Noninvasive ventilation for paediatric patients

Children with inherited neuromuscular conditions such as spinal muscular atrophy (SMA), Duchenne’s muscular dystrophy (DMD), Ullrich congenital muscular dystrophy and X-linked myotubular myopathies are at high risk of respiratory disorders during early childhood or adolescence.1

The British Thoracic Society guidelines for respiratory management of children with neuromuscular weakness recommends annual sleep studies for patients who have symptoms of sleep apnoea or nocturnal hypoventilation (shallow breathing).2 Children with rigid spine syndrome and diaphragmatic weakness should be closely monitored for symptoms and may need more frequent sleep studies.1,3

Noninvasive ventilation is recommended in patients with daytime hypercapnia (elevated levels of carbon dioxide) and symptomatic nocturnal hypoventilation. NIV use has been shown to:

  • Improve pulmonary gas exchange and manage chronic hypoventilation4,5
  • Improve health related quality of life4,5
  • Improve sleep related symptoms5
  • Reduce hospital readmissions for acute ventilatory decompensation1,5
  • Increase survival in neuroMND, type I SMA, ALS and DMD patients6,7,8,9

To ensure optimal outcomes, doctors should closely monitor the efficacy of NIV and patient adherence to therapy.1

Find out more about noninvasive ventilation on our dedicated ResMed page

References

  • 01

    Simonds AK, Chronic hypoventilation and its management. Eur Respir Rev 2013; 22: 325–3322.

  • 02

    Hull J, Aniapravan R, Chan E, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax 2012; 67: Suppl. 1, i1–i40.

  • 03

    Ramirez A, Khirani S, Delord V, et al. Assessment of sleep quality by pulse wave amplitude and actigraphy in children with sleep disordered breathing: evaluation at diagnosis and under non-invasive ventilation. Sleep Breath 2013; 17: 827–835.

  • 04

    Mellies U, Ragette R, Dohna Schwake C, et al. Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J 2003; 22: 631–636.

  • 05

    Young HK et al. Outcome of noninvasive ventilation in children with neuromuscular disease. Neurology. 2007 Jan 16;68(3):198-201.

  • 06

    Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 2006; 5: 140–147.

  • 07

    Eagle M, Baudouin SV, Chandler C, et al. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 2002; 12: 926–929.

  • 08

    Simonds AK, Muntoni F, Heather S, et al. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax 1998; 53: 949–952.

  • 09

    Oskoui M, Levy G, Garland CJ, et al. The changing natural history of spinal muscular atrophy type 1. Neurology 2007; 69: 1931–1936.