Abstract
Background
Phenylketonuria (PKU) is a metabolic disorder of phenylalanine (phe) metabolism caused by the liver enzyme phe hydroxylase deficiency resulting in mental retardation. The only treatment for this disorder is the lifelong restriction of natural protein supplemented with phe-free amino acid formula. The aim of this study was to demonstrate the beneficial effect of psychological support in relation to the educational status on mothers with PKU children, who suffered from social discrimination (SD) and damage of quality of life (QLD).
Methods
A total number of 42 mothers with PKU children with a mean age of 26.8 years took part in this study. According to their educational status, they were divided into three groups: group a, primary school (n = 13); group b, high school (n = 15) and group c, university degree (n = 14). Psychological support was given once per week for 1 year.
Results
University degree mothers achieved the best results in ameliorating their symptoms and signs. Mothers who had completed high school felt a moderate amelioration of their SD and QL, whereas those who had finished primary school only felt a mild reduction of their symptoms. Educational status of the affected mothers should be always taken into account for the classification of the affected mothers into therapeutic groups. Familial and professional environment are very important for the success of psychological support.
Conclusions
Overall, the psychological support related to symptoms and signs of this metabolic disorder is in comparison with that in diabetic patients.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
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