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CFTR modulator triple

Ivacaftor with tezacaftor and elexacaftor

Brand names: Kaftrio (with Kalydeco)

A triple CFTR modulator combination for cystic fibrosis, pairing the potentiator ivacaftor with the correctors tezacaftor and elexacaftor, used in patients with at least one F508del mutation. It addresses both processing and gating defects of CFTR.

Dosing — being independently re-sourced

ClinCalc Pro is rebuilding its dose data from primary open sources — the manufacturer SmPC (eMC), the WHO Model Formulary and other official references — under clinician review. This drug's structured dose is not yet published here. Confirm all doses against the product SmPC and your local formulary before prescribing.

Clinical monograph

How it works

Elexacaftor and tezacaftor are correctors that improve folding and trafficking of CFTR to the cell surface, while ivacaftor potentiates channel opening, together increasing functional chloride transport.

Prescribing in practice

  • Monitor liver function, as transaminase rises and rare serious liver injury can occur, and review the patient's genotype eligibility before starting.
  • All three components are affected by CYP3A, so strong inducers reduce efficacy and strong inhibitors require dose modification, with grapefruit avoided.
  • Baseline and periodic eye examinations are recommended in children because cataracts have been reported with CFTR modulators.

Monitoring

Monitor liver enzymes before and regularly during treatment, with clinical and respiratory response and ophthalmological review in paediatric patients.

Counselling the patient

  • Take the morning and evening doses with fatty food and avoid grapefruit and Seville oranges.
  • Report jaundice, dark urine, abdominal pain or new visual problems.

Evidence & guidelines

The triple combination is recommended by NICE for eligible F508del cystic fibrosis and delivered through specialist CF centres.

Reference: NICE TA787; SmPC; Drug verified in RxNorm (NLM); confirm dosing against the manufacturer SPC (eMC). Verify against your local formulary and current prescribing references before prescribing. Monograph status: clinician-reviewed (2026-07-04).

Related

Curated clinical cross-links plus same-class fallbacks.

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