Cystic Fibrosis
Cystic Fibrosis (CF), a life-shortening, predominantly Caucasian genetic disease primarily affects the respiratory and digestive systems in approximately 70,000 patients in North America and Europe. CF causes the production of abnormally thick mucus secretions in the lungs, leading to life-threatening lung infections. These secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help digest and absorb food.
The pathophysiology of lung disease in CF is characterized by a repetitive cycle that begins with obstructed airways which inevitably leads to infected and inflamed airways, which if left untreated causes significant loss of lung function. Pseudomonas aeruginosa (Pa) is the most prevalent opportunistic pathogen in CF and leads to progressive destruction of lung tissue via the inflammatory process. Approximately two-thirds of all CF patients have Pa. Patients infected with Pa can lose as much as 5% of lung function per year.
Current standard of care therapies in CF are used both intermittently and chronically to minimize airway obstruction and control Pa infection and thereby reduce the resulting inflammatory stimulus. The goal of intervention is to retard disease progression by maintaining or improving pulmonary function, increase time
Between pulmonary exacerbations and reduce the severity of pulmonary exacerbations.
For more information about cystic fibrosis contact either the American Lung Association at 1-800-LUNG-USA (www.lungusa.org), the Cystic Fibrosis Foundation at (800) FIGHT CF (www.cff.org), or the Boomer Esiason Foundation (www.esiason.org).
