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
Non-invasive 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 non-invasive ventilation on our dedicated ResMed page.
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.
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.
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.