What are general principles of treatment?
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.
Describe pharmacological interventions for preservation of lung function.
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]
List nonpharmacological interventions.
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)
Describe the prevention of infection and reduction of exacerbations in CF.
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
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