Dynamic Respiratory Muscle Function in Late-Onset Pompe Disease

Imaging of the diaphragm will be completed during resting breathing, and then during a maximal voluntary ventilation maneuver.

Upright forced vital capacity (FVC)

A spring-loaded pressure device will resist inspiration. The patient must generate the threshold inspiratory pressure to receive airflow. The changes in breathing timing, flow and volume will be recorded.

Measure the maximal airflow on inhalation

The breathing pattern will be assessed when the subject is seated and in a relaxed state.

Evaluates the time limit that a participant can maintain ventilation while breathing with a submaximal inspiratory threshold load.