Effectiveness of instructional guidelines implementation on mothers' knowledge and practice regarding their children with methylmalonic acidemia

Naglaa Fathi Mahmoud; Fatma Mohamed Amin; Manal Mohamed Ahmed Ayed

doi:10.53730/ijhs.v5nS2.13911

Authors

Naglaa Fathi Mahmoud Assistant Professor, Pediatric Nursing Department, Faculty of Nursing Cairo University, Egypt
Fatma Mohamed Amin Assistant Professor of Pediatric Nursing, Pediatric Nursing Department Faculty of Nursing Mansoura University, Egypt
Manal Mohamed Ahmed Ayed Assistant Professor of Pediatric Nursing, Faculty of Nursing, Sohag University, Egypt

Keywords:

children, instructional guidelines, knowledge, practice, methylmalonic academia, mothers

Abstract

Methylmalonic Acidemia (MMA) is a group of inborn errors of metabolism (IEMs), specifically of propionate catabolism characterized by gastrointestinal and neurometabolic manifestations resulting from a deficiency in the function of methyl malonyl-CoA mutase, methylmalonyl-CoA epimerase, and cobalamin metabolism. Aim: To investigate the effectiveness of instructional guidelines implementation on mothers' knowledge and practice regarding their children with methylmalonic academia. Design: A quasi-experimental design was used to achieve the aim of this study. Setting: This study was conducted at the Specialized Genetic Outpatient Clinic at Sohag University Hospital. Subject: A purposive sample of 50 mothers and their children with methylmalonic academia was selected from the previously selected settings. Three tools were used: (1) a structured interviewing questionnaire, (2) Mothers’ knowledge regarding methylmalonic academia, and (3) Mothers’ Reported Practices format regarding methylmalonic academia. Results: The current study results revealed that two-quarters of the mothers had unsatisfactory knowledge about methylmalonic acidemia pre-instructional guidelines implementation compared with the majority of them reported satisfactory total knowledge post-instructional guidelines implementation; As well, mothers' total reported practices were significantly improved post-instructional guidelines implementation.

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