Noninvasive Ventilation in Children with Spinal Muscular Atrophy Types 1 and 2

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Abstract

Objective:

Our aim was to assess the efficacy of noninvasive ventilation (NIV) for the treatment of thoracoabdominal asynchrony during sleep in children with spinal muscular atrophy (SMA) types 1 and 2.

Design:

Nine subjects underwent assessment for sleep apnea/hypopnea index (AHI), mean oxyhemoglobin saturation (SpO2), oxygen desaturation index, transcutaneous carbon dioxide tension (tcpCO2), and mean phase angle during sleep as a measure of thoracoabdominal coordination. A second sleep study was performed with use of NIV.

Results:

The nine patients (7 mos of age, range 2–33) had a baseline AHI of 2.1 events per hour (range 0.5–55.8), oxygen desaturation index of 3.7 events per hour (range 1.6–46.1), mean tcpCO2 of 46 mm Hg (range 37–60), and phase angle of 127 degrees (range 72.7–151.7). Comparing baseline and NIV sleep studies, we found significant improvement in oxygen desaturation index (P < 0.010), mean tcpCO2 (P < 0.001), and phase angle (P < 0.001). For five patients, phase-angle improvement became significant when using high-span bilevel positive airway pressure (PAP).

Conclusions:

NIV improved sleep breathing parameters and thoracoabdominal coordination during sleep in SMA types 1 and 2. Phase-angle improvement correlated with bilevel PAP pressures. Phase angle may be useful for the evaluation and monitoring of therapeutic interventions such as NIV.

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