Occupational Performance of Children and Adolescents with Mucopolysaccharidosis Using Assistive Technologies

Abstract

Mucopolysaccharidoses (MPS) are a specific group of genetic diseases in which due to the accumulation of glycosaminoglycans (GAGs) in different organs and tissues, causes multisystemic changes that compromise the functionality and occupational performance of individuals. Occupational performance, understood as the participation and execution of activities of daily living, may be favoured using Assistive Technology (AT). Since there are no studies reporting the influence of AT on the occupational performance of children and adolescents with MPS, the objective of this study was to evaluate the occupational performance in self-care activities, based on the use of low-cost AT in children and adolescents with Mucopolysaccharidosis. Six individuals with MPS types I, IV-A and VI, aged 9 to 16 years participated. The instruments used for data collection were the Pediatric Disability Assessment Inventory (PEDI) self-care area only, and the Canadian Occupational Performance Measure (COPM). The results showed that the tasks that presented the greatest disabilities in the performance are the areas of dressing, personal hygiene and bath. Thus, TA resources were made for five activities related to dressing and one for personal hygiene. After the use of AT, there was a positive and significant change in occupational performance and satisfaction of these individuals. Thus, the use of AT can significantly improve the occupational performance of this population.