Cystic Fibrosis

Cystic fibrosis is a chronic multisystem disorder that results in lifelong morbidity and premature mortality. The disease is manifested by dysfunction of exocrine glands and is associated with excessively viscous secretions in nearly all mucus-secreting organs of the body. The lungs and pancreas are the main organs affected and the primary contributors to morbidity and mortality. Lung disease, the primary cause of morbidity and mortality in cystic fibrosis, is characterized by cycles of infection and inflammation that slowly damage the lungs, culminating in respiratory failure. When the pancreas is affected, it becomes obstructed, which prevents enzymes from breaking down and absorbing food. The individual then has difficulty growing normally and maintaining a healthy weight and experiences painful, unpleasant gastrointestinal symptoms. In addition, as patients with cystic fibrosis live longer, progressive destruction of pancreatic tissue results in cystic fi-brosis-related diabetes, which is becoming more common (Cystic Fibrosis Foundation 2008). Treatment regimens vary according to specific clinical manifestations, but core treatment regimens include airway clearance therapies, antibiotics, nebulized medications, replacement of pancreatic enzymes, and dietary modifications.

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