الفهرس 
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Abstract
Phenylketonuria (PKU) is an autosomal recessive disorder caused by a deficiency of the enzyme phenylalanine hydroxylase needed to convert phenylalanine to tyrosine. If left untreated PKU can cause irreversible brain and nervous system damage. The incidence of Phenylketonuria in Egypt is unknown, but its prevalence in the population is approximately 4 cases per 100,000 children’s (Fouad & Abd Elmoneem, 2016).
The main study aim was to assess the effect of self-learning package on mother’s practices towards caring for their children having Phenylketonuria through the following:
1- Assessing the mothers’ knowledge about PKU.
2- Assessing the mothers’ self-reported practices towards caring for their children with PKU.
3- Designing and implementing of self-learning package for mothers practices toward caring for their children with PKU.
4- Evaluating the outcome of self-learning package to overcome the deficit of mothers’ knowledge and reported practices.
Subject and Methods
(I) Technical design
The technical design was included research setting, subjects and tools for data collection.
Research Design:
Pre / post quasi experimental design was utilized to achieve the aim of this study.
Research Setting:
This study conducted at the Metabolic Clinic, Department of Medical Genetics, Faculty of Medicine, Ain Shams University, in Cairo.
Research Subjects:
A purposive sample was utilized to conduct this study. The pre-post analysis will include numeric scores that can be transformed into categorical variables. A sample size of at least 128 mothers accompany their children having PKU will be satisfactory to detect an effect size of at least 0.25 (small to moderate effect size) using paired t-test at level of significance of 0.05 and power of 0.80. This sample size will be satisfactory to estimate the proportion that had improved knowledge/ reported practice of 50% with 95% confidence interval of 20% (40% to 60%).
Inclusion criteria:
The study subject was selected according to the following inclusion criteria:
1- Mothers having children suffering from classical PKU.
2- Mothers having children aged from birth until 18 years of age and from both genders.
Exclusion criteria: Illiterate mothers and children having variant PKU.
Tool for data collection: pre / post self-learning package.
Data was collected using:
Pre designed interviewing questionnaire
It was written in a simple Arabic language based on updated references by the researcher to meet mothers’ needs and suit their level of understanding and reviewed by supervisors. It consists of 4 parts:
• Part I: Personal characteristics of mothers as: Age, level of education, residence, income.
• Part II: Mothers’ knowledge about PKU was adapted from (Van Spronsen et al., 2017) including questions regarding: definition, causes, symptoms and signs, diagnosis, prevention of potential complications, and different methods of treatment (dietary restriction, special medical formula and medical treatment).
• Part III: Mothers’ Reported Practice
Mothers’ reported practice was developed after reviewing the current literature (National PKU Alliance, My PKU Binder, 2011). distributed along 4 domains regarding 1) measurement of medical diet (7 items), 2) follow up of growth parameters (baby weight at home (5 items), follow up of child weight at home (5items), follow up of length at home and follow up of height at home (10 items), follow up of (BMI) (4 items), 3) dental care (7 items), and 4) physical activity (5 items).
• Part IV: Mothers Reported Behavior
The portion of the questionnaire regarding mothers’ behavior was adapted from (Harrison and Oakland, 2015). It was distributed along 8 domains related to self care (17 items), home living (18), health & safety (16), communication (13 items), functional academic (21 items), self direction (14 items), social (20 items), and leisure (13 items),
Validity:
Tools of this study were judged by a panel of professor’s expertises and they were professor of medical genetics & pediatric nursing. The necessary modifications were done according to experts’ opinion to ensure validity of the content.
Reliability:
The reliability was conducted for the developed tools. The reliability was scaled as follows: <0-0.25 weak reliability, 0.25-0.75 moderate reliability, 0.75- <1strong reliability and 1 is optimum. The reliability for this questionnaire was:
Using Alpha Cronbach Reliability Analysis of the Used Tool
Items Alpha Cronbach
Knowledge 0.799
Practice 0.813
Mothers reported behavior 0.852
(II) Operational design:
Field Work
The actual field work was carried out over a period of 9 months from beginning of May 2021 up to the end of January 2022. The researcher was available in the study setting 4 hours for 2 days/week by rotation according to the clinic time. The actual field work was divided into four phases:
Assessment phase: (2 months)
In this phase, the researcher used the constructed tools for collecting data related to knowledge of mothers having children with PKU. The purpose of the study and the expected outcomes were explained by the researcher to the studied mothers before starting data gathering. The structured questionnaire forms were filled in by the researcher. The average time ranged between 20-30 minutes for collecting data for the questionnaire.
Planning phase: (1 month)
The self-learning package was designed on the light of the determined objectives and literature review which was modified to meet the actual mother’s needs.
