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What are the current guidelines for the use of CFTR modulators in the management of cystic fibrosis in adults?
Answer
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 (TA988, 2024) and represent the current approved CFTR modulator therapies for cystic fibrosis management.
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