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NCT Number: NCT02054832

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

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.

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Key information

Conditions

Glycogen Storage Disease Type IA Carbohydrate Metabolism, Inborn Errors Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Glycogen Storage Disease Glycogen Storage Disease IB Glycogen Storage Disease Type 0 Glycogen Storage Disease Type IB Glycogen Storage Disease Type III Hepatorenal form of glycogen storage disease Metabolic Diseases Metabolism, Inborn Errors Nutritional and Metabolic Diseases

Age range

2 year–50 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Montreal Children's hospital

Montreal, Quebec, H3H1P3, Canada

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Glycosade

Dietary Supplement

Primary outcomes

  1. 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.

    Time frame: 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 outcomes

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

    Time frame: 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.

Other outcomes

  1. To describe the variability in glucose fluctuations with Glycosade using a CGM sensor.

    Time frame: 1 week

    Glucose will be monitored with the aid of the CGM sensor for 5 to 7 days while starting Glycosade.

  2. To establish if metabolic control is maintained using Glycosade.

    Time frame: 24 hours

    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.

  3. To evaluate the safety profile of Glycosade, based on the frequency of side effects, e.g. gastrointestinal side effects while receiving Glycosade.

    Time frame: 1 month

    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.

  4. To assess the acceptability/palatability of Glycosade.

    Time frame: 1 month

    Patient report (or parent report on behalf of child) of palatability at 4 weeks after starting Glycosade compared to their previous regimen.

Sponsors and collaborators

Lead sponsor

John Mitchell

Other

Registry information

Official study title

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

Important dates

Study start
2013
Primary completion
2014
Study completion
2014
First posted
Feb 4, 2014
Registry last updated
Sep 25, 2015

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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