Self-Learning Package:
Self-learning package is a brochure designed after reading in depth in different resources and after the assessment that was done to mothers having children with PKU at medical genetic clinic and this brochure was written in a simple Arabic language to suit mothers different level of understanding, and to increase mothers’ knowledge about PKU. This will improve mothers’ practice regarding measurement of medical diet, follow up of growth, dental care and physical activity, which will have a possible impact on the quality of life of those children’s.
Self-learning package objectives:
By the end of self-learning package for mothers having children with PKU they would be able to:
(a) In relation to knowledge:
1. Illustrate definition, causes and incidence of PKU.
2. Recognize clinical manifestation, diagnosis, Prevention, complications and treatment of PKU.
(b) In relation to mothers’ reported practice related to nutrition & child health.
1. Classify the appropriate types of food list specialized for PKU.
2. Perform the nutritional medical formula for their children.
3. Handle child formula preparation by measuring cup.
4. Observe child normal growth.
5. Follow up child for Weight, Height or lenght, Body Mass Index at home.
6. Apply dental care for their childrens.
7. Learn the appropriate types of Physical activity for their childrens.
(c) In relation to behaviour:
1. Assess the child behavior related to self-care, self-direction, social skills and functional academics.
The designed self-learning package included 4 units, which covered knowledge needed for mothers having children with PKU as follows: Unit one: included: all knowledge about definition, causes, types and signs & symptoms of PKU, clinical manifestations, diagnosis, complications, prevention and treatment of PKU. Unit Two: included: Special medical formula & food instructions. Unit three: included: Follow up for growth as: (Weight, Height or length, Body Mass Index ”BMI”), Dental care, Physical activity. Unit four: Mothers’ reported behavior towards their children related to Self-care, Self-direction, Home living, Functional academic, Health and safety, Communication, leisure, and Social skills.
For each unit, there was pre/post test as well as for the self-learning package itself.
Implementing phase: (4 months)
At the beginning the researcher disscussed the self-learning package and explained to the mothers how to use the package purpose, content, how to go through the package and informed them the time allowed to study the SLP one month for the mothers. The researcher was available 2 days 4 hours a day. group meeting for 4 months & discuss all objectives of booklet content & then asking questions and answers. After one month the post assessment of knowledge and reported practice were applied.
Teaching methods:
- group discussions.
Teaching materials:
- Self –learning package.
Evaluating phase: (2 months)
The same tools were used after the implementation phase of self -learning package an indicator to determine the level of mother’s knowledge.
Exploratory phase:
A pilot study was carried out during November 2020 involved 10% (13 of mothers having children with PKU) of the total study sample. The result of the data obtained from the pilot study helped in removing of some repeated questions related to knowledge to avoid duplication of questions and then all mothers involved in the pilot study were excluded of the study sample.
(III) Statistical Design:
Data entry and statistical analysis were done using SPSS 23.0 statistical software package. Data were presented using descriptive statistics in the form of frequencies and percentages. Means, median and standard deviations for quantitative variables. Quantitative continuous data were compared using the non-parametric Kruskal-Wallis tests is used for comparing two or more independent samples of equal or different sample sizes. It extends the Mann–Whitney U test, which is used for comparing only two groups and t-test is a type of inferential statistic used to determine if there is a significant difference between the means of two groups. Qualitative categorical variables were compared using chi-square test. Spearman rank correlation was used for assessment of the inter-relationships among quantitative variables and ranked ones. Statistical significance was considered at p-value <0.05.
Results:
Findings of the current study can be summarized as the following:
More than half (55.5 %) of studied mothers’ age were 20-˂30 years old with mean score 23.7 ± 8.2.
Less than two thirds of the studied mothers had incorrect knowledge about definition pre SLP, while post SLP, more than one quarter (26.6%) of them had incorrect knowledge. Regarding symptoms of the disease, less than one fifth of them had correct knowledge while post SLP become 32.8% of them had correct knowledge.
More than three quarters 75.8% of the studied mother had unsatisfactory knowledge level at pre SLP, while post SLP, 48.4% of them had unsatisfactory knowledge level.
There was a high statistically significant difference in the total level of practice of among studied mothers at pre - post test.
Highly statistical significant difference between studied mother knowledge level and their education & residence, moreover there was a statistical significant difference between studied mother knowledge level and their age& monthly income.
A statistical significant difference between studied mothers self-reported practice and their age, monthly income, and residence.
A highly statistical significant difference between studied mother behavior and their month income. Moreover, there was a statistical significant difference between studied mother behavior and their age, and education.
Highly positive association between knowledge of studied mother and self-reported practice, reported behavior, the highest positive. In addition, the highest positively association with total self-reported practice and reported behavior.
Conclusions
Based on findings of the current study postulated that more than half of the studied mothers had poor practice level at pre SLP, while more than half of them had good practice post SLP. Also, about three quarters of the studied mothers had poor knowledge level at pre SLP, while more than half of them had good knowledge post SLP. So, this study proved its hypothesis in which self-learning package affecting positively on mother’s practices.