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The aim of the present study is to determine if there is a change in quality and quantity of sleep perceived by adults and children with GSD and their parents while starting a modified UCCS (Glycosade) to prevent nocturnal hypoglycemia. The investigators also aim to evaluate if there is a change in quality of life perceived by adults and children and their parents with Glycosade.
This is a prospective cohort study. Patients above 2 years old and their parents (for children only) will be enrolled during their usual follow-up. Parents will be asked to complete a quality of sleep questionnaire (as it pertains to both child and parent) relating to the past month on their current dietary regimen (standard UCCS) and a quality of life questionnaire (as it pertains to the child only). Parents will then complete a sleep diary (for both child and parent) and both child and parent will wear an actigraph that will record movements during sleep over a 1 week period. Adult GSD patients will complete their own questionnaires. Following this first assessment, they will be hospitalised over a 24 hour period as part of standard of care to start the modified UCCS, Glycosade, under supervision and with a continuous glucose monitoring (CGM) sensor. Following hospitalization, the family will return home. Glucose will be monitored with the aid of the CGM sensor for 5 to 7 days. The actigraphy and the sleep diary will be repeated after 2 weeks (for 1 week) while on Glycosade. One month after starting the modified UCCS, questionnaires on quality of sleep and quality of life will be repeated.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Glycosade | A prospective cohort design will be used to assess the impact on sleep and continue to monitor safety of Glycosade. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Glycosade | Dietary Supplement |
|
| Measure | Description | Time Frame |
|---|---|---|
| The aim of the present study is to determine if there is a change in quality and quantity of sleep perceived by GSD adults and children and their parents while starting a modified UCCS (Glycosade) to prevent nocturnal hypoglycaemia. | Parents will be asked to complete a quality of sleep questionnaire (as it pertains to both child and parent) relating to the past month on their current dietary regimen (standard UCCS). Parents will also complete a sleep diary (for both child and parent) and both child and parent will wear an actigraph that will record movements during sleep over a 1 week period prior to Glycosade. The actigraphy and the sleep diary will be repeated after 2 weeks (for 2 weeks) while on Glycosade. The quality of sleep questionnaire will be repeated after 1 month on Glycosade. Adult GSD patients will complete their own questionnaires. | 2 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| To evaluate if there is a change in quality of life perceived by GSD adults and children and their parents with Glycosade. | Parents (as it pertains to their child) and adult patients will be asked to complete quality of life questionnaire prior to Glycosade and 1 month after starting this new diet. | 1 month |
| Measure | Description | Time Frame |
|---|---|---|
| To describe the variability in glucose fluctuations with Glycosade using a CGM sensor. | Glucose will be monitored with the aid of the CGM sensor for 5 to 7 days while starting Glycosade. | 1 week |
| To establish if metabolic control is maintained using Glycosade. |
Inclusion Criteria:
Exclusion Criteria:
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Patients with Glycogen Storage Disease taking uncooked cornstarch to prevent hypoglycemia followed at the Montreal Children's Hospital and St-Luc Hospital.
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| Name | Affiliation | Role |
|---|---|---|
| John J Mitchell, MD | Montreal Children's Hospital of the MUHC | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Montreal Children's hospital | Montreal | Quebec | H3H1P3 | Canada |
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Metabolic control in hospital after starting Glycosade will be defined as the absence of deterioration of biochemical data at the end of fast compared to during the fast. |
| 24 hours |
| To evaluate the safety profile of Glycosade, based on the frequency of side effects, e.g. gastrointestinal side effects while receiving Glycosade. | Subjects will be constantly monitored for adverse events throughout the study. They will be asked to keep a diary to document side effects while receiving Glycosade. They will also be asked at each visit whether they experienced any known or unknown side effects. | 1 month |
| To assess the acceptability/palatability of Glycosade. | Patient report (or parent report on behalf of child) of palatability at 4 weeks after starting Glycosade compared to their previous regimen. | 1 month |
| ID | Term |
|---|---|
| C538655 | Hepatorenal form of glycogen storage disease |
| C562594 | Glycogen Storage Disease IB |
| D006010 | Glycogen Storage Disease Type III |
| ID | Term |
|---|---|
| D006008 | Glycogen Storage Disease |
| D002239 | Carbohydrate Metabolism, Inborn Errors |
| D008661 | Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
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