Quality of Life and Associated Factors in Japanese Children With Inborn Errors of Metabolism and Their Families

Abstract

To reveal the associated factors of quality of life (QoL) in children with inborn errors of metabolism (IEM), their siblings, and their primary caregivers and partners, we conducted an anonymous questionnaire survey in Japan. Descriptive, correlation, and multiple regression analyses were performed. Fifty-six children with IEM, 35 siblings, 143 primary caregivers, and 86 partners completed our questionnaires. There were significant positive correlations between higher QoL in children with IEM and lower disease influence (r ¼ 0.46) and higher perceived emotional support (r ¼ 0.67). We could not find any associated factor of siblings' QoL. Lower parental distress, higher family empowerment, and higher household income contributed to higher QoL in primary caregivers (adjusted R 2 ¼ 0.636). Higher household income, lower anxiety about childrearing, and higher satisfaction in the relationship with the child and entire family contributed to higher QoL of partners (adjusted R 2 ¼ 0.398). We concluded that developing effective interventions to improve QoL is needed for the entire family in future outpatient settings.

Health Related Quality of Life of Caregivers of Children and Adolescents With Phenylketonuria: A Systematic Review

“…32 However; in another study, it was informed that it is hard to determine the relationship between the age of the caregiver and their QOL. 34 Therefore, further research is needed to determine age as a predictor for HRQOL of the caregivers of children with PKU.…”

“…20 It has been reported that the primary caregivers and their partners had some anxieties related to raising a child with IEM. 34…”

“…33 Majority of caregivers’ partners had some concerns about raising a child with IEM, and the distribution was similar to that of primary caregivers. 34…”

“…Lower parental distress scores ( P < .001), and higher scores on the J-FES family subscale ( P < .001), contributed to higher QoL of primary caregivers. 34…”

“…Unlike data from previous studies, one of the study showed that lower parental distress scores, higher scores on the J-FES family subscale, and higher levels of household income contributed to higher QOL of primary caregivers and their partners. 34…”