The Impact of Children's Temperament, Parents' Rearing Attitudes and Parenting Stress on the Compliance in Children with Glycogen Storage Disease
Date Issued
2015
Date
2015
Author(s)
Wang, Ching-Ning
Abstract
Background Glycogen storage disease (GSD) is a rare autosomal recessive inborn error of metabolism resulting in the defect in glucose synthesis. This defect resulted in the glycogen storage in liver and muscle, and presenting with hypoglycemia, lactic academia, and hyperlipidemia in hepatic forms of GSD. Current treatment is carbohydrate restriction and utilization of corn starch. The goal is to prevent hypoglycemia. In this study, we explore if children characters, parental attitude, and parental stress will affect patients compliance. Material and method Patients diagnosed with hepatic form of GSD were enrolled into this study. Questionnaires to explore patients and parents’ characters, parental raising attitude, and parental stress were used. Medical charts for the patients’ treatment outcome and diagnosis information were reviewed and collected for analysis. Results Totally 26 GSD patients were enrolled into this study. The mean age of diagnosis of was 1.6±1.4 years (range 0-6 years) and current age was 12.1±8.9 years (range 0.4-37.6). In GSD Ia patients, 60% alleles are G727T; 32.5% alleles are G327A. After treatment initiation, their liver function tests, triglycerides and uric acid levels could be decreased. More than 80% of patients regarded themselves as having good compliance, however, some complained about bad odor, drink inadequate amount, and causing conflict to parent-child relationship. For patients with good compliance, they could have less cholesterol levels (p = 0.004) and liver function tests (p = 0.001,0.004). For patients who were less than 18 years old, better compliance correlated with better body height (p = 0.004). Children in this study tended to have high emotion response threshold, low degree of regularity, no avoidance, low adaptive ability, low emotion response, and easy to have bad mood. For patients with good compliance, they tended to have even higher emotion response threshold. Parents’ rearing attitudes didn’t affect compliance. Parents in this study tend to have higher parental stress but parents of good compliance had less stress. Conclusion Patient compliance to corn starch treatment needs further improve. Good compliance correlated with better treatment outcomes and less parental stress. Methods to rear those children especially suitable for their specific temperament may help to decrease parental stress and further improve the compliance.
Subjects
Glycogen storage disease
Compliance
Parenting stress
Parents' rearing attitudes
Children's temperament
Type
thesis
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