CYSTIC FIBROSIS IN LIMITED RESOURCE SETTINGS

Cystic fibrosis (CF) has been shown to affect people all over the world. While life expectancy for people with CF has increased substantially, CF is still associated with death in infants and young children in many regions, particularly in countries with limited resources. These countries face significant challenges to promote CF diagnosis and improvements to CF care due to financial constraints and a significant burden of other diseases. This chapter describes the status of CF diagnosis and care in limited resource settings, highlighting challenges and opportunities for CF practitioners in these areas to improve CF care and outcomes. While early CF diagnosis is the key to optimising outcomes, newborn screening may not be feasible for countries with limited resources. CF therapies and care in limited-resource settings need to be adapted to what is available in these countries. Most individuals with CF in countries with limited resources are not benefiting from CF transmembrane conductance regulator (CFTR) modulator treatments due to the prohibitive cost of these drugs. Disparities in CF outcomes between rich and poor countries will continue to widen if access to affordable CFTR modulator drugs is not equitable. Collaborative initiatives between global CF organizations, communities and countries with limited resources will improve CF care in these regions.