Health-related quality of life of parents of children with phenylketonuria in Tehran Province, Islamic Republic of Iran

Abstract

Background: Parents of children with phenylketonuria are at risk of reduced quality of life. Aims: This study determined the quality of life of parents of children with phenylketonuria in Tehran Province. Methods: The study was conducted in 2015 and included parents of children with phenylketonuria referred to three government children's hospitals in Tehran Province that provide phenylketonuria services. Data were collected using the Farsi version of the World Health Organization Quality of Life-Bref questionnaire. Analysis of variance, t-test, Pearson correlation coefficient and multiple linear regression were used to assess the relationship between quality of life domains and sociodemographic characteristics of the parent and child. Results: The study included 240 parents; 55% were mothers. Quality of life of parents in psychological, social relationships and environment domains was low. Significant relationships were found between: physical domain and age of child at phenylketonuria diagnosis (P = 0.044); psychological domain and parent's age (P = 0.019), child's age (P = 0.007) and parent's education (P = 0.015); social relationships domain and parent's age (P = 0.003), and education (P = 0.002), household income (P = 0.025) and child's age (P = 0.004; and environmental domain and residence (P = 0.034), parent's education (P = 0.007), household income (P = 0.002) and child's age (P = 0.049). In the multivariable analysis, parent's age and education, child's age, and household income were significantly associated with parent's quality of life. Conclusion: Given the low levels of quality of life in the parents, education and more financial support are recommended.

Health-Related Quality of Life of Mothers of Children With Phenylketonuria

“…This result is confirmed by other studies such as Etemad and associates. They reported the low level of QOL in parents of children with PKU and problems that happen in the families of children with PKU [23].…”

“…Another reason may be the severity of the child's illness in low-income people due to non-compliance with the diet. Non-compliance with diet in low-and middle-income families is due to the lack of support centers, large families, and financial restrictions [23,30]. Gunduz et al reported that low-income families suffer from stress and depression due to the pressure from the high cost of the child's diet and the limitations of low income that lowers the QOL of the mother [30].…”

“…In this study, there was a significant difference in the QOL of mothers of PKU children treated before 6 and after 6 months of age. To explain the findings of current and similar studies we can state that early diagnosis of the disease followed by rapid treatment with dietary intake reduces the level of phenylalanine and its metabolites in body fluids, prevents serious brain damage [31], causes natural growth of the baby [32], and naturally reduces the complications of the PKU disease, including high costs of medication and special treatments [23] and devoting less time special care of the child [33]. Given that early treatment reduces the side effects of the disease, the QOL of mothers can be better with the early treatment of children.…”

A Policy Analysis of the National Phenylketonuria Screening Program in Iran

“…Phenylketonuria (PKU) is the most common hereditary metabolic disorder in the world [1]. Patients with PKU show high levels of phenylalanine in the blood serum, and thus develop neurological disorders such as mental retardation, seizures, behavioral problems, and developmental delays [2][3][4].…”

A Policy Analysis of the National Phenylketonuria Screening Program in Iran

“…Phenylketonuria (PKU) is the most common hereditary metabolic disorder in the world [1]. Patients with PKU show high levels of phenylalanine in the blood serum, and thus develop neurological disorders such as mental retardation, seizures, behavioral problems, and developmental delays [2][3][4]. The prevalence of the disease is reported to be 1 in 10,000 in whites and 1 to 6,000 -8,000 in the Iranian population [5].…”