https://pubmed.ncbi.nlm.nih.gov/36265570/ inborn error of metabolism

J Pediatr. 2022 Oct 17;S0022-3476(22)00895-2.
doi: 10.1016/j.jpeds.2022.08.060.Online ahead of print.

Individual and family determinants for quality of life in parents of children with inborn errors of metabolism requiring a restricted diet: a multilevel analysis approach

Abdoulaye Ouattara 1, Noemie Resseguier 2, Aline Cano 3, Pascale De Lonlay 4, Jean-Baptiste Arnoux 4, Anais Brassier 4, Manuel Schiff 4, Samia Pichard 5, Alexandre Fabre 3, Celia Hoebeke 3, Nathalie Guffon 6, Alain Fouilhoux 6, Pierre Broué 7, Guy Touati 7, Dries Dobbelaere 8, Karine Mention 8, Francois Labarthe 9, Marine Tardieu 9, Loïc De Parscau 10, Francois Feillet 11, Chrystèle Bonnemains 11, Alice Kuster 12, Philippe Labrune 13, Magalie Barth 14, Lena Damaj 15, Delphine Lamireau 16, Julie Berbis 1, Pascal Auquier 1, Brigitte Chabrol 3
Affiliations expand
PMID: 36265570

DOI: 10.1016/j.jpeds.2022.08.060
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Abstract
Objective: To compare the quality of life (QoL) for parents of children with inborn errors of metabolism (IEM) requiring a restricted diet with French population norms and investigate parental QoL determinants.
Methods: This cross-sectional study included mothers and/or fathers of children < 18 years affected by IEM requiring a restricted diet (except phenylketonuria) from January 2015 to December 2017. Parents' QoL was assessed using the WHOQOL-BREF questionnaire and compared with age- and sex-matched reference values from the French general population. Linear mixed models were used to examine the effects of demographic, socioeconomic, disease-related, and psychocognitive factors on parental QoL, according to a two-level regression model considering individuals (parents) nested within families.

Results: Of the 1,156 parents invited to participate, 785 (68%) were included. Compared with the general population, parents of children with IEM requiring a restricted diet reported a lower QoL in physical and social relationship domains but a higher QoL in the psychological domain. In the multivariate analysis, characteristics associated with poorer parental QoL included both parent-related factors (being a father, older age, more educated parent, non-working parent, greater anxiety, seeking more social support and using less positive thinking and problem-solving coping strategies), and family-related factors (disease complications, increased number of hospital medical providers, child's younger age, single-parent family and lower family material wealth).

Conclusion: Parents of children with IEM requiring a restricted diet reported poorer QoL in physical and social relationship domains compared with population norms. Psychocognitive factors, beyond disease-specific and family-related characteristics, were the most important determinants influencing parental QoL and may represent essential aspects for interventions.