Context: Phenylketonuria (commonly known as PKU) is an inherited disorder that increases the levels of a substance called
phenylalanine in the blood. If PKU did not diagnose early in life or the affected children with PKU do not be compliant with the
treatment regimen (food), it leads to severe cognitive or behavioral problems, seizures, and autistic symptoms.
Aim: The current study aimed to evaluate the effect of empowerment program on nurses' management of children with phenylketonuria.
Methods: Quasi-experimental research (one group pre/post-test) design was used to conduct this study. The study was carried out in the
hereditary unit, and hereditary outpatient clinics at Ain Shams Specialized Hospital for children affiliated to Ain shams University. A
convenient sample of all available nurses working in the previously mentioned study settings (80 male and female bedside nurses) from
the beginning of May 2018 to the end of October 2018. Two tools were utilized for data collection for the current study. They are the
structured interview questionnaire to assess the nurses' knowledge regarding the management of children with phenylketonuria and the
nurses’ performance observation checklists regarding nursing care provided for children with phenylketonuria
Results: nurses' knowledge regarding phenylketonuria was unsatisfactory before the empowerment program, while there is a
statistically significant improvement between pre and post empowerment program implementation. Nurses' practice regarding
phenylketonuria was incompetent before the empowerment program compared to post empowerment program. There is a positive
statistical relationship between nurses' knowledge and practice post-program implementation.
Conclusion: The study revealed that the empowerment program had a positive effect on the nurses' knowledge and practice towards
the management of children with phenylketonuria. The current study recommended continuous education and training sessions about the
management of children with phenylketonuria that should be provided to upgrade the knowledge and improve the practice of nurses
caring for PKU children. Availability and accessibility of printed guidelines, posters, and simple handouts regarding the management of
children with phenylketonuria. |
