How should I manage a patient with cystic fibrosis who is experiencing a pulmonary exacerbation in primary care?

Management of a patient with cystic fibrosis experiencing a pulmonary exacerbation in primary care:

• Assess the severity of the exacerbation clinically, including symptoms, signs, and oxygen saturation levels.
• Obtain respiratory secretion samples for microbiological investigations if possible, preferably sputum samples, to guide antibiotic therapy.
• Start empirical antibiotic treatment promptly; choice depends on the patient's known colonizing organisms and previous antibiotic history.
• For patients with chronic Pseudomonas aeruginosa infection who become clinically unwell, consider oral antibiotics or combination intravenous antibiotics of different classes, with specialist advice if needed.
• Ensure the patient continues or initiates airway clearance techniques to aid mucus clearance, and offer training if not already taught.
• Monitor treatment response by assessing symptom resolution and lung function if feasible.
• Consider chest X-ray if the exacerbation does not respond to treatment or if new radiological abnormalities were previously noted.
• Refer urgently to specialist cystic fibrosis care if the patient is severely unwell, has significant comorbidities, or does not improve with initial treatment.
• Provide advice on infection control measures to prevent cross-infection with other cystic fibrosis patients.

In summary, initial management in primary care involves prompt assessment, empirical antibiotics guided by microbiology, airway clearance support, monitoring, and timely referral to specialist care as needed ¹.