Primary therapeutic interventions for CF patients are pancreatic enzymes, supplementation of vitamins A, D, E, and K, and proper nutrition with supplementation of fats and highly caloric foods. Pulmonary interventions include the use of bronchodilators in conjunction with physiotherapy, and possibly even mechanical percussion vests. Pulmozyme, a recombinant DNAse mucolytic agent, helps to reduce pulmonary exacerbations and need for hospitalization. Antibacterial therapy is beneficial both as a suppressive therapy, which diminishes frequency of exacerbations and slows disease progression, and empirically with acute symptoms. The most common regimen is the combination of an aminoglycoside with a penicillin or cephalosporin for a three-week course.
Fewer options exist for Pseudomonas aeruginosa infections.
The U.S. CF foundation recommends quinolones for patients of all ages for this infection. Inhaled or aerosolized anti-pseudomonal therapy has been used for many years for early treatment or suppressive therapy. They achieve high local concentration with minimal risk of systemic toxicity. Clinical data show that high but variable sputum concentrations are achieved. This is associated with significant sustained improvement in lung function, decreased use of anti-pseudomonal antibiotics, and decreased hospitalizations.