Sickle Cell Disease in Eastern Uttar Pradesh, India: Sociodemographic and Clinical Profile from a Previously Undocumented Area.

BACKGROUND: Sickle cell disease (SCD) is a significant public health concern in India, with varying prevalence across regions. Data from eastern Uttar Pradesh remains scarce. Aim of the study was to describe the sociodemographic and clinical profile of patients with SCD in this previously undocumented area. METHODS: A cross-sectional study was conducted among diagnosed SCD patients attending tertiary care healthcare facility in Eastern Uttar Pradesh. Sociodemographic variables and clinical features (age at start of symptoms and diagnosis, frequency of pain episodes/crises, complications, and treatment modalities) were recorded and analyzed. RESULTS: One hundred and twenty patients (90 HbSS; 30 HbSβ) were included in the study. Majority (65%) were from Sonbhadra district (Uttar Pradesh) followed by Gadhwa and Palamu districts (Jharkhand). Hindu and muslim patients comprised 58.3% and 41.7% respectively. Other backward castes were maximally represented (58.4%) followed by scheduled tribes (21.7%), general (16.7%) and scheduled castes (3.3%). Mean age was 9.13 ± 4.08 years with male to female ratio of 2.5:1. Painful crises was experienced by 71.1% patients. Splenomegaly was present in (104) 86.7% patients. Mean HbF% was 17.71 ± 17.51 vs. 25.69 ± 9.23 in HbSS and HbSβ groups. HbF% had no correlation with frequency and intensity of pain episodes or blood transfusions. Frequency of pain episodes had positive correlation with frequency of blood transfusions. Hydroxyurea therapy was underutilized. CONCLUSION: This study provides the first detailed account of SCD in Eastern Uttar Pradesh, highlighting demographic, ethnic and clinical profile with gaps in clinical management. Findings underscore the need for targeted health interventions in this underserved region.