Consultations
Patients with glycogen storage disease type VI (GSD VI), also known as Hers disease, should be referred to a dietitian experienced with glycogen storage diseases (GSDs) and the management of disorders associated with an increased risk of hypoglycemic episodes.
Diet
Dietary management is the only form of treatment necessary for this rather mild glycogen storage disease. Avoiding prolonged fasting and eating frequent meals are recommended. [16] A high-protein diet is recommended for patients with GSD VI who exhibit fasting hypoglycemia. [4, 17]
A small 2015 study reported the use of extended-release cornstarch to maintain normal glucose levels during overnight fasting in patients with different types of GSDs, including GSD VI. It was found to be beneficial in some patients. [18]
Activity
Activity should not be restricted unless significant hepatomegaly is present; recommend that patients with significant hepatomegaly avoid contact sports and activities.
Prevention
Instruct patients who exhibit episodes of fasting hypoglycemia to avoid prolonged fasts (in excess of 5-7 hours). During an acute illness with decreased oral intake, maintain normoglycemia with intravenous infusion of glucose-containing solution.
Long-Term Monitoring
Perform follow-up evaluation to assess physical growth and to prevent hypoglycemic episodes by adjusting diet, as needed.