Nutrition is the cornerstone of managing GSD1b, helping to maintain stable blood sugar levels, prevent serious complications like hypoglycemia and lactic acidosis, as well as minimizing the amount of glycogen that is stored in the liver, kidneys and other organs. Because the body cannot properly access stored glucose, carefully timed meals and specialized dietary plans are essential to provide a continuous source of energy while balancing all of these needs. Working closely with a metabolic dietitian ensures that each person’s nutritional needs are met safely and effectively. While some specific recommendations may vary between regions and medical providers, the general guidance is to maintain a diet very low in all forms of sugar (fructose, lactose, galactose, sucrose). You will find information on GSD1b diet recommendations below and be sure to watch the Ask The Expert Webinar on Diet and Nutrition in GSD1b HERE.
