Mobility assessment using wearable technology in patients with late-onset Pompe disease.

ABSTRACT: Late-onset Pompe disease (LOPD) is a rare genetic disorder due to the absence or deficiency of acid alpha-glucosidase enzyme resulting in slowly progressing reduction of muscle strength, causing difficulties with mobility and respiration. Wearable technologies offer novel options to evaluate mobility in a real-world setting. LOPD patients self-reporting LOPD, ≥18 years, US residents, walking (with or without aid), and not on invasive ventilation were recruited for a 6- to 8-week wearable study via patient organizations. Eligible patients were shipped a wearable tracker (Fitbit One™) and completed self-assessment questionnaires. Mobility outcome measures were median step count and peak 1-min activity. In the analyses cohort (N = 29), engagement in data sharing was high (94% of patien

SUBMITTER: Hamed A 

PROVIDER: S-EPMC6646308 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature