Paeds Vivas · respiratory-sleep-and-airway
Neuromuscular respiratory failure and airway clearance — branching viva
Branching viva from a boy with Duchenne muscular dystrophy with morning headaches and recurrent chest infections, through the recognition of the two-part pump-and-cough failure, the monitoring of vital capacity, peak cough flow and nocturnal carbon dioxide, the two parallel treatments of non-invasive ventilation and mechanical insufflation-exsufflation, and the chest-infection rule to clear secretions and ventilate rather than merely oxygenate, to an infant with spinal muscular atrophy whose acute management and anticipatory plan are tested.
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Target exams
Opening — framing the problem
The examiner begins: a 13-year-old boy with Duchenne muscular dystrophy, now a full-time wheelchair user, has morning headaches, daytime sleepiness and three chest infections this winter with a weak cough. How do you frame his respiratory problem? [1] [2]
I would frame it as the two-part respiratory failure of neuromuscular disease. Weak inspiratory muscles cause hypoventilation that appears first in sleep, which explains the morning headaches and daytime sleepiness, and weak expiratory and bulbar muscles cause an ineffective cough with secretion retention, which explains the recurrent chest infections. His lungs are structurally normal, so I would treat the pump and the cough as two separate problems. [1] [6]
You have read the opening of this viva. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.
References6Show ledgerHide ledger
- [1]Hull J; Aniapravan R; Chan E; et al British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax, 2012.PMID 22730428
- [2]Birnkrant DJ; Bushby K; Bann CM; et al Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol, 2018.PMID 29395990
- [3]Finder JD; Birnkrant D; Carl J; et al Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med, 2004.PMID 15302625
- [4]Finkel RS; Mercuri E; Meyer OH; et al Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscul Disord, 2018.PMID 29305137
- [6]Chatwin M; Toussaint M; Gonçalves MR; et al Airway clearance techniques in neuromuscular disorders: A state of the art review. Respir Med, 2018.PMID 29501255
- [7]Fauroux B; Khirani S; Griffon L; et al Non-invasive Ventilation in Children With Neuromuscular Disease. Front Pediatr, 2020.PMID 33330262