الفهرس 
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Abstract
O
steogenesis Imperfecta (OI) known as brittle bone disease, it is a genetic disorder of connective tissue which is characterized by bones that fracture easily from little or no apparent trauma. It is highly variable in severity from child to child ranging from very mild to lethal. However, the multiple fractures will lead to progressive bone deformities, short stature and restricted mobility (Marini et al., 2017).
All family members take an active role in keeping the child safe from fractures. They face worries that even basic handling and care could result in a fracture. Parents may doubt in their ability to care for their fragile child and wanted additional support. However, the diagnoses of OI come as a shock and the parents will be unaware of the implications for their child (Santos et al., 2018).
Nurses play a key role in guidance and support the families and ensuring that the multidisciplinary team is involved and helping to instigate a policy of continuity of care for children and their families. So, the earliest nursing intervention should be at the time of diagnosis (McDowell et al., 2019).
Aim of the study
This study aimed to evaluate the effect of guideline program intervention on mothers regarding to care of their children suffering from Osteogenesis Imperfecta (OI). This aim attainted through:
A- Assess mothers’ knowledge and reported practices regarding care of their children having osteogenesis imperfecta.
B- Design and implement guidelines program intervention for mothers regarding to care of their children suffering from osteogenesis imperfecta.
C- Evaluate the effect of guidelines program intervention on mothers’ knowledge and practices regarding to care of their children suffering from osteogenesis imperfecta.
The research hypothesis:
The guideline program intervention has a positive effect on mothers’ knowledge and practices regarding to care of their children suffering from Osteogenesis Imperfecta (OI).
Research Design:
A quasi- experimental design was utilized to conduct the study.
Technical design
The technical design was included research setting, subjects and tools for data collection.
Research Setting:
This study conducted at Genetic Department in Children’s Hospitals affiliated to Ain Shams University Hospitals.
Research Subjects:
A purposive sample of mothers accompanying their children suffering from osteogenesis imperfecta who was attended to the previously mentioned setting. The sample size was determined statistically by power analysis considering the total number of children during the year 2016-2017. Accordingly, the sample size was 80 mothers accompanying their children.
Inclusion Criteria:
The study subject was selected according to the following inclusion criteria:
- Confirmed diagnosis of OI
- Free from other physical or mental health problems
- Aged 5:15 years
Tools of data collection: pre /post intervention:
Data was collected through using the following tools pre/post intervention:
First tool:
Protocol: (Appendix I).
A questionnaire sheet (Appendix II):
It was developed in a simple Arabic language by the researcher after reviewing the related literatures and reviewed by the thesis supervisors; it was consisted of three parts as the following:
Part 1: characteristics of the study subjects:
Characteristics of the studied children which included age, gender, birth order and educational level.
Characteristics of the studied mothers which included age, educational level, occupation, residence, marital status.
Characteristics of the studied family which included type of family, number of children and adequacy of family income.
Part 2: Past and present medical history of children suffering from Osteogenesis Imperfecta which included:
Family history of OI, presence of consanguinity relation, onset of OI detection, method of detection, previous hospitalization in previous 6 months, presence of OI complications and its types, onset of first fracture, the most bone exposure to fracture, reason for attendance to genetic department, occurrence and number of fractures in the last 6 month, previous orthopedic surgery and the presence of deformity and its sites, regular follow up in genetic department. Also, it included data about nature of child movement, degree of dependence, participation in sporting activities, impact of OI on child,s school achievement, impact of OI on child,s social events and celebrations, number of sleeping hours and the presence of sleep problems and its types.
Part 3: Knowledge of mothers regarding to osteogensis imperfecta which included:
Meaning, types, causes, clinical features, diagnosis, preventive measures for complications of OI, types of conventional treatment, medication used and its precautions and side effects. Also, data was obtained related to mothers, knowledge about the compliance with medical treatment and knowledge about preventive measures for the expected complications of OI and source of mothers’ information about OI.
Scoring system:
Mothers’ answers were checked with the model key answer. Each correct answer was scored one degree and zero for incorrect answer.
Total score were summed up and accordingly, total level of mothers, knowledge was classified into:
Satisfactory level of knowledge, if score ≥ 60%.
Unsatisfactory level of knowledge, if score<60%.
Second tool: Mothers, reported practices assessment sheet (Appendix III):
It included reported practices of mothers regarding to care of their children suffering from osteogensis imperfecta which included measures to prevent injury (6 steps) avoid fracture during clothing (5 steps), first aid of fracture (7 steps), management of pain result from fracture (6 steps) and care of cast (9 steps). Also, mothers’ reported practices related to their role in dental care of their children (8 steps).
Scoring system:
Mothers’ reported practices were checked and scored as one degree to yes response and zero for no response. The total score of mothers reported practices was 41 point. All scores were summed up and accordingly, total level of mothers’ reported practices was classified into:
Adequate level of mothers reported practices, if score ≥ 60%.
Inadequate level of mothers reported practices, if score<60%.
Third tool: Quality of Life Scale for Mothers having Children Suffering from OI (WHO, 1998): (Appendix IV).
