Gycogen Storage Disease Treatments

Treatment

Treatment will depend on the type of GSD and the symptoms. Your doctor will develop a plan based on your specific symptoms.

Treatment of GSDs That Affect the Liver

These general guidelines apply to people with types I, III, IV, VI, and IX:

The goal of treatment is to maintain normal blood glucose levels. This may be done with:

Nasogastric infusion of glucose—for infants and children under age two
Dietary changes, including:
- Children over age two—frequent small carbohydrate feedings throughout the day; may include uncooked cornstarch (provides a steady slow-release form of glucose)
- Type I only—elimination of foods that are high in fructose or lactose
Allopurinol (Aloprim, Zyloprim)—reduces uric acid levels in the blood to prevent gout and kidney stones
Type IV—sometimes treated with liver transplantation

Treatment of GSDs That Affect the Muscles

These general guidelines apply to people with types V and VII:

The goal of treatment is to avoid muscle fatigue and/or cramps induced by exercise. This is done by:

Regulating or limiting strenuous exercise to avoid fatigue symptoms
Improving exercise tolerance by oral intake of glucose or fructose (fructose must be avoided in people with type I), or an injection of glucagon
Eating a high protein diet

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition. Copyright © 2024 EBSCO Publishing All rights reserved.