Optimising respiratory health in children with cystic fibrosis

Cystic fibrosis is a multi-system genetic disorder causing thick secretions, lung infection and pancreatic insufficiency. Optimising respiratory health in children with cystic fibrosis depends upon meticulous attention to maintaining general health, in addition to preserving lung health. Maximising nutrition and growth are essential as these are independent predictors of lung function and survival.Neonatal screening has enabled an earlier, more proactive approach to optimising health. However the primary predictor of deterioration is the acquisition of the opportunistic bacterium Pseudomonas aeruginosa. Eradication of chronic infection with this organism is impossible, leading to lung destruction and shortened life expectancy for individuals with CF. The optimal strategies for managing this critical complication of cystic fibrosis are the subject of ongoing research, however these strategies may depend upon antibiotic regimens to which the bacteria may gain resistance. Novel strategies, adopted alongside continued improvements in care, are needed to further defer the complications and deterioration experienced by patients with cystic fibrosis, enhance quality of life and extend survival.