NCT02354651

Response to Diaphragmatic Pacing in Subjects With Pompe Disease

• Status: Completed
• Phase: —
• Study type: Observational
• Enrollment: 9 (actual)
• Sponsor: University of Florida · Academic / Other
• Sex: All
• Age: 2 Years – 65 Years
• Healthy volunteers: Not accepted

Summary

The purpose of this study is to determine the effect of diaphragm pacing on respiratory function in subjects with Pompe disease by evaluating the duration and pattern of spontaneous respiratory function versus paced ventilation respiratory function in patients with Pompe disease who have received the NeuDx Diaphragm pacer (DPS).

Detailed description

Subjects with Pompe disease who are scheduled to receive a NeuRx Diaphragm pacer (DPS) will be invited to participate in this study. The following tests will be performed during participation in the study: 1. Forced expiratory tests 2. Maximal Inspiratory Pressure (MIP) 3. Resting Breathing Pattern 4. Phrenic Nerve Function Evaluation with EMG 5. Severe Respiratory Insufficiency Questionnaire 6. Magnetic stimulation 7. Diaphragm conditioning consisting of: 1. The external stimulator settings 2. The duration of diaphragm stimulation 3. Off-ventilator spontaneous breathing (SB)

Conditions

• Pompe Disease

Interventions

Type	Name	Description
OTHER	Diaphragm conditioning via phrenic nerve stimulation	Diaphragm pacing will be prescribed by the patient's clinical team.

Timeline

• Start date: 2013-05-01
• Primary completion: 2022-06-01
• Completion: 2023-01-01
• First posted: 2015-02-03
• Last updated: 2023-08-01
• Results posted: 2023-06-05

Locations

1 site across 1 country: United States