What are the current guidelines for the use of CFTR modulators in the management of cystic fibrosis in adults?

Guideline-aligned answer with reasoning, red flags and references. Clinically reviewed by Dr Kola Tytler MBBS CertHE MBA MRCGP.

Posted: 16 August 2025Updated: 16 August 2025 Guideline-Aligned (High Confidence) Clinically Reviewed
Dr Kola Tytler MBBS CertHE MBA MRCGPClinical Lead • iatroX

Current guidelines recommend the use of CFTR modulators as a key treatment option in the management of cystic fibrosis in adults.

Specifically, ivacaftor–tezacaftor–elexacaftor (IVA–TEZ–ELX) plus ivacaftor (IVA) alone is recommended for treating cystic fibrosis in people aged 2 years and over, including adults.

Tezacaftor–ivacaftor (TEZ–IVA) plus ivacaftor alone is recommended for people aged 6 years and over.

Lumacaftor–ivacaftor (LUM–IVA) is recommended for people aged 1 year and over.

These recommendations are based on NICE technology appraisal guidance and represent the current approved CFTR modulator therapies for cystic fibrosis management.

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