Maple syrup disease (MSD) is an inherited metabolic disease that has the capacity to
kill or cause severe neurological damage to children at early years of life. The aim of this study was to
evaluate the effect of Educational Program on nurses' management of children with Maple Syrup Urine Disease
during acute intermittent late-onset. A quasi experimental design was used to conduct this study. Settings: The
study was carried out in the Pediatric unit and hereditary unit at Ain Shams Specialized Hospital for children
affiliated to Ain shams University. Sample: A convenient sample of all nurses working in pediatric and
hereditary units (80 bedside male and female nurses) from the beginning of February 2016 to the end of July
2016. Two tools were utilized to collect data for this study; A structured Interviewing questionnaire sheet, and
observational checklists regarding nursing care provided for children with MSUD. Results: nurses' knowledge
regarding MSUD was unsatisfactory on pre – program phase, while there is a significant statistical difference
pre and post program implementation. There is a positive relation between nurses' knowledge, practice and
personal data pre and post program implementation. Conclusion: There is an improvement in nurses' practice
between pre and post program implementation regarding management of children with MSUD. The study
revealed that the program had positive effect on the nurses' knowledge and practice towards management of
children with MSUD. Recommendations: The study recommended that continuous education and training
sessions about management of children with MSUD should be provided to upgrade nurses' knowledge and
improve their practice level. Availability and accessibility of printed universal guidelines and standard
precautions regarding MSUD.