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Prepublished online as a Blood First Edition Paper on April 17, 2003; DOI 10.1182/blood-2002-05-1453.

Submitted May 17, 2002
Accepted April 2, 2003
Effect of a comprehensive clinical care program on disease course in severely ill children with sickle cell anemia in a sub-Sahara Africa setting
Mohamed Cherif Rahimy*, Annick Gangbo, Gilbert Ahouignan, Roselyn Adjou, Chantal Deguenon, Stephanie Goussanou, and Eusebe Alihonou
Department of Pediatrics, Faculty of Health Sciences, Cotonou, Benin
* Corresponding author; email: mrahimy{at}syfed.bj.refer.org.
Rational: Clinical severity of sickle cell anemia (SS) in Africa may not be solely determined by genetic factors. Objective: To evaluate the effects of intensive parental education and adequate clinical care on the course of SS in children in Benin. Methods: SS children referred to the National Teaching Hospital in Cotonou were included in the study. Teaching about SS was repeated frequently, emphasizing the importance of keeping clinic appointments, improvement of the nutrition of the affected children and institution of anti-pneumococcal and anti-malarial prophylaxis. Frequency and severity of SS-related events, changes in physical growth, frequency of malarial attacks, causes of transfusion, and causes of death were the principal variables assessed. Results: 236 young children with repeated SS-related acute complications were studied from July 1, 1993 to December 31, 1999 (983 patient-years). A marked reduction in the frequency and severity of SCD-related acute events was observed. Improvement in general status and physical growth was noted in 184 patients (78%): in addition 22 of the remaining 52 patients showed similar improvement after re-motivating of the parents for compliance. There were 10 deaths, primarily in this cohort of 52 patients. Conclusion: Intensive socio-medical intervention can produce sustained clinical improvement in many severely ill SS children in sub-Saharan Africa.

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