Sleep quality in children with hepatic glycogen storage diseases, a prospective observational pilot study

Abstract

Background

Hepatic glycogen storage diseases (GSDs) are characterised by enzyme defects affecting liver glycogen metabolism, where carbohydrate supplementation to prevent overnight hypoglycaemia is common. Concerns around sleep quality in hepatic GSDs relate to emerging evidence that overnight dysglycaemia impacts sleep quality.

Methods

This prospective observational study reported sleep quality and duration in children with hepatic GSDs over 7 days utilising: actigraphy (Actiwatch 2 by Phillips Respironics), sleep diaries, proxy reported age-appropriate sleep and quality-of-life (QoL) questionnaires, in the context of nocturnal glycaemic profiles continuous glucose monitor (CGM, Dexcom G6) and nocturnal dietary management strategies. Significant hypo- and hyperglycaemia were defined as ≥1% of sleep diary documented nocturnal period, recording <3.5 and >10.0 mmol/L, respectively.

Results

Seven children with hepatic GSD (aged 1–17 years) participated. Objective sleep quality was poor, with actigraphy demonstrating that no child achieved the minimum sleep duration recommended for age. Subjective sleep quality was also poor, with 4/5 documenting significant daytime sleepiness and 6/6 reporting poor sleep hygiene. Children prescribed overnight bolus feeds (OBF) (n = 2) recorded shorter sleep duration compared to other nocturnal management strategies. Parent-reported QoL suggested poor disease-related QoL outcomes for this cohort.

Conclusion

Objective and subjective sleep disturbances and reduced QoL are common within our sample of children with hepatic GSD. From our observations these outcomes may be linked to nutritional overnight interventions, especially OBFs, rather than overnight glucose levels. Consideration of the impacts of overnight feeding strategies on sleep quality and QoL in children with hepatic GSD should inform future management strategies.