Cystic Fibrosis: Clinical, Epidemiological, and Care Aspects and the Role of Respiratory Physiotherapy

Abstract

Introduction: Cystic fibrosis is a chronic, multisystemic genetic disease characterized by alterations in the CFTR gene, leading to the production of thick, viscous secretions, particularly in the respiratory system. Objective: To analyze the evidence regarding the clinical, epidemiological, and care-related aspects of cystic fibrosis, with an emphasis on the role of respiratory physiotherapy. Methods: This is an integrative literature review of a qualitative and descriptive nature. The search was conducted in the PubMed, SciELO, and Virtual Health Library (VHL) databases using descriptors related to cystic fibrosis, respiratory physiotherapy, airway clearance techniques, and pulmonary function, combined using Boolean operators. Studies available in full text, in Portuguese and English, addressing aspects related to physiotherapy practice, pulmonary assessment, and care for patients with cystic fibrosis were included. Following the identification, screening, and eligibility process, five studies comprised the final sample. Results: The analyzed studies presented diverse methodological designs and addressed aspects related to respiratory physiotherapy, pulmonary function tests, care in specialized centers, family care, and the epidemiological characteristics of cystic fibrosis. Findings indicated that bronchial hygiene techniques, positive expiratory pressure devices, breathing exercises, and other physiotherapy strategies can contribute to secretion clearance, improved pulmonary ventilation, and the maintenance of functional capacity. Periodic assessment of pulmonary function also proved relevant for monitoring clinical progression and guiding therapeutic approaches. Furthermore, challenges were identified regarding treatment adherence, caregiver burden, inequalities in access to specialized centers, and the need for comprehensive multidisciplinary teams. However, the heterogeneity of the studies and the outcomes evaluated limits the definition of standardized physiotherapy protocols. Conclusion: Pulmonary physiotherapy plays a significant role in the care of patients with cystic fibrosis, particularly in the management of respiratory secretions, the maintenance of lung function, and the preservation of functional capacity. However, results should be interpreted with caution due to the methodological diversity of available studies. Therefore, further clinical studies with greater methodological rigor and well-defined protocols are needed to strengthen the evidence base and support more individualized and effective physiotherapeutic strategies.