Paeds Cases · respiratory-sleep-and-airway
Non-invasive ventilation and home respiratory support — structured clinical encounter
Structured encounter testing the approach to a boy with Duchenne muscular dystrophy and nocturnal hypoventilation: recognising the problem, using a sleep study with carbon dioxide monitoring, starting nocturnal bilevel ventilation, assessing and supporting a weak cough, and planning safe home ventilation.
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Station brief (candidate)
You are the paediatric registrar in a respiratory and long-term ventilation clinic. A 13-year-old boy with Duchenne muscular dystrophy, a full-time wheelchair user, is referred with morning headaches, daytime sleepiness and a weakening cough; his last chest infection took two weeks to clear, and his daytime blood gas is normal. The team asks you to establish what is happening, order and interpret the investigations, choose the ventilatory treatment, address the cough, and plan for safe home ventilation. You have 12 minutes with the team and 5 minutes for examiner discussion. [6]
References6ShowHide
- [1]Sterni LM; Collaco JM; Baker CD; Carroll JL; Sharma GD; Brozek JL; et al An Official American Thoracic Society Clinical Practice Guideline: Pediatric Chronic Home Invasive Ventilation. Am J Respir Crit Care Med, 2016.PMID 27082538
- [2]Fauroux B; Abel F; Amaddeo A; Bignamini E; Chan E; Corel L; et al ERS statement on paediatric long-term noninvasive respiratory support. Eur Respir J, 2022.PMID 34916265
- [3]Hull J; Aniapravan R; Chan E; Chatwin M; Forton J; Gallagher J; et al British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax, 2012.PMID 22730428
- [5]Ward S; Chatwin M; Heather S; Simonds AK Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax, 2005.PMID 16299118
- [6]Mellies U; Ragette R; Dohna Schwake C; Boehm H; Voit T; Teschler H Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J, 2003.PMID 14582916
- [7]Chatwin M; Ross E; Hart N; Nickol AH; Polkey MI; Simonds AK Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J, 2003.PMID 12662009