Table 2 Formulary of drugs used in the prevention and management of pulmonary exacerbations in cystic fibrosis
DrugRouteAge/weightDoseFrequency (times daily)Maximum doseDuration
Amphotericin (doses are for liposomal formulation which is better tolerated)IV (infusion rate varies with preparation)Test dose100 μg/kg1 dose100 μg/kg1 dose
Start1 mg/kg/day15 mg/kg/day2 weeks
Increase by1 mg/kg/day1
Maximum dose5 mg/kg/day1
CeftazidimeIV (30 min infusion)1 month–18 years50 mg/kg33 g ×3 daily2 weeks
CiprofloxacinOral1 month–5 years15 mg/kg2750 mg ×2 daily2 weeks
5–18 years20 mg/kg2
ColistinIV<60 kg25000 units/kg32 million units ×3 daily2 weeks
(30 min infusion)>60 kg1–2 million units3
Co-trimoxazoleOral6 weeks–6 months120 mg2960 mg ×2 daily2 weeks
6 months–6 years240 mg2
6–12 years480 mg2
>12 years960 mg2
Dornase alfaNebulised>5 years2.5 mg1As indicated
DoxycyclineOral>12 years only200 mg loading dose, then 100 mg1 dose200 mg2 weeks
Give 200 mg in severe cases1
FlucloxacillinIV (30 min infusion)25 mg/kg41 g ×4 daily2 weeks
ItraconazoleOral<12 years3–5 mg/kg1200 mg ×2 daily has been usedMaintenance
>12 years200 mg1
LinezolidIV/oral (IV rarely needed)1 month–12 years10 mg/kg3600 mg ×2 daily10–14 days
>12 years600 mg2
MeropenemIV (bolus over 5 min or 15–30 min infusion)1 month–18 years40 mg/kg32 g ×3 daily2 weeks
Prednisolone (treatment ABPA)Oral0.5–1 mg/kg12–3 weeks
2–3 months
0.5–1 mg/kgAlternate days2–3 months
PrednisoloneOral1–2 mg/kg140 mg2 weeks and review
Tobramycin (trough level day 2)IV (30 min infusion)10 mg/kg1Max starting dose 660 mg2 weeks
TeicoplaninIV (bolus or 30 min infusion)Loading dose10 mg/kg2400 mg×3 doses
Continue on10 mg/kg12 weeks
TemocillinIV (bolus over 3–4 min or 30–40 min infusion)>12 years and >45 kg1–2 g22 g2 weeks
  • Many drugs, though commonly used in children, do not have a marketing authorisation for use in this age group. Some drugs listed in the table will not have a marketing authorisation for this indication or for the dose given here, although there will be evidence to support their use. This formulary is provided to aid safe and effective prescribing for patients with cystic fibrosis, but the user is advised to check all doses against a standard reference such as the British National Formulary (BNF) (,26 the BNF for children ( or the UK CF Trust Antibiotic Guidelines.24