# Feeding Difficulties in Children with Hepatic Glycogen Storage Diseases Identified by a Brazilian Portuguese Validated Screening Tool

## TL;DR

This study finds that most children with liver glycogen storage diseases face feeding difficulties, impacting caregivers' quality of life.

## Contribution

The study validates a Brazilian Portuguese screening tool for feeding difficulties in children with hepatic glycogen storage diseases.

## Key findings

- 76% of children with GSDs experienced feeding difficulties, ranging from mild to severe.
- Feeding difficulties were more severe in female patients and those who did not eat with family.
- Only one caregiver showed high parental stress, with no significant link to feeding difficulties.

## Abstract

Background/Objectives: Hepatic glycogen storage diseases (GSDs) are inherited metabolic disorders that affect glycogen synthesis or breakdown, primarily involving the liver and muscles. Treatment typically consists of strict dietary management, including the consumption of uncooked cornstarch. However, there is limited research on feeding challenges and the associated stress experienced by parents of children with GSDs. This study aims to assess feeding difficulties in children with GSDs and the level of parental stress. Methods: A total of 29 caregivers of children aged 6 months to <7 years participated. Feeding difficulties were evaluated using the Brazilian Infant Feeding Scale (Escala Brasileira de Alimentação Infantil—EBAI), while parental stress was measured using the Parental Stress Scale (Escala de Estresse Parental—EEPa). Data were collected in 2020, and the study was approved by the ethics committee. Results: The majority of the children were male (19/10), with a mean age of 47.75 months and an average age of diagnosis of 8.39 months. GSD type Ia (n = 15) and type Ib (n = 5) were the most prevalent, followed by types III and IX (n = 2). Among the participants, 22 out of 29 (76%) reported feeding difficulties, categorized as mild (n = 7, 24%), moderate (n = 7, 24%), and severe (n = 8, 28%). EBAI scores were higher in female patients and in those who did not eat meals with their family. Only one caregiver exhibited high levels of parental stress, as measured by the EEPA scale. No significant correlation was found between feeding difficulties and parental stress. Conclusions: The findings confirm a high prevalence of feeding issues in children with GSDs, which significantly affects caregivers’ quality of life. Although no significant link between feeding difficulties and parental stress was identified, further research is needed to improve GSD management and provide better support for caregivers.