A Retrospective Analysis of Demographics, Clinical Features, and Treatment Patterns in Sickle Cell Disease Patients at a Tertiary Healthcare Centre of North East India.

Background Sickle cell disease (SCD) is a hereditary disorder marked by abnormal hemoglobin (HbS), leading to chronic hemolytic anemia, vaso-occlusive crises (VOCs), and multi-organ complications. In India, the prevalence of SCD is highest among tribal populations in states like Madhya Pradesh, Maharashtra, Odisha, and Assam, with the disease burden exacerbated by limited healthcare access, especially in rural regions. This study provides a comprehensive analysis of the demographic profile, clinical features, and treatment patterns of SCD patients at a tertiary healthcare center in Upper Assam, where the prevalence of SCD is high among the tea tribe communities. Methods This retrospective observational study included 250 patients diagnosed with various SCD subtypes who presented with SCD-related complications at Assam Medical College and Hospital between January 2020 and December 2023. Data were obtained from medical records in the departments of medicine and pediatrics, covering demographic variables (age, gender, ethnicity), clinical characteristics (complications, hemoglobin levels, genotype, history of hospitalizations), and treatment details (frequency and type of blood transfusions, use of hydroxyurea and chelating agents). Descriptive statistics summarized demographic and clinical features, while chi-square tests and t-tests were used for bivariate analysis. Logistic regression identified factors associated with high transfusion requirements. Results The study population had a mean age of 17.2 years, with 54.4% male predominance. Most patients (87%) had sickle cell anemia (HbSS), while the remainder had other genotypes including HbSA, HbSE, and sickle cell thalassemia. The most common presenting symptoms were fever (61.2%) and bone/joint pain (48.4%), indicative of VOCs and frequent infections. Pallor (30%) and abdominal pain (25.6%) were also prominent. Half of the patients (125) received hydroxyurea, though its uptake was limited by availability and cost. A high transfusion burden was noted, with 72.4% of patients requiring between five to 12 transfusions annually. However, only 22.4% received chelation therapy to manage iron overload, reflecting the cost constraints in accessing these agents. Laboratory findings indicated a mean hemoglobin level of 7.19 g/dL and elevated serum ferritin levels due to repeated transfusions. The frequency of blood transfusions was higher compared to Western studies, emphasizing the need for more accessible disease-modifying therapies in resource-limited settings. Conclusions The findings of this study illustrate the significant clinical and transfusion burden experienced by SCD patients in Upper Assam. This population relies heavily on blood transfusions due to limited access to hydroxyurea and other advanced therapies. The study underscores a critical need for improved access to hydroxyurea, expanded availability of chelation therapy, and greater healthcare support for managing SCD-related complications in resource-limited settings. As India's National Sickle Cell Anemia Elimination Mission is implemented, regional studies such as this are essential for tailoring public health interventions to meet the specific needs of high-prevalence areas, especially among underserved tribal communities.