Respiratory muscle training could be beneficial for patients with Pompe disease (PD) and other neuromuscular disorders, according to an article recently published in the European Respiratory Review.

“Respiratory muscle training improves lung volumes and respiratory muscle strength in neuromuscular disease, but confidence is tempered by limitations in the underlying research,” the authors wrote.

This systematic review and meta-analysis included 40 published randomized controlled trials from 1986 to 2021 in 7 databases. Among the reviewed publications, 951 patients were included. The researchers analyzed the benefits of respiratory training in children and adults previously diagnosed with 9 different neuromuscular disorders.


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Only 1 patient had PD, specifically, the late-onset subtype. The most common diagnosis was spinal cord injury, followed by multiple sclerosis, Duchenne muscular dystrophy, Parkinson disease, amyotrophic lateral sclerosis, general neuromuscular disease, Huntington disease, myasthenia gravis, and Down syndrome.

The degree of lung involvement varied greatly according to vital capacity or forced vital capacity (FVC), ranging from less than 2 L or 50% of the predicted value to above 3 L or 80% of the predicted value.

Respiratory muscle training achieved overall improvements in FVC with a standardized mean difference (SMD) of 0.4, maximal inspiratory pressure with an SMD of 0.53, and maximal expiratory pressure with an SMD of 0.7. Conversely, the control group that received usual care or alternative treatments did not showcase such improvements.

The researchers did not observe symptomatic relief or improvement in quality of life, although these findings could be due to the limitations of this study.

“A paucity of data renders it impossible to determine whether the demonstrated improvements in respiratory function translate into clinically important changes in dyspnea, voice, [quality of life] or physical capacity and there are insufficient data to formulate recommendations regarding optimum training dosage or frequency,” the authors explained.

Reference

Watson K, Egerton T, Sheers N, et al. Respiratory muscle training in neuromuscular disease: a systematic review and meta-analysis. Eur Respir Rev. Published online November 29, 2022. doi:10.1183/16000617.0065-2022