ASSESSMENT OF DISEASE SEVERITY IN SICKLE CELL DISEASE PATIENTS FROM THE NANDURBAR REGION OF MAHARASHTRA

Background: Sickle cell disease (SCD) is one of the most prevalent inherited hemoglobinopathies, caused by a point mutation in the B-globin gene leading to the formation of abnormal hemoglobin S (HbS). This results in sickle shaped red blood cells that cause chronic hemolytic anemia, recurrent vaso-occlusive episodes, and progressive multi-organ damage. Globally, over 7% of the population carries hemoglobin variants, and India ranks second in SCD burden, with a particularly high prevalence among tribal communities due to endogamous and consanguineous practices.

Aim and Objectives: This study aims to assess disease severity among SCD patients in the tribal-dominated Nandurbar district of Maharashtra, using a composite of clinical and hematological parameters. It seeks to address the lack of standardized severity scoring in rural and tribal populations and to support the development of region-specific treatment strategies.

Material and Method:A cross-sectional study was conducted among SCD patients from Nandurbar. Disease severity was assessed using clinical manifestations, signs and symptoms. Hematological parameters including hemoglobin (Hb) levels and white blood cell (WBC) counts were measured to support severity classification. Data were statistically analyzed to categorize patients into mild, moderate, and severe disease groups.