Cystic Fibrosis
Key Points
- Cystic fibrosis is an autosomal recessive disease caused by CFTR dysfunction.
- Thick, sticky secretions obstruct airways and increase recurrent respiratory infection risk.
- Pancreatic duct obstruction reduces digestive-enzyme delivery and can cause malabsorption.
- Diagnosis commonly uses newborn screening plus sweat chloride and genetic testing.
- Long-term care combines airway clearance, nutrition support, mutation-targeted therapy, and infection prevention.
Pathophysiology
CF results from inherited mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Defective chloride transport leads to dehydrated secretions that become thick and difficult to clear.
In the lungs, retained secretions narrow airways and support chronic infection-inflammation cycles that can progressively damage lung tissue. In the pancreas, thick secretions block enzyme flow into the intestine, reducing nutrient absorption and growth.
Classification
- Pulmonary-dominant pattern: Recurrent cough, mucus retention, infection exacerbations, and chronic lung decline.
- Pancreatic/nutritional pattern: Malabsorption with weight-gain and nutrition challenges.
- Mutation-targeted therapy eligible pattern: Selected CFTR variants that respond to CFTR modulators.
Nursing Assessment
NCLEX Focus
Prioritize airway clearance, oxygenation trend, and nutrition status in every CF assessment cycle.
- Assess cough pattern, sputum burden, dyspnea, and breath-sound changes.
- Monitor fever and exacerbation cues suggesting acute respiratory infection.
- Track weight trend, appetite, stool pattern, and signs of malabsorption.
- Review newborn-screening history, sweat chloride results, and genetic-testing status.
- Assess medication adherence and technique for inhaled and mucus-thinning therapies.
- Monitor functional status and fatigue progression to identify worsening pulmonary reserve.
Nursing Interventions
- Support airway-clearance routines and secretion-management therapies as ordered.
- Administer and monitor prescribed bronchodilators, mucolytics, anti-inflammatory agents, and antibiotics.
- Coordinate nutrition-focused care planning for absorption challenges and growth support.
- Reinforce infection-prevention practices, including hand hygiene and symptom-reporting thresholds.
- Teach family and patient how mutation-targeted medications (CFTR modulators) fit the overall plan when prescribed.
- Escalate for worsening respiratory distress, refractory hypoxemia, or frequent exacerbations.
- Reevaluate outcomes after each reassessment and update the care plan when goals are partially met or unmet.
Exacerbation Progression Risk
Delayed response to increased sputum burden or breathing effort can accelerate lung damage.
Pharmacology
| Drug Class | Examples | Key Nursing Considerations |
|---|---|---|
| bronchodilators | Inhaled bronchodilator regimens | Improve airflow before airway-clearance interventions. |
| Mucus-thinning therapies | Ordered mucolytic regimens | Support secretion mobilization and airway clearance. |
| anti-inflammatory-medications | Ibuprofen or corticosteroid pathways | Reduce airway inflammation burden in selected care plans. |
| antibiotics | Intermittent or continuous regimens | Target respiratory pathogens and reduce exacerbation frequency. |
| CFTR modulators | Mutation-specific combinations | Use only for eligible genotypes; monitor response and tolerance. |
Clinical Judgment Application
Clinical Scenario
A school-aged client with CF has increased cough, thicker sputum, fatigue, and reduced appetite over several days.
- Recognize Cues: Pulmonary and nutrition decline pattern with possible exacerbation.
- Analyze Cues: Airway obstruction and infection risk are increasing.
- Prioritize Hypotheses: Highest priorities are oxygenation protection and early infection management.
- Take Action: Intensify assessment, implement ordered respiratory therapies, and escalate for provider reevaluation.
- Evaluate Outcomes: Breathing effort decreases, sputum clearance improves, and intake begins to recover.
Related Concepts
- respiratory-system - Airway and gas-exchange framework for chronic obstruction risk.
- genetic-influences-on-cardiopulmonary-disorders - Inherited cardiopulmonary disease context.
- pneumonia - Frequent infectious exacerbation linkage in chronic secretion retention.
- oxygen-therapy - Escalation support during pulmonary decline.
Self-Check
- Why does CFTR dysfunction cause both pulmonary and gastrointestinal effects?
- Which findings most strongly suggest a pulmonary exacerbation?
- Why must nutrition surveillance be integrated with respiratory care in CF?