الفهرس 
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Abstract
Thalassemia is one of the most common chronic inherited disorders encountered among children worldwide particularly in developing countries. It is characterized by an abnormality in Hb synthesis that results from a reduction in or absence of one of the chains found in normal Hb. This is resulting in marked RBCs damage, hemolysis, hyperbilirubinemia, ineffective erythropoiesis and severe anemia.Depending on the involved gene, the defect is identified as alpha-thalassemia or beta-thalassemia. Beta-thalassemia is the most common and severe form of thalassemia and also known as thalassemia major or Cooley’s anemia.It negatively affects all aspects of children’s QOL either physical, emotional or social functioning as well as scholastic achievement. A highly qualified nurse has a pivotal role in assessing and maintaining QOL of children with thalassemia.
The aim of the current study was to assess the QOL of children with thalassemia at University Children’s Hospital in Alexandria. The study was conducted in the Hematology clinic at the Outpatient Department of Alexandria University Children’s Hospital atEl –Shatby inAlexandria.A convenience sample of 200 children with thalassemia and their parents who fulfilled the following criteria, comprised the study subjects;had confirmed diagnosis with thalassemia, age ranged from 8 to 12 years old andfree from any other chronic disease.
Two tools were used to collect the necessary data.Tool one was the bio-socio-demographic data of parents and their children with thalassemia structuredinterview schedule which was developed by the researcher after thorough review of related literature. The second tool was the pediatric QOL scale, which was developed by Varni et.al (1998)and was adopted by the researcher to measure QOL of children. It included children and their parents’ reports. Each report consisted of four domains which arephysical functioning,emotional functioning,socialfunctioning and school functioning The childrenand their parents respondedto these statements on a five point Likert Scalethat ranged from0-4 as follows: Never = 0,Almost never = 1,Sometimes = 2,Often = 3, Almost always = 4. The total score of QOL was 92. Itwas transformed into a qualitative manner as:high QOL ”75%- 100%”,moderate QOL ”50% to less than 75%” andlow QOL ” 0% to less than 50%”.Every child and his parents were individually interviewed to collect the necessary data. The data were collected over a period of six months starting from the beginning of September to the end of February 2017.
The main results of the present study revealed the following:
• Slightly more than half of mothers (50.5%) were from 30 to less than 40 years old.
• More than half of the mothers (55.0%) were illiterate.
• The majority of the mothers (89.5%) do not work.
• More than half of the fathers (52.0%) were 40 years old or more.
• Fifty one percent of the fathers were illiterate.
• The majority of the fathers (86.5%) are working.
• Almost two thirds of the children’s families (65.0%) had not enough income.
• Nearly two thirds of children (62.0%) were from rural areas.
• Consanguinity was found among 57.0% of the parents.
• Approximately two thirds of children (62.5%) were from 10 to 12 years old.
• Female constituted more than half of the children’s sex (53.5%).
• The birth order of the children was the first for 40.5% of the study subjects.
• More than three quarters of the children (76.5%) had primary education.
• The vast majority of the children had pallor face and yellowish eyes (98.0% and 94.0% respectively).
• The majority of the children (80.5%) received blood transfusion once per month.
• All children (100.0%) received medications as iron chelation therapy (x-jet), analgesic, folic acid, L- carantine and decal.
• Nearly three quarters of the children (71.5%) had complications in the form of hemosiderosis (65.1%).
• It was reported by the studied children that they were almost always finding difficulty in taking bath and hygiene (69.0%), making activities (65.5%), walking (49.5%), doing light chores in home (49.0%), running (39.5%) and lifting something (34.5%).
• Approximately two fifths of the children were almost always feeling pain in all body and getting tired of least effort (39.5% and 36.0% respectively).
• Nearly three quarters of the children (71.0%) had ”low” QOL regarding physical functioning.
• It was noted that almost always feeling afraid, sad or being worried while waiting for medical examination and analysis were reported by 74.5%, 74.0%, and 71.0% of children respectively.
• It was obvious that almost always having sleeping troubles and any concern about what would happen in the future were mentioned by 86.0% and 78.5% of the children respectively.
• The majority of the children (87.5%) attained ”low” QOL in relation to emotional functioning.
• It was mentioned by the studies children that they were almost always had a disease limitation in social activities such as others kids do not want their friendship (88.5%), teasing from other kids (85.5%), getting a lonely with kids (84.0%), can not do things as others (69.5%) and finding difficulty when playing with kids (60.5%).
• The vast majority of the children (94.5%) had ”low” QOL concerning social functioning.
• It was reported by the studied children that they were almost always had learning difficulties related to the disease such as finding difficulty in paying attention in class (70.0%), getting a problem inschool work (70.0%) and attainingless scholastic achievement level than before (66.0%).
• Around half of the children reported that they were sometimes missing school due to illness, going to doctor as well as hospital admission (43.5% and 46.0% respectively).
• The majority of the studied children (82.5%) attained ”low” QOL considering school functioning.
• It was revealed that the total percent scores that were mentioned by the studied children were more than that were reported by their parents as well as in all domains concerning QOL (i.e. physical functioning, emotional functioning, social functioning and school functioning) with no statistical significant differences except in emotional functioning (P=0.03).
• It was clarified that there was a positive correlation and significant associations between the children’s reports and their parents’ reports in different domains of QOL as well as in the total percent score where P< 0.001.
• There were no statistical significant relations between the children’s total percent score of QOL and bio- socio demographic characteristic of either them or their parents.
• There were no statistical significant relations between the children’s total percent score of QOL and their medical history except regarding the complications occurred (P = 0.015).