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DMD – NIV — RACP Paediatrics MCQ

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HardNeurologyDMD – NIVRACP Paediatrics

A 15-year-old with Duchenne muscular dystrophy has morning headache, fragmented sleep and daytime somnolence. Sitting FVC is 58% predicted, daytime saturation is 97% and chest examination is normal. What is the best next respiratory step?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: E — Refer now for specialist sleep and ventilatory assessment

Explanation lettering: E = shown as A · C = shown as B · D = shown as C · A = shown as D · B = shown as E

B is correct because symptoms of nocturnal hypoventilation trigger specialist respiratory and sleep assessment regardless of an FVC still above 50%; FVC alone can miss evolving nocturnal failure. A uses an unsafe late threshold. C may worsen unrecognised hypercapnia and does not correct hypoventilation. D does not treat respiratory-muscle weakness. E bypasses non-invasive ventilation, cough augmentation and shared specialist planning. The specialist neuromuscular respiratory recommendation is jurisdiction-neutral and consistent with Australian multidisciplinary DMD care.

Reference: Healthdirect Australia, Duchenne muscular dystrophy: https://www.healthdirect.gov.au/duchenne-muscular-dystrophy; Duchenne muscular dystrophy respiratory care guideline: https://pmc.ncbi.nlm.nih.gov/articles/PMC11041593/