Pediatrics
Cystic Fibrosis
An autosomal recessive genetic disorder causing thick, viscous secretions in the respiratory, digestive, and reproductive systems.
Respiratory Nursing
- Chest physiotherapy and postural drainage
- Encourage aerobic exercise and deep breathing
- Administer dornase alfa (DNase) to thin secretions
- Monitor for hemoptysis or pneumothorax
Dietary Needs
- High-calorie, high-protein diet
- Fat-soluble vitamin supplementation (A, D, E, K)
- Pancreatic enzyme replacement with all meals and snacks
Diagnostic Test
- Sweat chloride test (>60 mEq/L is diagnostic)
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