43. Cystic fibrosis

All patients with CF require periodic follow-up with a multidisciplinary team for specialized management.

Management should include the following goal-directed interventions:

• Preservation of lung function

  • Pharmacological and nonpharmacological interventions

  • Prevention of infection and reduction of exacerbations

• Optimization of nutrition

• Screening and monitoring for comorbidities and complications

Patients with certain mutations may benefit from treatment with CFTR modulators.

Acute pulmonary exacerbations require rapid and effective treatment.

High-dose ibuprofen: can slow the progression of lung disease

Bronchodilators: SABA (e.g., albuterol), LABA (e.g., salmeterol)

Mucolytics

• Hypertonic saline nebulization (6–7% NaCl): mucociliary and osmotic effect that can improve mucociliary clearance and thin the mucus

• Dornase alfa, aerosolized : a recombinant DNase that thins the mucus by breaking down extracellular DNA in sputum [30]

Other therapies

• N-acetylcysteine: efficacy is unproven in CF [20][29]

• Corticosteroids (inhaled and systemic): insufficient evidence; not routinely used [30]

Airway clearance techniques: a mainstay of CF treatment that loosens and mobilizes mucus secretions

• Conventional chest physiotherapy (CPT): postural drainage with percussion and/or clapping

• Alternative airway clearance methods

  • High-frequency chest compression

  • Airway oscillating devices

  • Positive expiratory pressure devices

• Huff coughing

Exercise (e.g., swimming, jogging, cycling)

Pulmonary exacerbations in patients with CF are often triggered by chronic lung infections of pathogenic organisms.

Eradication and/or suppression regimens can prevent exacerbations and improve lung function.

Consider treatment after early detection of relevant pathogens during routine surveillance sputum cultures.

Eradication regimens include:

• P. aeruginosa: inhaled tobramycin

• MRSA: inhaled vancomycin PLUS oral antibiotics , PLUS extrapulmonary eradication