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THE IMPLICATIONS FOR ADULT CARE IN THE EVOLVING CYSTIC FIBROSIS EPIDEMIOLOGY


The following is a summary of the “Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care,” published in the January 2023 issue of Chest by Burgel, et al.


Mutations in the CF transmembrane conductance regulator gene cause a multisystem disease characterized by digestive and respiratory symptoms. This disease is known as cystic fibrosis (CF). In the middle of the twentieth century, infant mortality due to CF was relatively common. However, recent decades have seen significant improvements in the prognosis of people with CF thanks to advances in disease management such as systematic neonatal screening, multidisciplinary symptomatic CF care, lung transplantation, and, most recently, highly effective CF transmembrane conductance regulator modulators.


Adults now outnumber children in most countries with well-established CF care, and life expectancy is expected to increase further, narrowing the survival gap with the general population. Even within high-income countries, the presence and quality of a specialized CF care provision network can make a significant difference in the prognosis of CF. The current evidence suggests that differences in patient clinical status and survival may be attributable to factors other than the inherent severity of the disease.


Adults with CF often exhibit increased pulmonary severity and complications and increased occurrence of comorbidities, highlighting the need for specialized adult CF centers due to the progressive nature of CF. This article aims to provide a historical context for CF, forecast future trends, and determine how to care for adults with CF will be delivered.


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