Cystic Fibrosis: NCLEX Priorities
CF: thick mucus, pancreatic enzymes, chest physiotherapy, CFTR modulators.
CF is an autosomal recessive disorder affecting CFTR. NCLEX tests airway clearance, pancreatic enzymes, nutrition.
Pathophys
CFTR mutation → thick secretions in lungs, pancreas, sweat glands. Recurrent pulmonary infections, malabsorption, salty sweat.
Pulmonary management
Chest physiotherapy (CPT, vest, autogenic drainage) BID. Mucolytics (dornase alfa, hypertonic saline). Bronchodilators. Antibiotics for exacerbations.
Pancreatic enzymes
Take with every meal/snack. Don't crush. Improves fat absorption. Fat-soluble vitamins (ADEK) supplemented.
Nutrition
High-calorie, high-protein diet. Salt supplementation in hot weather. Often require enteral feeds.
CFTR modulators
Ivacaftor, elexacaftor/tezacaftor/ivacaftor (Trikafta) for eligible mutations. Game-changing improvement in lung function.
Frequently asked
Sweat chloride test?
Diagnostic. >60 mmol/L confirms CF.
Why high-calorie diet?
Malabsorption + increased metabolic demand from chronic infection.
Quick check
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