Progressive Pulmonary Disease in CF
Cystic fibrosis (CF) is a life-threatening genetic disorder that results in the accumulation of thick, sticky mucus in the lungs, clogging airways and leading to infection and chronic inflammation. Moreover, because of the inability to clear the airways, bacteria colonize and form biofilms that are difficult for antibiotics to penetrate, which in turn leads to inflammation.
The accumulation of mucus and biofilms which lead to chronic infections and inflammation are key drivers of pulmonary exacerbations. Biofilm growth in progressive lung disease is associated with an increased frequency of mutations, slow growth and adaptation of the life-threatening bacteria to the conditions in the lungs, and to antibiotic therapy.
- Recurrent, intractable infections in patients with CF lead to frequent pulmonary infections and exacerbations resulting in a decline in pulmonary function and, ultimately, cause premature death.
- Current treatment options for complications of progressive pulmonary disease and pulmonary exacerbations are limited and require the use of multiple products.
CF results in destruction of lung tissue, lung fibrosis, pancreatic insufficiency, CF-related diabetes, malabsorption, malnutrition, growth retardation, and liver disease, including cirrhosis.
Learn more about how a rationally designed, broad spectrum mechanism of action therapeutic holds potential in reducing infection, mucus and biofilm accumulation, airway congestion and inflammation–key drivers of pulmonary exacerbations, symptoms and pulmonary decline in CF and other progressive pulmonary diseases. Go to page >
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