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Perspectives

Paediatric cystic fibrosis. Part 2: Pulmonary management

Hiep Pham, John Massie

Figures

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Abstract

Lung disease is a common problem in people with cystic fibrosis. Daily treatment to control airway infection, delay development of bronchiectasis and limit pulmonary exacerbations primarily involves airway clearance, mucolytic therapy and antibiotics, but new medications are also helping a growing number of patients.

Key Points

  • Cystic fibrosis (CF) lung disease is a result of airway infection, inflammation and mucociliary obstruction, with structural airway changes occurring from an early age.
  • Daily treatment with airway clearance and mucolytic therapy aims to delay the progression of bronchiectasis.
  • Pulmonary exacerbations are often managed in an outpatient setting with antibiotics and additional airway clearance.
  • Treatment with CF transmembrane conductance regulator modulators lowers sweat chloride levels, improves lung function and reduces pulmonary exacerbations for CF patients with responsive mutations.
  • CF teams encourage the involvement of GPs in the care of patients with CF and are readily available to guide management decisions.

Figures

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