Hemophilia is the most common hematopoietic, hereditary disorder, and the third most common disease associated with the X chromosome due to a deficiency or lack of coagulation factors VIII and IX. In this disease, the body's ability to form blood clots and coagulate to prevent bleeding is disrupted if the artery ruptures [1]. The World Hemophilia Association estimates that about 400,000 people in the world and about 8,200 in Iran have the disease. In Khuzestan province, more than 400 people have hemophilia, of which Ahvaz with 198 and Dezful with 103 have the highest rate [3].
Numerous studies have shown that the quality of life of hemophilia patients is lower than that of healthy individuals [9-7]. Much research has been done on improving the quality of life of hemophilia children in the treatment and prevention of coagulation factors [11, 10], but it seems that prevention programs alone cannot guarantee quality of life.
Nazari attributes family empowerment to increasing life expectancy and improving the quality of life of patients with genetic diseases such as thalassemia and phenylketonuria [18]. Research by Teymouri et al. shows that family empowerment model improves the quality of life of parents of children with asthma and the burden of parental care [19]. Due to the lack of similar research in Iran that has examined the effect of family empowerment model on the quality of life of children with hemophilia and according to the obvious statistics of children with hemophilia in Iran, especially the city of Ahvaz, there is a need to conduct research in this field. Therefore, the aim of this study was to determine the effect of family empowerment on the quality of life of children with hemophilia in Ahvaz in 2016.
Participants
This semi-experimental study was conducted with the control group in the hematology clinic of Baqaei Hospital 2 in Ahvaz from January to February 2016. The sample size was estimated according to previous research [1] and with a score of 𝛼=0.05 and 𝛽=22.2, to be 22 pairs of children with hemophilia and the main caregiver for each group; taking into account 10% possible loss for each group, 25 pairs were considered. Criteria for entering the study included a willingness to participate in research, diagnosis of a child with hemophilia type A or B by a doctor and having a file in the hematology clinic of Baqaei Hospital 2 in Ahvaz with no history of diabetes, asthma or thalassemia. Children who participated in at least three sessions of family empowerment programs and caregivers who became incapacitated during the study and were unable to participate in the training program were excluded. Samples were randomly divided into blocks (including 6 blocks of 4) into two equal groups (25 pairs) of intervention and control.
The necessary permission was obtained from the ethics committee of Ahwaz University of Medical Sciences for research, and the research design was registered on the Iranian Clinical Trial website (IRCT2018 registration number 12123041733N1 and Th-9710 design code). Prior to sampling, written consent was obtained from all participants in the study.
Study Design
In addition to receiving routine care for 4 weeks, members of the intervention group received two 60-minute sessions each week to teach self-care behaviors based on the family empowerment model. This model was designed in 2003 by Alhani et al. [2]. The Family Empowerment Model, based on the four steps of understanding threat, problem solving, educational participation, and evaluation, was implemented by the researcher in the hospital's conference hall with the presence of primary caregivers and children with hemophilia. The sessions were conducted face-to-face with discussion, group discussion, and questions and answers between the researcher, principal caregivers, and children, along with slides and videos and pamphlets.
This pattern consisted of four steps:
Step 1: Understand the threat - sensitivity (2 sessions) and severity (1 session)
a. Sensitivity
B. Severity
Step 2: Troubleshooting (3 sessions)
Step 3: Educational Participation (2 sessions)
Step 4: Evaluation
Check variables
Before the start of the study and 4 weeks after the last intervention session, the quality of life of the participants (children with hemophilia) was measured by self-eradication method. If participants were unable to complete the questionnaire, the researcher would complete the questionnaire for them by interviewing the participants.
To measure quality of life, a special questionnaire for measuring the quality of life of children with hemophilia 8 to 12 years of age was used, which was designed by the Department of Life Quality Research of the University Center of Hamburg, Germany from 1998 to 2002 [23].
The demographic information questionnaire included: age of the child, age of the primary caregiver, gender of the child, main caregiver's occupation, primary caregiver's education, child status in the family and number of family members. The content validity of this questionnaire was investigated and confirmed by 5 faculty members of Ahvaz University of Medical Sciences.
The validity of this tool in Von Mackensen's research was calculated by convergent validity method with simultaneous completion of Kindl questionnaire and the correlation coefficient between these two tools was estimated to be higher than 0.3. The reliability of this tool was estimated by completing a questionnaire for 117 children aged 8 to 12 years and calculating the internal consistency (Cronbach's alpha) in the range of 0.60-7.79 for the dimensions of the questionnaire and 0.85 for questions [24]. The content validity of the instrument was examined and confirmed in 2013 by ten professors of Tarbiat Modares University and Tehran Univerdity. The formal validity of the instrument was also assessed on 5 children with hemophilia. The reliability of the instrument was estimated by internal consistency using Cronbach's alpha test for the dimensions between 0.7-0.8 and 0.78 for all items [29]. It should be noted that the reliability of this tool in this study was calculated by the internal consistency method using Cronbach's alpha test for 0.71-02.81 total 0.83.
Statistical Analysis
Kolmogorov-Smirnov test was used to check whether the distribution of variables was normal. To compare the quality of life score between the two groups, the independent t-test was used to compare the average quality of life score between the two groups of the t-test, and for qualitative variables, the Chi-square test and the Fisher exact test were used. Data were analyzed by SPSS software version 22 (SPSS Inc., Chicago, IL., USA). In all cases, the significance level was considered to be 0.05.
The study showed no significant difference in total score of quality of life in children (P=0.444) before intervention between the two groups; but after the intervention a significant difference was observed in terms of quality of life (P=0.030) and family dimension (P=0.019), friends (P=0.015) and perceived support (P=0.011) between the two groups.
According to the results of this study, it can be said that family-centered empowerment can be used as a low cost and easy way to improve the quality of life of hemophilia patients by nurses.
This study has been approved by the ethics committee in the research of Ahwaz University of Medical Sciences with the code of ethics of IR.AJUMS.REC.1397.571 and the code of plan of Th-9710. We would like to thank the Vice Chancellor for Research of Ahwaz University of Medical Sciences and the patients who have cooperated with us in conducting this research.
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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