Table 1. Maintenance pulmonary treatment and reduction in pulmonary exacerbations.
| Intervention (approximate reduction) | Subjects n | Age years | Duration of intervention | Reduction in exacerbations |
| Inhaled Tobramycin solution 300 mg twice daily, 4 weeks on, 4 weeks off (quarter) | 520 | 21 | 24 weeks | 26% (2–43%) less likely to be hospitalised and 36% (17–51%) less likely to require i.v. anti-pseudomonal antibiotics [6] |
| 128 | 13–17 | 2 years | Number of hospital admissions reduced by 19% and i.v. antibiotic courses per patient year reduced by 32% [112] | |
| DNAse 2.5 mg twice or once daily (third) | 968 | ≥5 | 24 weeks | Risk of a PEx requiring parenteral antibiotic therapy reduced 28% (2–48%) for once daily and 37% (13–54%) for twice daily [8] |
| 474 | 6–10 | 96 weeks | Risk of PEx reduced by 34% (0–56%) [113] | |
| Ibuprofen 20–30 mg·kg−1 to a maximum of 1600 mg in two divided doses (third) | 85 | 5–39 | 4 years | Fewer hospital admissions Peto OR 0.64 (95% CI 0.39–1.05) [114] |
| Mannitol 400 mg inhaled dry powder mannitol twice daily (third) | 389 | ≥6 | 26 weeks | 35.4% (risk ratio 0.65 (95% CI 0.34–1.21)) reduction in the incidence of having a PEx [31] |
| Azithromycin 250 mg patients <40 kg and 500 mg patients >40 kg three times a week (half) | 609 | ≥6 | 6 months | Twice as likely to be free of PEx at 6 months (OR 1.96 (95% CI 1.15–3.33) [115] Subjects uninfected with Pseudomonas had a 50% (31–79%) reduction in pulmonary exacerbations treated with new oral antibiotics but not in pulmonary exacerbations requiring the initiation of i.v. or new inhaled antibiotics [116] |
| Nebulised hypertonic saline 7% 4 mL twice daily (half) | 164 | ≥6 | 48 weeks | 56% relative reduction (the mean number of pulmonary exacerbations per participant in the control group was 0.89 compared with 0.39 in the hypertonic-saline group) (difference 0.5, 95 % CI 0.14–0.86) [9] |
| 321 | 4–60 months | 48 weeks | No difference: mean PEx rate (events per person-year) was 2.3 (95% CI 2.0–2.5) in the active treatment group and 2.3 (95% CI 2.1–2.6) in the control group [117] | |
| Ivacaftor 150 mg every 12 h (half) | 161 | ≥12 (with at least one G551D CFTR mutation) | 48 weeks | 55% less likely to have a pulmonary exacerbation [118] |
| Growth hormone | Insufficient evidence [119] | |||
| Antibiotic adjuvant therapy | Insufficient evidence [120] |
Data are presented as n or % (95% CI), unless otherwise stated. PEx: pulmonary exacerbation; CFTR: cystic fibrosis transmembrane conductance regulator.