The reproducibility and validity of the Canadian Occupational Performance Measure in parents of children with disabilities

OBJECTIVE: To study the reproducibility (inter-rater agreement), the construct and criterion validity of the Canadian Occupational Performance Measure (COPM) in the parents of children with disabilities. DESIGN: The COPM was administered twice by two different occupational therapists. The inter-rater agreement of the content of the prioritized problems was explored. Data analysis of the reproducibility of the scores was based on the Bland and Altman method.Measures used: The construct validity was studied by comparing the results of the COPM with the Pediatric Evaluation of Disability Inventory, and a quality of life questionnaire. The criterion validity was verified with an open-ended question. SETTING: Occupational therapy departments of a university hospital and three rehabilitation institutes. SUBJECTS: One hundred and twenty-nine consecutive parents of children referred for occupational therapy. RESULTS: Data were obtained for 80 children with a mean age of 3.7 years (range 1-7.5). Of the prioritized problems identified in the first interview, 80% were also prioritized in the second interview. The limits of agreement were - 2.4 to +2.3 for the mean performance score and - 2.3 to + 2.6 for the mean satisfaction score. Assumptions about the construct and criterion validity were confirmed. CONCLUSIONS: The inter-rater agreement of the prioritized problems is good enough for client-centred occupational therapy. The reproducibility of the performance and satisfaction scores is moderate. The results support the construct and criterion validity. The COPM identifies many child-unique problems that are not assessed with existing standardized measurement instruments or with a simple open-ended questio

Development and validation of a severity scoring system for Zellweger spectrum disorders

The lack of a validated severity scoring system for individuals with Zellweger spectrum disorders (ZSD) hampers optimal patient care and reliable research. Here, we describe the development of such severity score and its validation in a large, well-characterized cohort of ZSD individuals. We developed a severity scoring system based on the 14 organs that typically can be affected in ZSD. A standardized and validated method was used to classify additional care needs in individuals with neurodevelopmental disabilities (Capacity Profile [CAP]). Thirty ZSD patients of varying ages were scored by the severity score and the CAP. The median score was 9 (range 6-19) with a median scoring age of 16.0 years (range 2-36 years). The ZSD severity score was significantly correlated with all 5 domains of the CAP, most significantly with the sensory domain (r = 0.8971, P = <.0001). No correlation was found between age and severity score. Multiple peroxisomal biochemical parameters were significantly correlated with the severity score. The presently reported severity score for ZSD is a suitable tool to assess phenotypic severity in a ZSD patient at any age. This severity score can be used for objective phenotype descriptions, genotype-phenotype correlation studies, the identification of prognostic features in ZSD patients and for classification and stratification of patients in clinical trials

Development and validation of a severity scoring system for Zellweger spectrum disorders

The lack of a validated severity scoring system for individuals with Zellweger spectrum disorders (ZSD) hampers optimal patient care and reliable research. Here, we describe the development of such severity score and its validation in a large, well-characterized cohort of ZSD individuals. We developed a severity scoring system based on the 14 organs that typically can be affected in ZSD. A standardized and validated method was used to classify additional care needs in individuals with neurodevelopmental disabilities (Capacity Profile [CAP]). Thirty ZSD patients of varying ages were scored by the severity score and the CAP. The median score was 9 (range 6-19) with a median scoring age of 16.0 years (range 2-36 years). The ZSD severity score was significantly correlated with all 5 domains of the CAP, most significantly with the sensory domain (r = 0.8971, P =  <.0001). No correlation was found between age and severity score. Multiple peroxisomal biochemical parameters were significantly correlated with the severity score. The presently reported severity score for ZSD is a suitable tool to assess phenotypic severity in a ZSD patient at any age. This severity score can be used for objective phenotype descriptions, genotype-phenotype correlation studies, the identification of prognostic features in ZSD patients and for classification and stratification of patients in clinical trial