Sleep and quality of life of patients with glycogen storage disease on standard and modified uncooked cornstarch

• Published in: Molecular genetics and metabolism 2018-03, Vol.123 (3), p.326-330
• Main Authors: Rousseau-Nepton, Isabelle, Huot, Céline, Laforte, Diane, Mok, Elise, Fenyves, Daphna, Constantin, Evelyn, Mitchell, John
• Format: Article
• Language: English
• Subjects: Actigraphy; Adult; Blood Glucose - physiology; Fasting - physiology; Female; Glucose - administration & dosage; Glycogen storage disease; Glycogen Storage Disease - blood; Glycogen Storage Disease - physiopathology; Humans; Hypoglycemia - blood; Hypoglycemia - diet therapy; Hypoglycemia - drug therapy; Hypoglycemia - physiopathology; Male; Middle Aged; Prospective Studies; Quality of Life; Sleep; Sleep - physiology; Starch; Treatment Outcome; Uncooked cornstarch; Young Adult
• ISSN: 1096-7192; 1096-7206
• DOI: 10.1016/j.ymgme.2017.09.003

Glycemic control in hepatic glycogen storage diseases (GSDs) relies on specific nutritional recommendations, including strict avoidance of a fasting period. Uncooked cornstarch (UCCS) is an important therapeutic component. A new modified UCCS, Glycosade™, was created with the objective of prolonging euglycemia. We aimed to determine the length of euglycemia on Glycosade™ using a continuous glucose monitor (CGM) and to evaluate whether longer euglycemia and thus less nighttime interruptions would improve sleep and quality of life (QoL) after the introduction of the modified cornstarch. We conducted a prospective cohort study to assess quality and quantity of sleep and quality of life (QoL) in patients with GSDs on standard UCCS and after the introduction of Glycosade™. Sleep and QoL evaluation was done for patients using validated questionnaires, a standardized sleep diary and actigraphy. Length of fast and glucose variability were determined with CGM. Nine adults with GSD Ia took part in the study. Glycosade™ introduction was done under close supervision during a hospital admission. Comparison of sleep in 9 patients showed sleep disturbances on standard UCCS that were improved with Glycosade™. QoL was normal both pre and post Glycosade™. The CGM confirmed maintenance of a longer fasting period with Glycosade™ at home. Glycosade™ represents an alternative option for GSD patients. We showed possible benefits in terms of sleep quality. We also confirmed the longer length of fast on Glycosade™. A new modified form of uncooked starch for patients with glycogen storage disease represents an alternative option as it showed a longer length of fast and improvements in sleep quality. •Glycosade has been shown to increase fasting tolerance in glycogen storage disease patients.•We document sleep disturbance and quality of life in a cohort of 9 patients.•Glycosade had a modest impact on sleep quality as measured by actigraphy.