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

John Mitchell

Status

Completed

Conditions

Glycogen Storage Disease Type 0

Glycogen Storage Disease Type IA

Glycogen Storage Disease Type III

Glycogen Storage Disease Type IB

Treatments

Dietary Supplement: Glycosade

Study type

Observational

Funder types

Other

Identifiers

NCT02054832

12-337-PED

Details and patient eligibility

About

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.

Full 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.

Enrollment

11 patients

Sex

All

Ages

2 to 50 years old

Volunteers

No Healthy Volunteers

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