First and Common Clinical Presentation of Sickle Cell Disease in Children

Abstract

Background: sickle cell disease (SCD) is the most common inherited blood disease worldwide with the vast majority of cases occurring in sub-Saharan Africa. In Sudan, sickle cell anemia is one of the commonest chronic hemolytic anemias with high mortality and morbidity. Objective: the objective of this study is to determine the first and common presentation of sickle cell in children to assist early diagnosis, prevent complications and decrease morbidity and mortality of sickle cell disease in children. Methodology and Results: the study represented a combined descriptive study (hospital-based) and analytic study (cross-sectional observational) at Mohamed El-Amin Hamed Pediatric Emergency Hospital for Children in Omdurman, Khartoum State where 50 children diagnosed with sickle cell anemia, for the first time, aged 4 months -17 years were included during period of six months (January – June 2016). The commonest first presentation of SCD was sickle cell crises in 46%, features of infection in 18% and the rest 36% presented with combined symptoms. 62% of children presented with pallor as a feature of anemia, 32% with jaundice, 14% with fatigability, 14% with shortness of breathing and 6% presented with other symptoms related to anemia. Conclusion and Recommendations: It is clear that features of vaso-occlusive crises were the first presenting clinical features of sickle cell disease followed by features of infections and anemia. We believe that starting premarital screening, neonatal screening, expand and support SCD clinics and stablishing social support networks will help in decreasing morbidity and mortality from SCD.