The evolution of cystic fibrosis : therapeutic approaches for adult patients

Abstract

Introduction: Cystic Fibrosis (CF), also known as mucoviscidosis, is a hereditary disease with an autosomal recessive pattern and an early onset, affecting children in the first years of life. Patients with this condition produce thick and viscous mucus secretions, leading to obstruction of the ducts of exocrine glands. This contributes to three main characteristics: chronic obstructive lung disease, elevated electrolyte levels in sweat, and pancreatic insufficiency, resulting in poor digestion, malabsorption, and consequent secondary malnutrition. The prognosis of CF has improved over the past decades, allowing patients to reach adulthood with well-established care for the disease. This progress has been achieved through the implementation of multidisciplinary care in specialized centers, including the use of systemic and inhaled antibiotics, airway clearance therapy, pancreatic enzyme replacement, and a high-calorie diet. Main objective: The primary objective of the research is to discourse about the pathology and the importance of specialized treatment. Materials and methods: as methodology will be used the bibliographic survey through the main online collections (PubMed, SciElo, BVS), using a filter to select articles with greater relevance within from 2006 to 2025. Results: It is noted that the development of CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) protein modulators improved lung function and significantly increased the life expectancy of patients. Conclusion: It is concluded that early diagnosis, combined with an individualized therapeutic approach, is essential to improve the prognosis and provide a better quality of life for patients with Cystic Fibrosis.