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Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive disease that is inherited predominantly in the Caucasian population. It is one of the most common life-threatening, childhood-onset inherited diseases. Cystic fibrosis affects the entire body and impacts growth, breathing, digestion, and reproduction. In the United States, 1 in 3900 children is born with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene impairing the movement of chloride ions and resulting in production of viscous mucus.

CF progression

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Chronic pulmonary disease in the majority of CF patients results from the production of viscous mucus and impairment of mucociliary clearance due to alterations in airway surface liquid. Chronic bacterial infections in the viscous mucus result in tissue damage and a robust host inflammatory response, leading to the development of bronchiectasis and progressive lung disease. In the digestive system of CF patients, thick mucus secretions block pancreatic enzymes that help digest fats and proteins, and prevent absorption of key vitamins. Although there is no cure for CF, treatments have improved greatly in recent years. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, CF patients live, on average, to be more than 35 years old.

Chronic colonization in CF patients by an evolving spectrum of bacterial pathogens leads to debilitating inflammatory exacerbations and progressive lung damage that can be fatal. Symptoms of exacerbations include increased cough, sputum production, dyspnea, and a decline in lung function from baseline. Lung transplantation often becomes necessary for individuals with cystic fibrosis as lung function declines.

Chronic infection with Burkholderia cepacia complex and Pseudomonas aeruginosa has often been shown to be an independent risk factor for increased morbidity and premature mortality among the CF population.

Inherent antibiotic resistance of these bacterial pathogens tend to deprive patients of effective antibiotic therapy. Thus infection control plays a critical role in the management of CF lung disease.

Oil-in-water nanoemulsions have been shown to be inherently microbiocidal. Our laboratory is focused on formulating nanoemulsions that are non-toxic to the lung and are microbiocidal to the colonized pathogens, and simultaneously are also able to improve lung function and reduce pulmonary exacerbations due to mucus accumulation.

This work has been made possible by a generous donation by the Carroll J. Haas Foundation.

Cystic Fibrosis 2

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