Current Pediatric Research

Background: Phenylketonuria is an inherited disorder that increases the levels of a substance called phenylalanine in the blood.

Aim: Improve adjustment patterns of children suffering from phenylketonuria.

Methodology: This study was conducted at genetic clinic in outpatient department belonging/ children’s hospital affiliated to Ain Shams university hospitals. A quasi experimental research design was utilized.

A purposive sample of 60 children accompanied by their mothers at the previously mentioned setting over a period of 6 months and satisfying the inclusive criteria of the study.

Three tools were utilized in this study pre/post nursing intervention. The first tool was a structured questionnaire sheet to assess children’ knowledge regarding phenylketonuria, the second tool was psychometric assessment to assess psychological problems of children with phenylketonuria; the third tool was adjustment patterns scale of children with phenylketonuria.

Results: The study findings revealed that there was statistical significant difference between the studied children’s knowledge pre and post nursing intervention implementation and there was statistical significance difference regarding to total adjustment patterns of the studied children pre and post nursing intervention implementation. Also, there was an extremely statistical significant difference and positive correlation between children’s total knowledge and total adjustment patterns pre and post nursing intervention implementation.

Conclusion: There was there was a positive effect of the nursing intervention on the adjustment patterns of children suffering from phenylketonuria.

Recommendations: Continuous health educational programs based on their actual need assessment about adjustment patterns should be provided for children suffering from phenylketonuria to cope effectively with their disease.

Author(s): Manar Fathi Hassan*, Wafaa El-Sayed Ouda, Safaa Salah Ismail

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