Lifelong management of glycogen storage disease (GSDIa)

Living with GSDIa requires frequent blood glucose monitoring and ongoing surveillance for serious complications^{1,
2}

Frequent Monitoring

Frequent blood glucose monitoring is a critical component of daily management to help ensure glucose homeostasis and guide adjustments to cornstarch dosing and nutritional plans.^1-3

Additional Monitoring

Additional monitoring is essential for detecting multisystemic complications.² A multidisciplinary care team can help determine the frequency of monitoring and type of evaluation based on the patient’s age, and other patient-specific factors.²

Understand the daily demands of GSDIa management

Sample “Day in the Life” – Living with GSDIa

Meghan is a 31-year-old woman living with GSDIa. She has been managing her condition with a cornstarch regimen comprised of 6 doses and a strict nutrition plan recommended by her dietitian. Below is a sample schedule of a day in her life.

Disclaimer: Meghan is a fictitious patient. For illustrative purposes only. Patients should work with their care team to develop an appropriate plan that meets their unique needs. This sample schedule should not be provided to patients.

Explore GSDIa resources

Abbreviation: GSDIa, glycogen storage disease type Ia.

References: 1. Kishnani PS, Austin SL, Abdenur JE, et al. Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics. Genet Med. 2014;16(11):e1. 2. Bali DS, El-Gharbawy A, Austin S, et al. Glycogen storage disease type I. 2006. In: Adam MP, Feldman J, Mirzaa GM, et al, eds. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024. Accessed June 27, 2025. https://www.ncbi.nlm.nih.gov/books/NBK1312/ 3. Derks TGJ, Rodriguez-Buritica DF, Ahmad A, et al. Glycogen storage disease type Ia: current management options, burden and unmet need. Nutrients. 2021;13(11):3828.

Lifelong Management of GSDIa

Living with GSDIa requires frequent blood glucose monitoring and ongoing surveillance for serious complications1, 2

Frequent Monitoring

Additional Monitoring

Understand the daily demands of GSDIa management

Sample “Day in the Life” – Living with GSDIa

Living with GSDIa requires frequent blood glucose monitoring and ongoing surveillance for serious complications^{1,
2}