How is glycogen storage disease (GSD) treated?
Treatment varies depending on the type of GSD. For types of GSD that involve the liver, treatment is aimed at keeping the right level of glucose in the blood. This is often enough to maintain the cells fuel needs and prevent long-term complications associated with poorly controlled GSD. Treatment consists of taking regular doses of uncooked cornstarch and/or nutrition supplements. Cornstarch is a complex carbohydrate that is difficult for the body to digest; therefore it maintains normal blood sugar levels for a longer period of time than most carbohydrates in food. Eating many small meals that are low in sugar can help keep blood sugar levels normal while preventing excess glycogen storage in the liver.
Carbohydrate solutions may be given continuously during the night to prevent a drop in blood glucose level during sleep, but this carries more risk of severe hypoglycemia compared to using uncooked cornstarch around the clock.
Type IV GSDs with progressive liver disease may have to be considered for liver transplantation after a thorough evaluation.