| Paediatric | A 7-year-old girl with no exacerbations for last 2 years No symptom improvement after 4 weeks of oral amoxicillin/clavulanic acid and chest physiotherapy FEV1% predicted is reduced from 105 to 90 Sputum culture ‘mixed oral flora’ with no previous results Haemophilus influenzae cultured from previous throat swab IgE levels undetectable
| A 12-year-old boy with wet cough for last 5 weeks Known colonisation with Pseudomonas aeruginosa No symptom improvement after nebulised tobramycin 300 mb two times per day x4 weeks FEV1% predicted is reduced from 85 to 74 IgE levels undetectable Normal recent oral glucose test Responded well to intravenous piperacillin/tazobactam and intravenous tobramycin 6 months previously
| A 15-year-old boy with increased wet cough, dyspnoea, lethargy and low appetite Known colonisation with Staphylococcus aureus Sputum culture: moderate growth S. aureus sensitive to flucloxacillin FEV1% predicted is reduced from 65 to 50 IgE levels are 100kU/L (stable) Normal recent oral glucose test Responded well to intravenous cefepime and intravenous tobramycin 3 months previously
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| Adult | A 22-year-old woman not attended clinic for 6 months. Shortness of breath, increased chest tightness, lethargy and weight loss (2.0 kg) Regular meds: Hypertonic saline No regular airway clearance/exercise New crackles audible over right middle lobe and lingula regions. FEV1% predicted is reduced 85 to 60. Sputum culture ‘mixed oral flora’ (same as 6 months ago).
| A 34-year-old man with first episode of haemoptysis (20 mL fresh blood on two occasions/past 2 days) CF-related diabetes Chronically infected with P. aeruginosa (multiresistant) History of recurrent allergic bronchopulmonary aspergillosis (treated 2 years ago) IgE level was 300 kU/L (8 months ago) Regular meds: azithromycin 250 mg daily, 6% Hypertonic saline daily, cyclical inhaled dry powder tobramycin, nebulised colistin (month on/off; stopped 2 days ago due to haemoptysis) FEV1% predicted is 50 Sputum culture: P. aeruginosa and Stenotrophomonas maltophilia (2 days ago)
| A 25-year-old woman with increased productive cough for the past month. FEV1% predicted is reduced 63 to 50 (2 weeks ago and unchanged) Chronic P. aeruginosa infection. Sputum culture: P. aeruginosa and flucloxacillin susceptible S. aureus (2 weeks ago). Regular meds: azithromycin 250 mg daily, nebulised 6% Hypertonic saline daily. No symptom improvement after 2 weeks of oral ciprofloxacin and inhaled tobramycin.
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