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Advanced cystic fibrosis lung disease — SCE Respiratory MCQ

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HardRare respiratory diseaseAdvanced cystic fibrosis lung diseaseSCE Respiratory

A 27-year-old man with cystic fibrosis has FEV1 34% predicted, recurrent intravenous antibiotic courses and resting SpO2 91%. He has pancreatic insufficiency and chronic Pseudomonas infection. He asks about disease-modifying treatment and future planning. What is the most appropriate management?

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Correct answer: B — Specialist CF MDT review for CFTR modulator eligibility and early transplant discussion

Advanced CF lung disease should prompt specialist MDT review for CFTR modulator eligibility, optimisation of infection care and timely transplant discussion. Airway clearance remains important and transplant referral should not wait until end-stage deterioration. ICS monotherapy and chronic oral steroid are not definitive CF disease-modifying strategies.

Reference: Cystic Fibrosis Trust Standards of Care; NICE CFTR modulator technology appraisals