Drug Action
Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) protein potentiator that increases chloride transport in the abnormal CFTR protein.
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Drug reference
Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) protein potentiator that increases chloride transport in the abnormal CFTR protein.
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Cystic fibrosis (specialist use only) ▶BY MOUTH ▶Adult: 150 mg every 12 hours
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DUE TO INTERACTIONS ▶Manufacturer advises reduce dose to 150 mg twice a week with concurrent use of potent inhibitors of CYP3A4. ▶Manufacturer advises reduce dose to 150 mg once daily with concurrent use of moderate inhibitors of CYP3A4.
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Organ transplantation (no information available)
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Ivacaftor should be prescribed by a physician experienced in the treatment of cystic fibrosis.
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Scottish Medicines Consortium (SMC) decisions The Scottish Medicines Consortium has advised (December 2016) that ivacaftor (Kalydeco ®) is not recommended for use within NHS Scotland for the treatment of patients with cystic fibrosis aged 18 years and above who have an R117H mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, as insufficient clinical and economic evidence was submitted. All Wales Medicines Strategy Group (AWMSG) decisions The All Wales Medicines Strategy Group has advised (October 2017) that ivacaftor (Kalydeco ®) is recommended for restricted use within NHS Wales for the treatment of adults with cystic fibrosis (CF) who have an R117H mutation in the CF transmembrane conductance regulator (CFTR) gene. This recommendation will apply only when people are treated in line with the relevant Welsh clinical access policy and only in circumstances where the approved Wales Patient Access Scheme (WPAS) is utilised or where the list/contract price is equivalent or lower.
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→Appendix 1: ivacaftor
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▶Common or very common Breast abnormalities. diarrhoea. dizziness. ear discomfort. headache. ototoxicity. rash. tympanic membrane hyperaemia ▶Uncommon Gynaecomastia ▶Frequency not known Hepatic function abnormal SIDE-EFFECTS, FURTHER INFORMATION Manufacturer advises interrupt treatment if transaminase levels more than 5 times the upper limit of normal or transaminase levels more than 3 times the upper limit of normal and blood bilirubin more than twice the upper limit of normal—consult product literature.
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Manufacturer advises use only if potential benefit outweighs risk—limited information available.
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Manufacturer advises avoid—present in milk in animal studies.
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Manufacturer advises caution in moderate to severe impairment (limited information available). Dose adjustments Manufacturer advises reduce dose to 150 mg once daily in moderate impairment; in severe impairment reduce starting dose to 150 mg on alternate days, adjust dosing interval according to clinical response and tolerability.
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Caution in severe impairment.
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Manufacturer advises monitor liver function before treatment, every 3 months during the first year of treatment, then annually thereafter (more frequent monitoring should be considered in patients with a history of transaminase elevations).
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Tablets should be taken with fat-containing food.
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There can be variation in the licensing of different medicines containing the same drug. Tablet CAUTIONARY AND ADVISORY LABELS 25 ▶Kalydeco (Vertex Pharmaceuticals (UK) Ltd) Ivacaftor 150 mg Kalydeco 150mg tablets | 28 tablet P £7,000.00 | 56 tablet P £14,000.00
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Patients or carers should be given advice on how to administer ivacaftor tablets. Driving and skilled tasks Manufacturer advises that patients and their carers should be counselled on the effects on driving and skilled tasks—increased risk of dizziness.
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150 mg every 12 hours
First-line fragment. Read the complete Indications and Dose section above.
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