ein in people with cystic fibrosis. As a result, one hallmark of CF is the presence of a thick mucus secretion which clogs the bronchial tubes in the lungs and plugs the exit passages from pancreas and intestines, leading to loss of function of these organs. Progressive lung disease is the predominant cause of illness and death in people with CF. Mucus blocks the airway passages and results in a predisposition toward chronic bacterial infections. Although the genetic defect underlying CF has been characterized, exactly how and why individuals become infected with Pseudomonas is unknown. The lungs of most children with CF become colonized by Pseudomonas aeruginosa before their 10th birthday. Chronic infection with these bacteria reduces an individuals quality of life, causing acute symptoms of cough, sputum production, and inflammation, which causes repeated exacerbations or episodes of intense breathing problems. Eventually leading to scarring and destruction of lung tissue and, ultimately, death. While it is clear that antibiotic therapy directed against these organisms lengthens the life span of individuals with CF, increasing antibiotic resistance develops. Although antibiotics can decrease the frequency and duration of these attacks, the bacterium establishes a permanent residence and can never be completely eliminated from the lungs. Management of cystic fibrosis lung disease requires a multipronged approach. Outpatient management of pulmonary exacerbation usually includes a combination of 2 IV antipseudomonal antibiotics (an aminoglycoside plus a beta-lactam), appropriate antimicrobial treatment, effective airway clearance, optimization of nutritional status, and anti-inflammatory therapies. Additionally, prevention of respiratory viral disease and avoidance of exposure to irritants, such as smoke, is recommended. Usual duration of therapy is 14 to 21 days, and clinical response is assessed by physical exam, pu...
