What is the treatment for acute shortness of breath in cystic fibrosis?

Treatment of Acute Shortness of Breath in Cystic Fibrosis

For acute shortness of breath in cystic fibrosis, intensified airway clearance therapy combined with bronchodilators should be the first-line treatment, with consideration of non-invasive ventilation for severe cases.

Initial Management

Airway Clearance Therapy

• Airway clearance therapy should be immediately intensified as part of treating acute shortness of breath 1
• Increase both the duration and frequency of airway clearance sessions 1
• Consider the following techniques:
  • Positive Expiratory Pressure (PEP) therapy is preferred due to its effectiveness and ability to be self-administered 2
  • For patients with weak cough or excessive secretions, mechanical insufflation and exsufflation should be used alongside standard physiotherapy techniques (Grade B recommendation) 1
  • In severe cases, mini-tracheostomy may aid secretion clearance (Grade D recommendation) 1

Bronchodilator Therapy

• Administer bronchodilators before airway clearance to mobilize secretions 2
• Both β-agonists and anticholinergic drugs should be assessed separately and in combination 1
• For acute episodes, bronchodilators can be given via a nebulizer inserted into ventilator tubing if the patient is dependent on NIV 1
• Consider heated humidification if the patient reports mucosal dryness or if respiratory secretions are thick 1

Respiratory Support for Moderate to Severe Shortness of Breath

Non-Invasive Ventilation (NIV)

• NIV is the treatment of choice when ventilatory support is needed in CF patients (Grade C recommendation) 1
• Start NIV when pH < 7.35 and pCO2 > 6.5 kPa persist despite optimal medical therapy (Grade A recommendation) 1
• Monitor for complications such as mask-related issues and pneumothorax 1
• If the patient is agitated or distressed on NIV, intravenous morphine 2.5-5 mg (± benzodiazepine) may provide symptom relief and improve NIV tolerance 1

Oxygen Therapy

• Use controlled oxygen therapy targeting saturations of 88-92% in all causes of acute hypercapnic respiratory failure (Grade A recommendation) 1

Pharmacological Management

Antibiotics

• Consider oral antibiotics for unexplained prolonged cough (>2 weeks) 1
• For severe exacerbations, intravenous antibiotics may be necessary, preferably administered in a hospital setting 1
• Standard approach for treating Pseudomonas aeruginosa is to use two antipseudomonal drugs 1
• Nebulized antibiotics can be selectively offered for positive respiratory cultures 1

Mucolytics

• Dornase alfa (rhDNase) should be considered for patients with inflammation of lower airways 2, 3
• Continue chronic therapies for maintenance of lung health during treatment of an acute exacerbation (Grade B recommendation) 1

Special Considerations

Severe Cases

• If intubation is not intended should NIV fail, sedation/anxiolysis is indicated for symptom control in the distressed patient (Grade D recommendation) 1
• In patients with CF who develop acute hypercapnic respiratory failure, NIV is preferred over invasive mechanical ventilation 1
• For patients dependent on NIV, specialized physiotherapy is needed to aid sputum clearance (Grade D recommendation) 1

Treatment Setting

• Consider hospitalization for severe shortness of breath, especially in patients with comorbidities or severe exacerbations 1
• The CF Foundation recommends against delivery of intravenous antibiotics in a non-hospital setting unless resources and support equivalent to the hospital setting can be assured (Grade I recommendation) 1

Monitoring Response to Treatment

• Regularly assess for potential complications of NIV 1
• Monitor for improvement in respiratory symptoms, pulmonary function, and oxygen saturation
• For patients with CF and unexplained prolonged cough, selectively offer oral antibiotics 1
• If pneumothorax develops, intercostal drainage is usually required, and continuation of NIV should be reviewed 1

Common Pitfalls to Avoid

• Avoid overtightening of masks during NIV 1
• Don't discontinue chronic maintenance therapies during an acute exacerbation 1
• Avoid manually assisted cough in patients with airflow obstruction as it may be detrimental 2
• Previous episodes of ventilator-associated pneumothorax warrant consideration of admission to HDU/ICU and use of NIV at lower than normal inspiratory pressures 1

By following this approach, acute shortness of breath in cystic fibrosis can be effectively managed to improve outcomes and reduce morbidity and mortality.