Quality of life and associated factors in parents of children with late diagnosed phenylketonuria. A cross sectional study in a developing country (Tunisia)

Rim Ben Abdelaziz; Amel Ben Chehida; Henda Kachouri; Sana Ben Messaoud; Maherzia Ferchichi; Zeineb Ben Ameur; Yosra Sassi; Mohamed Slim Abdelmoula; Hatem Azzouz; Néji Tebib

doi:10.1515/jpem-2020-0035

Article

Quality of life and associated factors in parents of children with late diagnosed phenylketonuria. A cross sectional study in a developing country (Tunisia)

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Published/Copyright: July 9, 2020

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From the journal Journal of Pediatric Endocrinology and Metabolism Volume 33 Issue 7

Abstract

Objectives

We investigated the quality of life (QOL) in parents of children with late treated phenylketonuria (PKU) and its associated factors.

Methods

We conducted a cross sectional study in the reference center of inherited metabolic disease in Tunisia. We used the Tunisian version of the 36-item short-form health survey questionnaire (SF-36). We compared variables in the groups with and without impaired QOL and the SF-36 scores between subgroups of parents and children and between our sample and the Tunisian general population based on published data. We looked for associations between SF-36 scores and quantitative variables. Linear regression and logistic binary regression were used for multivariate analysis.

Results

Sixty-five parents from 42 families participated. QOL was impaired in 61% of them. The mean SF-36 score was 55.3 ± 25.07. The physical component sub-score was higher than that reported in the Tunisian general population (63.66 ± 27.77 vs. 50.11 ± 8.53; p<0.001). The mental component sub-score was comparable to that reported in the Tunisian general population (46.99 ± 25.94 vs. 47.96 ± 9.82; p=0.830). Gender (mothers) (p=0.008), low monthly income (p = 0.027), low education (p=0.011), and autism in PKU children (p = 0.001) were associated with impaired QOL.

Conclusions

We identified at risk parents for altered quality of life among parents of PKU children. Our findings were used to develop a psychological and social support strategy for at-risk parents and to promote the implementation of newborn screening of this treatable disease in our low-income country.

Keywords: autism; children; parents; phenylketonuria; quality of life; SF-36; Tunisia

Corresponding author: Rim Ben Abdelaziz, Department of Pediatrics, La Rabta Hospital, Jabbari, 1007, TunisTunisia; Faculty of Medicine of Tunis, University Tunis El Manar, Djebal Lakhdhar Street, La Rabta, 1007, Tunis, Tunisia; Research LaboratoryLR12SPO2, University Tunis El Manar, Tunis, Tunisia, E-mail: rimelair@gmail.com, Phone: 00216 98 921 440, Fax: 00216 71 570973.

Acknowledgments

Authors would like to think all the participating families.

Authors’ contributions: RBA: conceptualization, methodology, formal analysis, investigation, resources, data curation, writing original draft and project administration. ABC: conceptualization, resources, review and editing. HK: investigation, data curation and writing original draft. SBM: data curation. MF: investigation, resources. ZBA: investigation, resources. YS: investigation, resources. MSA: review and editing. HA: resources, review and editing. NT: resources, supervision
Funding sources: None declared.
Informed consent: Informed consent was obtained from all individual participants included in the study.
Ethical considerations: All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The ethics committee of our institution approved this study.
Conflict of interest: The authors declare that they have no conflict of interest.

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Received: 2020-01-27

Accepted: 2020-03-29

Published Online: 2020-07-09

Published in Print: 2020-07-28

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Articles in the same Issue

https://doi.org/10.1515/jpem-2020-0035

Keywords for this article

autism; children; parents; phenylketonuria; quality of life; SF-36; Tunisia