Treatment of Glycogen Storage Disease Type V
Glycogen storage disease type V is managed through a structured programme combining specific exercise strategies with dietary modification. The protocol targets measurable improvements in physical activity tolerance, muscle mass, and aerobic capacity.
Management Approach
The core of treatment is a specific activity-pacing strategy built around eliciting the ‘second-wind’ response, alongside targeted dietary measures timed around exercise. The complete regimen — including sequencing, dietary specifics, and conditioning structure — is detailed in the full protocol.
Clinical Targets
- Increased muscle mass
- Decreased baseline creatine kinase (CK)
- Improved aerobic capacity (VO2max)
References
DOI: 10.1016/j.nmd.2021.10.006
- This ‘slow-pause-resume’ pattern should be repeated as necessary until a marked improvement in physical activity tolerance is achieved.
- A carbohydrate-rich diet has proven beneficial compared to a protein-rich diet.
- In the case of GSD V, strength training can increase muscle mass, lower the severity class, decrease baseline CK, and avoid fixed muscle weakness.
- The ‘right’ type of exercise can improve VO2max.
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