-referenced assessment for measuring quality of performance in children with limited cognitive and communication abilities could be the Perceive, Recall, Plan, and Perform (PRPP)-System of Task Analysis[15]. This assessment is used by occupational therapists (OT) to gain insight into occupational performance by observing children performing meaningful activities. When administering the PRPP-Assessment, the OT observes and analyses in a structured way what children do, how they do it and which cognitive strategies they use. Cognitive strategies are essential to human functioning as they are the thinking processes required to think
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