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Cystic fibrosis with eligible CFTR genotype — SCE Respiratory MCQ

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HardCystic FibrosisCystic fibrosis with eligible CFTR genotypeSCE Respiratory

A 26-year-old woman with cystic fibrosis has genotype F508del/G551D and recurrent exacerbations. FEV1 is 58% predicted despite optimised airway clearance, pancreatic enzyme replacement and inhaled antibiotics. What is the most appropriate management?

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Correct answer: B — Assess eligibility for CFTR modulator therapy

A patient with CF and an eligible gating mutation should be assessed for CFTR modulator therapy as part of specialist CF care. Long-term prednisolone is not a disease-modifying substitute and has substantial toxicity. Pancreatic enzyme withdrawal and pleurodesis do not address the underlying CFTR defect.

Reference: NICE NG78 Cystic fibrosis; NICE CFTR modulator technology appraisals