Sleep and Quality of Life in Patients With Glycogen Storage Disease on Standard Versus Modified Uncooked Cornstarch

Completed

• Conditions: Glycogen Storage Disease Type IB; Glycogen Storage Disease Type III; Glycogen Storage Disease Type 0; Glycogen Storage Disease Type IA
• Interventions: Dietary Supplement: Glycosade

• Registration Number: NCT02054832
• Lead Sponsor: John Mitchell

Brief Summary

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.

Detailed Description

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.

Study Timeline

• Study Start: 2013-11
• Study First Submitted: 2014-01-31
• Study First Submitted QC: 2014-02-03
• Study First Posted: 2014-02-04
• Primary Completion: 2014-07
• Study Completion: 2014-07
• Status Verified: 2015-09
• Last Update Submitted: 2015-09-23
• Last Update Posted: 2015-09-25

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 11

Inclusion Criteria

• Patients of ≥ 2 years old with a diagnostic of GSD 0, I, III, VI, IX or XI based on a liver biopsy, mutation in the appropriate gene or clinical evidence of GSD with a positive familial history
• Medical history of fasting hypoglycemia
• Currently taking standard UCCS
• With a stable condition
• Followed in GSD clinics at the Montreal Children's Hospital and the Hôpital St-Luc
• With informed consent obtained

Exclusion Criteria

• Continuous overnight feeds

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Glycosade	Glycosade	A prospective cohort design will be used to assess the impact on sleep and continue to monitor safety of Glycosade.

Primary Outcome Measures

Name	Time	Method
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.	2 weeks	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.

Secondary Outcome Measures

Name	Time	Method
To evaluate if there is a change in quality of life perceived by GSD adults and children and their parents with Glycosade.	1 month	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.

Trial Locations

Locations (1)

Montreal Children's hospital; 🇨🇦 Montreal, Quebec, Canada