Diaphragm pacing and independent breathing in individuals with severe Pompe disease.

ABSTRACT:

Introduction

Pompe disease is an inherited disease characterized by a deficit in acid-α-glucosidase (GAA), an enzyme which degrades lysosomal glycogen. The phrenic-diaphragm motor system is affected preferentially, and respiratory failure often occurs despite GAA enzyme replacement therapy. We hypothesized that the continued use of diaphragm pacing (DP) might improve ventilator-dependent subjects' respiratory outcomes and increase ventilator-free time tolerance.

Methods

Six patients (3 pediatric) underwent clinical DP implantation and started diaphragm conditioning, which involved progressively longer periods of daily, low intensity stimulation. Longitudinal respiratory breathing pattern, diaphragm electromyography, and pulmonary function tests were completed when possible,

SUBMITTER: Liberati C 

PROVIDER: S-EPMC10423945 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature