Long­term noninvasive ventilation in children and adolescents with neuromuscular disorders

Abstract

The aim of the current study was to investigate the long­term impact of nocturnal noninvasive (positive­pressure) ventilation (NIV) on sleep, sleep­disordered breathing (SDB) and respiratory function in children and adolescents with progressive neuromuscular disorders (NMD).Thirty patients (12.3±4.1 yrs) with various inherited NMD were treated with NIV for ventilatory insufficiency (n=14) or symptomatic SDB (n=16). Patients were prospectively followed with sleep studies, spirometry and peak inspiratory muscle pressure. Ten patients were studied before and after 3 nights withdrawal from NIV.NIV normalised nocturnal gas exchange in all patients and diurnal gas exchange in patients with ventilatory insufficiency. The effects persisted over 25.3±12.7 months. Nocturnal transcutaneous partial pressure of carbon dioxide improved from (baselineversuslatest control) 7.1±1.3 to 5.5±0.6 kPa (53.7±9.9 to 41.6±4.8 mmHg), diurnalcarbon dioxide arterial tension from 6.3±1.6 to 5.4±0.5 kPa (47.5±11.9 to 40.6±3.6 mmHg).NIV improved respiratory disturbance index, arousals from sleep, nocturnal heart rate and sleep architecture. Vital capacity decreased in five adolescents with Duchenne muscular dystrophy −183±111 mL·yr−1but remained stable in 25 children with other conditions (8±78 mL·yr−1). Three nights withdrawal of NIV in 10 previously stable patients resulted in prompt deterioration of SDB and gas exchange back to baseline but could be instantly normalised by resumption of NIV.Noninvasive (positive­pressure) ventilation has favourable long­term impact on nocturnal and diurnal gas exchange and sleep and in patients with non­Duchenne neuromuscular disorders on vital capacity as well. It is indicated in children and adolescents with symptomatic sleep­disordered breathing or ventilatory insufficiency due to neuromuscular disorders.

Non‐invasive ventilation reduces respiratory tract infections in children with neuromuscular disorders

“…Non-invasive ventilation (NIV) is the standard therapy of chronic respiratory failure and nocturnal hypoventilation. [6][7][8][9] But it has also been shown to be effective during acute deterioration of respiratory function during chest infection. 10 Insufficient cough leads to reduced pulmonary secretion clearance and therefore increases the risk of pneumonia.…”

Evaluation of Ventilators for Mouthpiece Ventilation in Neuromuscular Disease

“…1,2 Indeed, NIV normalizes nocturnal gas exchange and initially also daytime gas exchange. 3 However, as respiratory muscle weakness progresses, daytime ventilatory failure may occur, which requires the extension of NIV during the day.…”

A Comparison of Leak Compensation During Pediatric Noninvasive Ventilation: A Lung Model Study

“…2 In adult patients, high-level evidence supports the use of NIV in cardiogenic pulmonary edema, exacerbation of COPD, neuromuscular disorders, and respiratory distress in the immuno-compromised patient. 1 Although the application of NIV for pediatric patients is less established than for adult patients, NIV is considered acceptable in children with severe obstructive sleep apnea syndrome, 3 post-extubation respiratory failure, 4,5 immunocompromised acute respiratory failure, 6 and chronic respi-ratory failures such as neuromuscular disease 7 and cystic fibrosis. 8 While NIV theoretically allows for respiratory system muscle unloading, alveolar recruitment, oxygenation, and CO 2 wash-out, patient-ventilator asynchrony is a major issue leading to NIV treatment failure.…”