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A clinico-epidemiological study of sickle cell anaemia in Saudi Arabia
R Mulik1, A Butikofer, G Aramouni
1Department of Paediatrics, Northwest Area Armed Forces Hospital, Tabuk, Saudi Arabia.
Insights
Sickle cell anaemia (SCA) in Saudi Arabia
Area of Science:
- Hematology
- Pediatrics
- Public Health
Background:
- Sickle cell anaemia (SCA) is a significant health concern in Saudi Arabia.
- Previous studies in the Eastern Province suggested milder disease presentation.
- The severity and management of SCA in the western and southwestern regions were less understood.
Purpose of the Study:
- To assess the clinical severity and complications of SCA in children from western and southwestern Saudi Arabia.
- To compare the disease burden with previously reported data from the Eastern Province.
- To evaluate the need for penicillin prophylaxis in this population.
Main Methods:
- Retrospective review of case notes for 99 children with SCA (3 months–15 years).
- Interviews conducted during clinic visits.
- Data collected from patients residing in the southwest and west regions of Saudi Arabia.
Main Results:
- Despite no infant screening, 73% were diagnosed before age 3, indicating early severe symptoms.
- Complications were twice as high, admissions three times higher, and pneumococcal meningitis nine times higher than in the Eastern Province.
- Hand-foot syndrome was the most common complication (58%).
Conclusions:
- SCA in western and southwestern Saudi Arabia is as severe as in American black populations.
- Current recommendations against penicillin prophylaxis based on Eastern Province data are insufficient for these regions.
- Penicillin prophylaxis is recommended for children with SCA in the southwest and west regions of Saudi Arabia.
Abstract:
Ninety-nine Saudi Arabian children aged between 3 months and 15 years attending the sickle cell anaemia (SCA) clinic were studied using a review of case notes and an interview at clinic visits. All the patients come from the southwest and west area of Saudi Arabia. Despite the lack of infant screening for SCA, 73 per cent were diagnosed before the age of 3 years, reflecting severe early symptoms of the disease. The serious complications of SCA were two times higher, the number of admissions were three times higher and the attack rate for pneumococcal meningitis was nine times higher in our study population than previously reported from the Eastern Province of the country. Hand-foot syndrome was the commonest complication affecting 58 per cent of the children. It has been stated previously that there is no need to give penicillin prophylaxis to SCA children in Saudi Arabia, based on the observations in the Eastern Province only. Our study shows that SCA in western and southwestern population of Saudi Arabia is as severe as is described in American blacks. Therefore, we recommend penicillin prophylaxis to be given to the SCA children in the southwest and west regions of the country.