Cystic fibrosis is an autosomal recessive disease that affects 1 in 3,300 live births. It is the most common life-shortening inherited disease among Caucasians. This population has a wide array of clinical manifestations that include obstructive pulmonary diseases, high-sweat electrolyte concentrations, as well as sinus, endocrine, and reproductive disorders. Because there is complex multisystem involvement, a thorough understanding of the pathophysiology will help the anesthesia provider better understand anesthetic management for the adult CF patient.
CF is characterized by the failure of chloride transport in the epithelial plasma membranes due to a gene mutation of the CF transmembrane–conductance regulator (CFTR). This defect affects the body’s ability to secrete sodium, chloride, and water across the epithelial membrane. The result is predominantly manifested in the airways where the CFTR cannot adequately secrete chloride and bicarbonate, consequently producing viscous mucus that blocks small airways.
Dyspnea, chronic cough, purulent sputum, nasal polyps, chronic sinusitis, and frequent upper respiratory tract infections are common in CF patients. The lower respiratory system of the CF patient has an abundance of neutrophils associated with chronic infection. Thick mucus impairs the tracheobronchial clearance of secretions and leads to bacteria trapping in the lungs. As the patient ages, a regular bacterial infection results that may become resistant to antibiotic therapy. CF patients also show an increase in hypersensitivity (allergic) reactions to antibiotics that creates further treatment problems.
In addition to these, the patient with CF is also at risk for developing non-pulmonary complications. These complications include pancreatic insufficiency with blockage of the pancreatic ducts from mucus. Mucous may also cause bile-duct obstruction, leading to hepatic cirrhosis and portal hypertension. Treatments for CF are directed toward preventing lung disease and undernutrition. Daily management includes pulmonary physiotherapy, pharmacologic therapy, and consumption of a high-calorie diet. This regimen requires vigilance on the part of the adult patient.
