Table 1

Key features of hypothetical patient scenarios

Scenario 1Scenario 2Scenario 3
  • 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

  • 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.

  • CF, cystic fibrosis; FEV1, forced expiratory volume in 1 s; IgE, Immunoglobulin E.