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British Thoracic Society guideline for respiratory management of children with neuromuscular weakness
  1. Jeremy Hull1,
  2. Roona Aniapravan2,
  3. Elaine Chan3,
  4. Michelle Chatwin4,
  5. Julian Forton5,
  6. Jayne Gallagher1,
  7. Neil Gibson6,
  8. Jill Gordon7,
  9. Imelda Hughes3,
  10. Renee McCulloch8,
  11. Robert Ross Russell2,
  12. Anita Simonds4
  1. 1Department of Paediatrics, Oxford University Hospitals NHS Trust, Oxford, UK
  2. 2Department of Paediatrics, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  3. 3Royal Manchester Children's Hospital, Manchester, UK
  4. 4National Institute of Health Research Respiratory Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, UK
  5. 5University Hospital of Wales, Cardiff, UK
  6. 6Royal Hospital for Sick Children, Glasgow, UK
  7. 7Child Development Centre, Suffolk Community Healthcare, UK
  8. 8Great Ormond St Hospital, London, UK
  1. Correspondence to Jeremy Hull, Level 2, Children's Hospital, John Radcliffe Hospital, Oxford, OX3 9DU, UK; jeremy.hull{at}

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Summary of recommendations

Identifying children at risk of respiratory complications

  • Clinical assessment of respiratory health should be part of every medical consultation for children with neuromuscular weakness (NMW) and should be directed towards identifying progressive muscle weakness, ability to cope with respiratory infection, aspiration, progression of scoliosis and sleep-disordered breathing. [D]

  • Ulna length or armspan should be used to predict lung function in children with neuromuscular disease whose height cannot be accurately measured. [B]

  • Vital capacity should be measured in all patients with neuromuscular disease who are capable of performing spirometry as part of the respiratory assessment. [C]

  • Cough peak flow should be used as part of the assessment of effective secretion clearance in children with neuromuscular disease over the age of 12 years.

  • Assessment for sleep-disordered breathing should be carried out no less than annually for children with neuromuscular disease who have a vital capacity of <60% predicted and for children who have become non-ambulant because of progressive muscle weakness or who never attain the ability to walk. [D]

  • Assessment for sleep-disordered breathing should be carried out no less than annually for all infants with weakness, children with NMW who have symptoms of obstructive sleep apnoea or hypoventilation, children with clinically apparent diaphragmatic weakness and children with rigid spine syndromes. [√]

  • In young children whose rate of disease progression is uncertain, or in older children who have shown a clinical deterioration or who are suffering with repeated infections, or who develop symptoms of sleep-disordered breathing, sleep assessment may need to be more frequent than once a year. [√]

  • All children with abnormal overnight oximetry should undergo more detailed sleep monitoring with at least oxycapnography. [√]

  • When there is doubt about the cause of sleep-disordered breathing, overnight polysomnography or sleep polygraphy should be performed. [√]

  • Portable overnight oxycapnography or polygraphy in the home may be the most appropriate option for some …

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  • Competing interests None.

  • Provenance and peer review Not commissioned; internally peer reviewed.