Everyone is asking, “Why Cornstarch?”
Glucose is the primary fuel for the body. When a person without glycogen storage disease eats a meal, energy from the food is stored in the liver as glycogen. During periods of fasting, glycogen is broken down and glucose is released. Unfortunately, people with GSD cannot do this. The fuel gets stored, but it cannot be released. Consequently, the liver gets large, and any fasting can result in hypoglycemia, seizures, or even death.
Prior to the 1970s, glycogen storage disease was almost universally fatal. Research was performed looking for ways to maintain the blood glucose, and uncooked cornstarch was found to be best. Cornstarch is digested over a period of 4-5 hours, and it has the unique property to release glucose almost at the same rate that the body uses it. Cornstarch was initially described as a treatment in 1982, but it took over 20 years to learn how to use the product correctly. Most patients receive cornstarch every 3-4 hours during the day, and it is administered every 3-5 hours overnight.