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Cystic fibrosis advanced lung disease — SCE Respiratory MCQ

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HardRare/MiscellaneousCystic fibrosis advanced lung diseaseSCE Respiratory

A 31-year-old woman with cystic fibrosis has FEV1 persistently 28% predicted despite maximal therapy, recurrent intravenous antibiotic courses and increasing resting hypoxaemia. She asks about future options. What is the most appropriate management?

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Correct answer: E — Refer early for lung transplant assessment

Advanced CF lung disease with very low FEV1, recurrent exacerbations and hypoxaemia warrants early transplant referral before a crisis. Waiting until invasive ventilation may remove the opportunity for planned assessment. Pleurodesis and salbutamol alone do not address end-stage suppurative lung disease.

Reference: Cystic Fibrosis Trust Standards; ISHLT Lung Transplant Candidate Guideline