[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Pharmacology](https://durgical.com/en/pharmacology/) › [CFTR modulators](https://durgical.com/en/pharmacology/classes/cftr-modulators/) › Elexacaftor + tezacaftor + ivacaftor

# Elexacaftor + tezacaftor + ivacaftor

## CFTR modulators?

CFTR modulators

A triple CFTR modulator: two correctors bring the faulty F508del channel to the cell surface and ivacaftor opens it.

Updated Nov 1, 2026

## Quick facts

**Group**
[CFTR modulators](https://durgical.com/en/pharmacology/classes/cftr-modulators/)

**Target**
CFTR chloride channel (cystic fibrosis transmembrane conductance regulator)

**Used for**
Cystic fibrosis in patients

**Key effects**
Headache · Upper respiratory infections · Diarrhoea

**Monitor**
Liver tests · Eye examinations before · Mood

**ATC**
R07AX32

No doses: the clinician sets them by the current guideline.

On this page

- The basics Mechanism of action
- Indications
- Adverse effects
- Pharmacokinetics
- Memory aid

- Safety Contraindications
- Key interactions
- What is monitored
- Administration and safety
- Pregnancy and breastfeeding
- Nursing care

- Study Test yourself

- Connections Connections

## Mechanism of action

⇅**CFTR chloride channel (cystic fibrosis transmembrane conductance regulator)**Elexacaftor + tezacaftor + ivacaftor

ⓘ The figure shows human F508del CFTR (cryo-EM) with all three drugs bound; the highlighted ligand is elexacaftor, and tezacaftor and ivacaftor sit at their own sites.

**Target in 3D:** CFTR chloride channel (cystic fibrosis transmembrane conductance regulator). PDB structure 8EIQ (RCSB PDB, public domain). [View the structure at RCSB PDB](https://www.rcsb.org/structure/8EIQ) · Drag to rotate · pinch to zoom

The most common cystic fibrosis variant, F508del, makes a misfolded CFTR protein that is destroyed before it reaches the cell membrane. Elexacaftor and tezacaftor are correctors: they bind CFTR at different sites during folding and increase the amount of channel that reaches the surface. Ivacaftor is a potentiator: it raises the probability that the channel at the surface is open. Together they restore part of chloride and water transport, so mucus in the airways and other organs becomes thinner.

- F508del CFTR misfolds and is destroyed before reaching the membrane
- Elexacaftor and tezacaftor help it fold and reach the cell surface
- Ivacaftor holds the channel open more of the time
- Chloride and water flow again and mucus becomes thinner

## Indications

- Cystic fibrosis in patients with at least one F508del variant or another responsive CFTR variant [Cystic fibrosis →](https://durgical.com/en/conditions/cystic-fibrosis/)

## Adverse effects

### Common

- Headache
- Upper respiratory infections, runny or stuffy nose
- Diarrhoea, gas and abdominal pain
- Rash
- Conjunctivitis (pink eye)

### Serious (rare, important)

- Drug-induced liver injury, sometimes life-threatening
- Cataracts in children and adolescents
- Depression, anxiety, sleep problems and suicidal thoughts
- Severe allergic reactions

## Contraindications

- Hypersensitivity to any of its components
- Not recommended with strong CYP3A inducers
- Caution in liver disease

## Key interactions

**Strong CYP3A inducers (St John's wort, rifampicin)**
Much lower drug levels and loss of effect

**CYP3A inhibitors (ketoconazole) and grapefruit**
Higher drug levels; the regimen is adjusted

## What is monitored

- Liver tests (transaminases, bilirubin) before and during treatment
- Eye examinations before and during treatment in children
- Mood, sleep and signs of depression
- Lung function and symptoms

## Administration and safety

- Oral tablets or granules; taken with food that contains fat
- Grapefruit and grapefruit juice are avoided
- Prescribed only after the patient's CFTR genotype is known

## Pregnancy and breastfeeding

Human data are limited; the decision weighs the benefit of controlling cystic fibrosis against unknown risks, with the specialist team.

## Nursing care

- Check liver test results and teach the signs of liver injury: jaundice, dark urine, right upper abdominal pain
- Ask about mood changes and sleep at each visit
- Review the medication list for rifampicin, other enzyme inducers and herbal products such as St John's wort
- Teach that it is taken with a fat-containing meal or snack

## Pharmacokinetics

**Absorption**
Oral; taken with fat-containing food

**Metabolism**
Liver, by CYP3A enzymes (grapefruit raises and St John's wort lowers its levels)

## Memory aid

Two Correctors Carry the protein, one Potentiator Pops the gate open: E + T + I = Escort, Transport, open It.

## Test yourself

Think of the answer, then tap the question to see it.

## Which components of the triple combination are correctors and which is a potentiator?

Elexacaftor and tezacaftor are correctors; ivacaftor is the potentiator.

## Which CFTR variant is the main target of the triple combination?

F508del, the most common cystic fibrosis variant (one copy is enough).

## Which serious adverse effect requires liver tests during treatment?

Drug-induced liver injury, which can be life-threatening.

## Why does rifampicin make this treatment fail?

It strongly induces CYP3A and lowers the drug levels.

[Add to my cards (4)](https://durgical.app/estudiar/?add=/en/pharmacology/elexacaftor-tezacaftor-ivacaftor/)

[More Pharmacology questions →](https://durgical.com/en/practice/?f=pharmacology)

## Connections

## Related drugs 4

- [Ivacaftor](https://durgical.com/en/pharmacology/ivacaftor/)
- [Dornase alfa](https://durgical.com/en/pharmacology/dornase-alfa/)
- [Pancrelipase](https://durgical.com/en/pharmacology/pancrelipase/)
- [Rifampicin](https://durgical.com/en/pharmacology/rifampicin/)

## Related conditions 1

- [Cystic fibrosis](https://durgical.com/en/conditions/cystic-fibrosis/)

[← Back to the class: CFTR modulators](https://durgical.com/en/pharmacology/classes/cftr-modulators/)

Educational content on how drugs work; not a prescribing guide and no substitute for a health professional's advice. No doses are given.

Sources: [Elexacaftor, Tezacaftor, and Ivacaftor: MedlinePlus Drug Information](https://medlineplus.gov/druginfo/meds/a619061.html) (MedlinePlus (U.S. National Library of Medicine), 2026); [CFTR gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/cftr/) (MedlinePlus (U.S. National Library of Medicine), 2026); [eEML - Electronic Essential Medicines List](https://list.essentialmeds.org/?query=elexacaftor) (World Health Organization, 2025).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/pharmacology/elexacaftor-tezacaftor-ivacaftor/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/pharmacology/elexacaftor-tezacaftor-ivacaftor/