Relação entre a funcionalidade, gravidade clínica e respiratória de crianças e adolescentes com fibrose cística

Abstract

Introduction: Cystic fibrosis (CF) is a chronic genetic condition with pulmonary and systemic repercussions that require extensive self-care and management to prevent rapid disease progression. In view of the multisystem characteristics and related functional repercussions, a multidimensional evaluation such as the Pediatric Evaluation of Disability Inventory Computer Adaptive Test (PEDI-CAT), which provides information from different domains, such as the activity and participation of the International Classification of Functionality, Disability and Health (CIF ), is desirable and timely for the assessment of CF children and adolescents, as well as to help guide intervention and follow-up strategies. Objectives: To evaluate the relationship between functioning, clinical and respiratory severity of children and adolescents with CF. Materials and method: This was an observational, cross-sectional study involving children and adolescents from 6 to 21 years old, with confirmed CF diagnosis, followed up at the CF outpatient clinic of the UFJF University Hospital. Participants were assessed for demographic, anthropometric, clinical, spirometry, six-minute walk test (6MWT), and Shwachman-Kulczycki severity score. The caregivers of these participants answered the PEDI-CAT functioning inventory based on an interview, on a laptop computer. Results: The participants were, on average, 11.62 ± 4.60 years old, 73% of whom were boys. All PEDI-CAT domains were moderately related to forced expiratory volume in the first second (r = 0.46 - 0.57, p < 0.05), and the mobility domain was moderately related to clinical severity (r = 0. 48, p = 0.01). No relationship was observed between the PEDI-CAT domains and the 6MWT outcomes. Participants with respiratory severity mild and moderate-severe showed significant difference at PEDI-CAT responsibility domain. Conclusion: Functioning and disability are associated with respiratory and clinical severity in children and adolescents with CF. PEDI-CAT is a comprehensive functional assessment tool to be used in this pediatric clinical population and capable of detecting difference at responsibility according to level of respiratory severity.