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Advanced cystic fibrosis — SCE Respiratory MCQ

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HardRare/MiscellaneousAdvanced cystic fibrosisSCE Respiratory

A 29-year-old man with cystic fibrosis has FEV1 persistently 28% predicted despite maximal therapy, recurrent IV antibiotic admissions and new pulmonary hypertension. BMI is falling despite nutritional support. What is the most appropriate management?

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Correct answer: D — Early referral for lung transplant assessment

Advanced CF lung disease with FEV1 below 30%, frequent exacerbations, weight loss and pulmonary hypertension should prompt early transplant referral. Waiting until invasive ventilation increases peri-transplant risk and may miss the window for assessment. Prednisolone and stopping airway clearance are not definitive strategies.

Reference: ISHLT Lung Transplant Candidate Selection Consensus; CF Trust Standards of Care