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CFTR modulator eligibility — SCE Respiratory MCQ

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ModerateCystic fibrosisCFTR modulator eligibilitySCE Respiratory

A 19-year-old man with cystic fibrosis has declining lung function despite adherence with airway clearance. Genotyping shows one F508del allele and one minimal-function CFTR variant. FEV1 is 58% predicted and he has recurrent Pseudomonas infection. Liver function tests are normal. What is the most appropriate management?

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Correct answer: D — Assess eligibility for elexacaftor/tezacaftor/ivacaftor therapy

Triple CFTR modulator therapy is appropriate to assess in many patients with at least one F508del mutation, subject to commissioning and contraindications. Ivacaftor monotherapy is mainly for specific gating mutations rather than any genotype. CF care combines mutation-directed treatment with airway clearance, infection management and nutrition.

Reference: NICE cystic fibrosis technology appraisal; CF Trust Standards of Care