Unveiling epidemiology and treatment strategies for efficient management of lung infection in cystic fibrosis

Cystic Fibrosis (CF) is a genetic disorder which results in abnormal Cystic Fibrosis Transmembrane Regulator (CFTR) protein. CFTR protein actively transports chloride and bicarbonate out of cells which produce mucus in epithelium of various organs. However, when there is an abnormality in this protein then ion and water transport across airway surface is affected and form viscous mucus due to dehydration. This disease affects lung, pancreases, intestine and hepatobiliary system. Infection of lungs is main reason of high morbidity and mortality. Patients with CF are more prone to bacterial infection of the respiratory tract. Mainly four bacterial pathogens are found in the respiratory tract of CF patient viz. Staphylococcus aureus, Haemophilus influenzae, Burkholderia cepacia, Pseudomonas aeruginosa. Mucoid P. aeruginosa is the most dangerous pathogen among them due to ability to form biofilm. There are many conventional and new treatment options available for CF patients. New therapies are used to correct the CFTR protein and antibiotic treatment is used to prevent or early infection or suppression of chronic infection. However, when P. aeruginosa turn into mucoid form, formation of extracellular polysaccharide biofilm starts with alginate as major constituent. The antibiotics fail to reach the target as EPS of the biofilm shield the microbe from antibiotics action. Alginate lyase, an alginate degrading has the ability to break the alginate and subsequently increases the efficacy of antibiotics therapy. The co-administration of alginate lyase and Dnase along with antibiotics has proved to be very efficient in the management of patients with CF.