Free or Reduced Priced Argo Cornstarch for those with Glycogen Storage Disease

Glycogen Storage Disease Type 1a (glucose-6-phosphatase deficiency, von Gierkes disease,) is a rare genetic metabolic disorder centered in the liver. In children afflicted with GSD1, a specific enzyme that breaks down certain carbohydrates, including glycogen (a stored form of sugar,) is either missing or not functioning properly.

Because of the missing /impaired enzyme, the body cannot create glucose from glycogen and it is difficult to maintain normal blood sugar levels between meals without constant feedings. Glucose is the essential energy source for every cell in the body. Dangerous by-products such as lactate, uric acids and triglycerides in combination with low blood sugar leads to numerous complications in GSD1 including impaired breathing, seizures, coma and death if a very strict dietary regimen is not followed.

Currently there is no cure for GSD1. The management of GSD1 is lifelong. Diet is the cornerstone of treatment and many types of foods are restricted, severely limiting dietary options. GSD1 children must minimize foods containing sucrose (table sugar,) fructose (sugar found in fruits,) and lactose and galactose (sugars found in milk products) because these sugars end up as glycogen trapped in the liver. They also must be fed every one to four hours in order to maintain blood glucose at an appropriate level. This feeding is currently done with Argo Cornstarch as this is the only type of carbohydrate that can be tolerated by those with GSD1. Because this can often be very difficult for the child to tolerate and because missing a meal or a feeding time can have catastrophic effects, most have a gastric or naso-gastric tube placed. In infancy, the tube is critical for frequent feeds during the day and for using a continuous feeding pump at night.

Argo currently is the only producer of cornstarch and they refuse give discounts to those with GSD1.