Table 1 Precipitating factors for pulmonary exacerbations in cystic fibrosis, their diagnosis and management
Precipitating factorDiagnosisManagement
Non-adherence to maintenance treatmentCareful sensitive history taking through the multidisciplinary teamRe-negotiating to treatment priorities, (rarely) child protection procedures
Viral infection
    Respiratory syncytial virusHistory—season. Immunofluorescence of NPASupportive. Adequate fluids and oxygen in infants. Intravenous antibiotics to treat secondary bacterial infection
    InfluenzaHistory—prevalence in sentinal sites. Immunofluorescence of NPA or throat swabAntibiotic and supportive treatment plus specific neuraminidase inhibitors (Zanamavir or Oseltamivir)
Fungal infection/hypersensitivity
    ABPARaised total IgE and aspergillus-specific IgE. New findings on chest radiographOral prednisolone 0.5–1 mg/kg for 2–3 weeks and then taper. Itraconazole
Mucus plugging
Lobar/segmental collapseRadiologicalConsider ABPA. Intravenous antibiotics. Oral prednisolone. Targeted physiotherapy. If no response proceed to flexible bronchoscopy with bronchial lavage under direct division ± direct instillation of dornase alfa. Physiotherapist administered (“blind”) bronchial lavage
Atypical mycobacteriaATS clinical and radiological criteriaGuided by specific organism. At least 3 drugs started sequentially. Treatment required for at least 12 months
Mycobacterium avium complexSputum cultureClarithromycin, rifabutin, ethambutol
M abscessus/fortuitum Cefoxtin, amikacin, clarithromycin
M kansasii Isoniazid, rifampicin, ethambutol
  • ABPA, allergic bronchopulmonary aspergillosis; ATS, American Thoracic Society; NPA, nasopharyngeal aspirate.