Quality of Life Scale for mothers having children with OI developed by WHO, (1998), it used to assess quality of life for mothers having children suffering from Osteogenesis Imperfecta. It was adapted by the researcher and translated into the simple Arabic language to suit the nature of the study. It included 24 self-administered scale that is a short version of the WHOQOL-100 scale. Statements were selected from this scale for data collection. The scale divided into physical domain (seven items), psychological domain (six items), social domain (three items) and environmental domain (eight items).
Scoring system:
The Quality of Life Scale for mothers having children with OI is a five point scale, the numerical values allotted to each response as following, The response options range from 1degree (very dissatisfied/very poor) to 5 degree (very satisfied/ very good).
Total scoring of the WHO Quality of Life Scale (WHOQOL):
Score ≤ 60% referred to low level of QoL.
Score between 60% < 75% referred to moderate level of QoL.
Score ≥75% referred to high level of QoL.
Fourth tool: Quality of Life Scale for Children Suffering from OI (Hill et al., 2014): (Appendix V):
Quality of Life Scale for children suffering from OI was developed by Hill et al., (2014). It was used to assess quality of life for children suffering from OI and adapted by the researcher and translated into the simple Arabic language to suit the nature of the study. It included 39 items related to child being safe and careful (six items), bodily function imbalance (eight items), child’s pain (six items), child’s fear (six items), child’s isolation (six items) and child’s independence (seven items).
Scoring system:
The Quality of Life Scale for children with OI is a five point Likert type scale, the numerical values allotted to each response as following:
The response options range from Always =5 degree, Most of the time = 4 degree, Sometimes = 3 degree, Not much = 2 degree and Never = 1 degree.
Total scoring of Quality of Life Scale for children was categorized as the following:
Score <60% referred to low level of QoL.
Score 60%: < 75% referred to moderate level of QoL.
Score ≥75% referred to high level of QoL.
Results:
Findings of the current study can be summarized as the following:
The mean age of studied mothers was 32.82±5.3 years and less than half (47.5%) of them had secondary level of education. Meanwhile, 88.7% of mothers were not working and more than half (57.5) of them were living in urban areas.
The mean age of the studied children was 8.14±5.71years and more than half (52.5%) of them were birth order as first child. Regarding to their gender, half (50%) of the studied children were boys and girls and less than half (45%) of them were not yet enrolled.
Majority (91.3%) of studied mothers had negative family history of OI and less than two thirds (63.7%) of studied mothers had consanguinity relation.
More than three quarters (78.6%) of the studied mothers had satisfactory knowledge regarding oesteogenesis imperecta post guideline program intervention compared to more than two fifth (42. 5%) of them pre guideline program intervention and this reflected statistical significant difference between pre and post guideline program intervention (x2=20.132 P - value <0.001**).
The guideline program intervention enhanced the mothers reported practices regarding to care of their children suffering from osteogensis imperfecta. where 82.9% of mothers had adequate reported practices regarding to care of their children suffering from osteogensis imperfecta post guideline program intervention compared to less than half (48.75%) of them pre guideline program intervention, and this reflected statistically significant differences pre and post guideline program intervention (X2 =19.007 P-value <0.001**).
There were statistical significant differences between pre/post guideline program interventions related to all domains of mothers, QoL. Where, 22.5% of the studied mothers had high social QoL pre guideline program intervention compared to 74.3% post guideline program intervention. In addition, this table shows that, (56.3%) of the studied mothers had low physical QOL pre guideline program intervention compared to 10% post guideline program intervention.
There were statistical significant differences between pre/post guideline program interventions related to all domains of children QOL. Where, 72.7% of the studied children had high being safe and careful in their QOL pre guideline program intervention compared 85% post guideline program intervention. In addition this table shows that, 40.9% of the studied children had low fear in their QOL pre guideline program intervention compared to 12.5% post guideline program intervention.
There was positive correlation between all variables of the study (Total knowledge, total reported practices, total QOL of mother & total QOL of child).
Conclusion:
Based on results of the current study, it can be concluded that, there was a positive effect and significant improvement of mothers’ knowledge, reported practices regarding to care of their children suffering from Osteogenesis Imperfecta (OI).Also, it was found that, their was an improvement in the quality of life of mothers and their children. Moreover, there were positive correlations between all variables of the study (total knowledge, total reported practices, total QoL of mothers and their children) post implementation of guideline program intervention.
Recommendations
In the light of findings of the present study, the following recommendations are suggested:
Educational program intervention should be conducted for mothers of children with osteogenesis imperfecta in Egypt to raise their awareness regarding care with their children with OI.
Continuous assessment for QoL for children with OI and their mothers to determine their needs for support.
Emphasis on the role of health team as a health educator to improve adherence of studied mothers to therapeutic regimen which leading to improve quality of life for mothers and their children.
Avaliability and distribution of pamphlets and booklet containing the basic knowledge and practices for mothers about the disease of their children.
Further study on large sample of mothers in Egypt to improve their practices regarding to care of their children suffering from OI.
Further researches should be conducted to determine the barriers/ challenges that affect negatively on the mothers in caring of their children with OI.