الفهرس 
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Abstract
Thalassemis is an autosomal recessive genetic disorder of hemoglobin synthesis characterized by a disturbance of globin chains’ production, leading to anemia. Thalassemia has a negative impact on quality of life (QOL) of affected children as they suffer in all dimensions of their lives; physically, psychologically and socially. The QOL of their families are also impacted as parenting children with chronic diseases is highly demanding and has practical and emotional consequences.
Family caregivers are important persons in the child’s care of this chronic
condition. Current evidence indicates that the management of chronic health
conditions leads to the improvement of their health outcomes and increased quality
of life.
Aim of the study:
The study was conducted to determine quality of life of thalassemic children and their families attending El-Shatby pediatrics university hospital in Alexandria and to identify family management behavior and the association between it, QOL and some other factors.
Target population and study sampling:
To achieve this aim, a cross sectional study was performed on a sample of 120 thalassemic children- caregivers pairs attending El-Shatby pediatrics university hospital in Alexandria by using simple random sampling based on hypothetical assumption that 37% of thalassemic children have good QOL.
Technical design:
A structured interview questionnaire was used to collect data and included:
a. Personal characteristics of the caregiver.
b. Child’s data.
c. Information about disease history.
d. Family management of child illness.
e. Family impact quality of life scale.
f. Child’s quality of life scales.
Results:
The results of the present study can be summarized as follows:
a. Socio-demographic characteristics of the sample:
- 63.3% of the caregivers were consanguineous.
- 45% of them were illiterates while only 7.5% were graduated from university.
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- 54.2% of the studied families had insufficient income while 41.7% had sufficient income.
- 42.5% were aged from 5 to ˂ 8 years while 35.8% were aged from 8 to ˂ 13 years with a mean age of 9.1±3.5 years.
- 41.7% of the sample had other affected siblings with thalassemia.
- The frequency of blood transfusion per year ranged from 6 to 36 with a median 12 transfusions.
b. Quality of life of thalassemic children:
- The mean percent total score of QOL of the whole study sample was 67.5.
- The most adversely affected QOL domain for the whole sample was school functioning followed by physical functioning, then, social functioning and lastly emotional functioning which represented by mean percent score 61.3±25.0, 65.7±19.6, 68.2±23.4 and 74.8±21.8 respectively.
- Children in the age category 13-18 years obtained the lowest scores in all subscales and total scale of QOL and these scores were statistically significant.
- 10.8% of children had bad and very bad levels, 54.2% of them had fair level and 35% of them had good level while none of them had very good level of mean percent total QOL score.
- No significant statistical difference was found in total QOL and its domains mean percent scores between male and female thalassemic children.
c. Quality of life of families of thalassemic children:
- The mean percent score of total family QOL was 59.5±15.
- The most affected QOL domain for parents was worry and emotional domains which scored 33.5±21.4 and 39.1±22.4 respectively.
- 62.5% had fair level, 23.3% had bad level while only 12.5% had good level regarding total family QOL.
- Families with low socio-economic status had the least mean percent score of total QOL; 56.22 while families with moderate and high socio-economic status had 61.5 and 59.6 respectively.
- Differences in QOL scores among families having different socio-economic state were statistically significant regarding social functioning domain only where those having low, moderate and high levels scored 53.51, 69.85 and 73.86 respectively.
d. Family management behavior about their children illness:
- Less than half of families had good level of management behavior (45.8%).
- The better the level of family management behavior, the higher was the mean percent score of QOL for both children and families.
- 96.7% of families had moderate level of perceived management.
- 87.5% of families had moderate level of management capability while 67.5% of them had high difficulty levels.
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e. Association between family management behavior, some contributing factors and quality of life of thalassemic children and their families:
- Perceived family management was positively and significantly correlated with family management behavior with correlation coefficient of 0.690 and p-value 0.000.
- Child total QOL was positively correlated in a significant moderate correlation with both family management behavior and total family QOL score with correlation coefficient of 0.261 and 0.305 (p-value 0.004 and 0.001) respectively.
- When applying stepwise multiple regression analysis, the following findings were found:
1- The most significant variables for child QOL were child age (a significant negative effect) and family QOL (a significant positive effect).
2- The most significant variables proved to affect family QOL were child QOL and education of caregiver (both of them had a significant positive effect).
3- Four variables have been found to be significantly associated to family management behavior; perceived family management, child scholastic achievement, education of caregiver and family QOL.
4- Two variables have been proven to have significant effect on perceived family management; family QOL (a significant negative effect) and family management behavior (a significant positive effect).
Based on the findings of the present study, many recommendations are suggested and summarized as follows:
The Egyptian health care system in coordination with school health services establishes policies to schedule blood transfusion for thalassemic
children on weekends to avoid frequent school absenteeism.
Periodic assessment of QOL domains of thalassemic children and their caregivers routinely as a policy priority of health care.
A psychologist and social workers are available in the hematological units to address psycho-social health for promoting lifelong psychosocial support to children and caregivers.
Population screening for carrier detection and genetic counseling for high risk couples.
Develop policies for raising students’ awareness about how to deal with
thalassemic students to avoid teasing and stigmatization.
School social workers assess of the psycho-social, educational and financial problems encountered by thalassemic children and their caregivers with the ultimate goal of improving their QOL.
Improving public awareness about the disease and its prevention through mass media and community workers.
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Launching thalassemia advocacy campaigns that entail social and financial support of caregivers.
Further research includes recruitment of a control group for comparison of QOL between diseased and healthy control groups. As well, exploration of other factors that affect QOL of thalassemic patients and family management practices and identifying the best intervention methods that can improve QOL.