Inspiratory Muscle Training (IMT) in Adult People With Pompe Disease

Launched by FONDAZIONE DON CARLO GNOCCHI ONLUS · Jul 11, 2023

Keywords

ClinConnect Summary

This clinical trial is studying the effects of a special exercise program called Inspiratory Muscle Training (IMT) for adults with Late-onset Pompe Disease, a condition that affects muscle strength and breathing. The main goal is to see if this training can improve various breathing measures and overall quality of life. Participants will be randomly assigned to use either a Powerbreathe device or a technique called Air-Stacking to strengthen their breathing muscles. Researchers will track changes in how well participants can breathe, walk, and their feelings about shortness of breath and fatigue over the course of a year.

To join the study, participants must be diagnosed with Late-onset Pompe Disease, have been receiving enzyme replacement therapy for at least 12 months, and have specific breathing difficulties. Those with severe heart or lung problems or who depend completely on a ventilator are not eligible. Throughout the trial, participants will engage in aerobic exercises and breathing training, and their progress will be checked at several points over the year. This research aims to find out which method of training works best to help improve breathing and quality of life for those affected by this condition.

Gender

ALL

Eligibility criteria

• Inclusion Criteria:
• • Late On-set Pompe Disease diagnosis already in Enzyme Replacement Therapy (ERT) since 12 months
• • Forced Vital Capacity \<80% predicted or Forced Vital Capacity \>80% but with Postural Drop \>25-30%
• Exclusion Criteria:
• • Severe Cardiologic or Respiratory comorbidities
• • Complete dependence on ventilation