Noninvasive ventilation for neuromuscular diseases in adults
A number of chronic neuromuscular disorders such as muscular dystrophy and motor neurone disease lead to progressive respiratory muscle dysfunction, which in turn can lead to respiratory failure and death. Nocturnal and daytime noninvasive ventilation (NIV) is becoming the preferred method of treatment for these disorders1, and should be considered for:
Patients with observed sleep-disordered breathing or night time oxygen desaturation1,2
Patients with progressive respiratory muscle weakness who develop hypoventilation (shallow breathing) and Obstructive Sleep Apnoea (OSA)3,4
Noninvasive ventilation as a treatment for neuromuscular disease has several benefits. It has been shown to1,5,6 :
Improve blood gases
Decrease work of breathing
Improve symptoms of fatigue
Reduce daytime sleepiness
Improve morning headaches
Improve survival in Duchenne muscular dystrophy (DMD) and amyotrophic lateral sclerosis (ALS) patients7,8
Joshua O. Benditt1 and Louis J. Boitano. Pulmonary Issues in Patients with Chronic Neuromuscular Disease. Am J Respir Crit Care Med Vol 187, Iss. 10, pp 1046–1055, May 15, 2013
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Bourke SC, Gibson GJ. Sleep and breathing in neuromuscular disease. Eur Respir J 2002;19:1194–1201.
Birnkrant DJ, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Cwik VA, Finder JD, Iannaccone ST, Jacobson LE, Kohn GL, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest 2007; 132:1977–1986.
Mehta S, Hill NS. Noninvasive ventilation. Am J Respir Crit Care Med 2001;163:540–577.
Eagle M, Baudouin SV, Chandler C, Giddings DR, Bullock R, Bushby K. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 2002;12:926–929.
Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J 2006; 28:549–555
Radunovic A, Annane D, Jewitt K, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2009;4:CD004427. Update in: Cochrane Database Syst Rev 2013;3:CD004427.