Quality of life in cystic fibrosis patients

• Autor(a) principal: Duarte, Marta Cristina
• Data de Publicação: 2022
• Outros Autores: Chehuen Neto, José Antonio, Furtado Barbosa Felipe, Maura, Martins Moreira Elias, Carolina, Campos Dias, Alice Maria, de Freitas Batista Mendes, Pedro, Erothildes Ferreira, Renato
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: HU Revista (Online)
• Texto Completo: https://periodicos.ufjf.br/index.php/hurevista/article/view/35344
• topic: Cystic Fibrosis; Quality of Life; Psychosocial Impact; Fibrose Cística; Qualidade de Vida; Impacto Psicossocial

Introduction: Cystic Fibrosis (CF) is a rare, hereditary, multisystemic, and potentially lethal disease.Currently,with the advancement of medicine and the emergence of new therapies, CF patients are allowed to reach 40 years of age in developed countries.Objective: To assess the quality of life (QoL) ofpatients with CF to optimize their multidisciplinary care, based on criteria that impact their well-being. Material and Methods: Cross-sectional, prospective, quantitative, and exploratory study. Forty-seven interviews were collected from patients and their parents or legal guardians at a university referral center for CF in Minas Gerais, Brazil. The evaluation method was the Cystic Fibrosis Questionnaire (CFQ), associated with the analysis of the Shwachman-KulczyckiScore (SKS). Results: Our data showed that most of the CFQ domains were satisfactory (mean>50) and the SKS had good/excellent values ​​(score>71 points) in all groups. The group of patients older than 14 years had a worse QoL.Moreover, there was a divergence between the response of the 6 to 11 and 12 to 13-year group in comparison with the response of their legal guardians (p < 0.05). Conclusion: We found satisfactory means in all groups for the weight, digestive, and respiratory domains. However, the social role, vitality, emotional, and social domains had lower and decreasing means according to advancing age, then it would beparamount a multidisciplinary approach focused on these domains that most impact theirQoL. A limitation of research on rare diseases is the small sample, therefore not being possible to generalize the results. However, the analysis is still significant and relevant, demonstrating areas of impact that need to be improved.