Pompe Disease - EMST150

Pompe Disease

Persons with pediatric onset and late onset Pompe disease have shown improvement with expiratory muscle strength training for increasing breath pressure.

In one trial after another, the EMST150™ training system brought about significant improvements in individuals with Parkinson’s, multiple sclerosis, spinal cord injuries and other forms of neuromuscular disease. Patients saw improved cough strength as a result of increased exhalatory muscle strength, allowing them to remain independent longer. In addition, EMST150 researchers recognized an opportunity for patients with spinal cord injuries or degenerative diseases that weaken respiratory muscles to use the device to strengthen weakened muscles, improving breathing, coughing, swallowing and speech. And, since the EMST150 is the only device of its kind that is calibrated, therapists are able to track and document progress during therapy programs.

We are motivated by the last 10 years of data and the new information being examined. Dr. Sapienza’s review of respiratory muscle strength training and the number of systematic and meta analyses of respiratory muscle strength training outcomes is strongly suggesting the capability of muscle strength training programs. Most importantly, the EMST150 is the only device to be actively used in randomized clinical trials as the most widely used device in the clinical market. The EMST150™, its protocol and performance are unmatched.

Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining; Jones, Harrison N. et al.Molecular Genetics and Metabolism , Volume 117, Issue 2 , 120 - 128