Aims to maintain metabolic control using a combination of restrictive diet and scheduled doses of uncooked cornstarch throughout the day and night.
Metabolic Control:
- Cornstarch on average every 2-4 hours (although it varies by person).
- Missed or late doses can have very severe consequences that include hypoglycemia, hospitalizations, seizures, and death.
- Meticulous diet that is low in sugar (under 5g per meal) and low in carbs.
- Timing and quantity are very important. It’s a constant balancing act and patients are often adjusting based on a myriad of reasons.
- Finger pricks (using a glucometer) are still the most accurate way to monitor blood sugar and verify a low or high, however Continuous Glucose Monitors are now being used more commonly for GSD to help monitor blood sugar trends and alert when a low is coming.
- Majority of children w/ GSD have feeding tubes due to the criticality of cornstarch and diet.
Neutropenia:
- G-CSF – Daily injection of Granulocyte colony stimulating factor (G-CSF) to boost white blood cells.
- G-CSF has many long-term risks due the stress on the bone marrow, and long-term use has been linked to leukemia.
- SGLT2 Inhibitor – A recent discovery of an SGLT2 Inhibitor, called empagliflozin (US brand name Jardiance), has shown promise as a potential alternative to G-CSF or in conjunction with it. Learn more by going to the GSD1b Resources tab on this website.
Additional Medications / Supplements:
- Some patients take over 20 different medications and supplements to help treat symptoms brought on or related to GSD1b.
