Cystic fibrosis
Structured condition card with NCLEX priority cues and nursing action focus.
Autoimmune / GeneticRespiratoryGI / Liver / PancreasPediatricshigh priorityneeds review
Cystic fibrosis
Also testable as: CF
Etiology / Pathophysiology
- Inherited CFTR gene disorder affects chloride transport and secretions.
- Thick sticky mucus blocks airways and pancreatic ducts, causing lung infection risk and malabsorption.
Medications
| Class | Why it matters |
|---|---|
| Bronchodilators | May support airway clearance when ordered. |
| Antibiotics by class | Used for bacterial pulmonary infections or prophylaxis in selected plans. |
Nursing actions
- Assess respiratory effort, cough/sputum, oxygenation, growth, stools, hydration, and infection signs.
- Support airway clearance, pancreatic enzyme timing with meals/snacks if ordered, high-calorie nutrition, and infection prevention.
- Teach salt/fluid needs, medication adherence, and when to report respiratory decline or fever.
Complications
- Bronchiectasis
- Respiratory failure
- Malnutrition
- Pancreatic insufficiency
- Diabetes
NCLEX cues
- Thick mucus plus recurrent respiratory infections.
- Greasy bulky stools indicate malabsorption.
- Pancreatic enzymes with meals/snacks.
Memory hooks
- CF clogs lungs and pancreas.
Labs / Diagnostics
- Sweat chloride test
- Newborn screening
- Sputum cultures
- Pulmonary function testing
- Weight/growth trends
Review notes
- Supplemental wife-requested study card. Use for NCLEX review only and verify against school materials, ATI/NCLEX review sources, current orders, and facility policy.