Authors
Xiaomeng Liu, Xiaojing Zhang, Yao Shu, Wei Wang
Published in
Journal of visualized experiments : JoVE. Issue 234. Aug 11, 2026. Epub Aug 11, 2026.
Abstract
Late-onset Pompe disease (LOPD) is a rare autosomal recessive lysosomal storage disorder caused by acid alpha-glucosidase deficiency, resulting in progressive skeletal and respiratory muscle weakness that may lead to chronic respiratory insufficiency and prolonged dependence on mechanical ventilation. Although enzyme replacement therapy (ERT) can slow disease progression, successful ventilator weaning often requires coordinated multidisciplinary management. This protocol describes a reproducible rehabilitation approach integrated with ERT to support ventilator weaning and extubation in patients with LOPD. The method combines pharmacological treatment with structured respiratory rehabilitation, physical training, nutritional management, psychological support, and traditional Chinese medicine-based interventions, including auricular acupuncture and seated Tai Chi. The protocol also incorporates standardized assessment procedures, individualized treatment planning, ventilator weaning strategies, and objective criteria for evaluating extubation readiness. Emphasis is placed on collaboration among physicians, rehabilitation therapists, nurses, nutritionists, and psychologists to address the complex needs of patients with neuromuscular respiratory impairment. The approach is illustrated through its application in an adult patient with LOPD who required prolonged mechanical ventilation. This protocol provides a practical framework that may be adapted across clinical settings to support respiratory recovery, rehabilitation participation, and ventilator weaning in patients with LOPD and related neuromuscular disorders.
PMID:
42611782
Bibliographic data and abstract were imported from PubMed on 19 Aug 2026.
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