British Thoracic Society Guideline for Respiratory Management of Children with Neuromuscular Weakness

Hull J, et al Department of Paediatrics, Oxford University Hospitals NHS Trust, Oxford, UK
Citation: Thorax. 2012 Jul;67 Suppl 1:i1-40,

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 patients. [√]
  • Children with neuromuscular disease with a history of swallowing difficulties should have a feeding assessment by a speech and language therapist including a video fluoroscopy swallow assessment if the swallow is thought to be unsafe. [√]
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