Prevalence of Sickle Cell

Abstract

Background: Sickle cell disease (SCD) is a common and neglected inherited disorder in the Indian tribal and nontribal population. Prevalent in scheduled populations, these are socioeconomically disadvantaged communities. SCD pathogenesis is widely studied at national and international levels which are limited to pain episodes and vaso–occlusive crisis. Objectives: In the present study, we studied the prevalence of SCD in tribal and rural population from Palghar. Materials and Methods: Subjects from primary health centers of Palghar, Maharashtra, were included in this study. Informed written consent was obtained from the all subjects. The investigation was done by solubility test and high–performance liquid chromatography, along with complete blood count. Results: Population is divided into three groups: sickle cell homozygous (HbSS), sickle cell heterozygous (HbAS), and control (HbAA). In the sample size of 5000 subjects, 1% sample was found to be affected by SCD (HbSS) and 4.08% were sickle cell heterozygous (HbAS). Comparison among hemolytic events versus vaso–occlusive single events suggests that hemolytic events, pallor and yellow sclera, counted more than other single vaso–occlusive event. Detailed screening and awareness will be the key to early intervention to reduce morbidity and mortality due to SCD. Conclusions: As SCD is becoming an increasing health concern within India, identification and creating awareness is of paramount importance. In this pilot study, heterozygous and homozygous for the sickle cell gene were explained and it is clear that SCD is a major hemoglobinopathy among the tribal people of Palghar. Further in–depth study is necessary for a proper understanding of pathogenesis of SCD.