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

Glycogen Storage Disease Type IA Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT02054832
• Other study ID #: 12-337-PED
• Status: Completed
• Phase: N/A
• First received: January 31, 2014
• Last updated: September 23, 2015
• Start date: November 2013
• Est. completion date: July 2014

Study information

• Verified date: September 2015
• Source: McGill University Health Center
• Contact: n/a
• Is FDA regulated: No
• Health authority: Canada: Health Canada
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 11
• Est. completion date: July 2014
• Est. primary completion date: July 2014
• Accepts healthy volunteers: No
• Gender: Both
• Age group: 2 Years to 50 Years

Eligibility

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

Observational Model: Cohort, Time Perspective: Prospective

Related Conditions & MeSH terms

• Glycogen Storage Disease
• Glycogen Storage Disease Type 0
• Glycogen Storage Disease Type I
• Glycogen Storage Disease Type IA
• Glycogen Storage Disease Type IB
• Glycogen Storage Disease Type III

Intervention

Dietary Supplement:
• Glycosade

Locations

Country	Name	City	State
Canada	Montreal Children's hospital	Montreal	Quebec

Sponsors (1)

Lead Sponsor	Collaborator
John Mitchell

Country where clinical trial is conducted

Canada, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Other	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	No
Other	To establish if metabolic control is maintained using Glycosade.	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	Yes
Other	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	Yes
Other	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	No
Primary	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	No
Secondary	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	No