الفهرس 
SICKLE CELL ANEMIAOnly 14 pages are availabe for public view
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Abstract
Background: Sickle cell disease impacts the physical, emotional and psychological aspects of life of the affected persons and alters the health related quality of life. Our aim to study the pediatric quality of life score in patients with sickle cell anemia in relations to social, emotional and disease variables.
Methods: A cross sectional study was conducted in hematology clinic –Ain Shams University pediatric hospital. It included 40 patients with sickle cell disease between 5 and 18 years. Details of diagnosis, sickle cell disease complications, socioeconomic and assessment of disease severity were revised. Psychological assessment using children depression inventory (CDI), revised Children’s Manifest anxiety scale (RCMAS). Health related quality of life was done using the sickle cell module
Results: The mean age of studied patients 15.35±5.53 years, (22 55%) males and 18(45%) females, 12 (30%) had illiterate fathers and 12 (12.8%) had college degree, nineteen (48.7%) of fathers were manual workers. As regards maternal education, 20(50%) were illiterate and 39 (39.5%) were housewives. The mean age at diagnosis was 20.56±21.9 months, with a mean frequency of 0.88±1.76 vasoocclusive crisis per year, and a mean frequency of hospitalization of 3.85±3.55 per year. Severe anxiety was present in 2(5%) of patients and severe depression in 36 (90%) of patients.
We found no significant difference in total QOL score in the three studied age group(5-8, >8-13 years and>13-18 years) (P=0.440) ; however there was higher pain score in 8-13 years compared with 13-18 years age group .A significant higher scores for worrying in males 59.06±21.20 compared to females4 4.31±20.73, P=0.033.There was significant higher scores for communication problems in mothers with College degree 93.50±8.19 compared with the illiterate mothers 56.76±19.20, P=.001 and mothers with Diplome /secondary schools /institute 67.46±16.50, P=.025 with no significant difference in quality of life in relation to father education. There was significant higher quality of life scores with less than 5 hospitalization/year 53.51±14.03, compare to more than 542.99±12.75, P=.032 in addition to increased communication problems with high rate of hospitalization (P=.021). There was significant higher scores for emotions in patients on regular transfusion 58.68 ±32.98, compared to those who are not transfusion dependent.There was better scores of quality of life in patients compared with their corresponding parents.
Conclusion: The main determinants of quality of life were the frequency of hospitalization and the blood transfusion therapy. Advanced mother education improves quality of life in patients especially in communications issues.