CFTR potentiator
Ivacaftor
Brand names: Kalydeco
Adult dose
Dose: 150mg PO BD with fat-containing food
Route: PO
Frequency: BD
Clinical pearls
- CF with G551D and selected gating mutations
- Ophthalmic exam for paediatric patients
Contraindications
- Severe hepatic impairment without dose adjustment
Side effects
- Headache
- Nasopharyngitis
- Abdominal pain
- Hepatotoxicity
- Cataracts (paediatric)
Interactions
- Strong CYP3A4 modulators
- Drugs raising gastric pH minimally affect
- Warfarin
Monitoring
- LFTs
- Ophthalmic (children)
Reference: BNF; NICE TA398; SmPC; https://bnf.nice.org.uk/drugs/ivacaftor/. Verify against your local formulary and the latest BNF before prescribing.
Related
Curated clinical cross-links plus same-class fallbacks.
Pathways
- Acute Asthma in Adults · BTS/SIGN British Guideline on Asthma 2019; NICE NG80
- Pulmonary Embolism Assessment · NICE NG158; ESC 2019 PE Guidelines
- Acute Exacerbation of COPD (AECOPD) · NICE NG115; GOLD 2024
- Spontaneous Pneumothorax (Adult) · BTS Pleural Disease 2023
- Atypical Pneumonia (Legionella / Mycoplasma / Chlamydophila) · BTS 2023; IDSA
- COPD Exacerbation Management · NICE NG115 / GOLD 2024