Abstract
Background: Sickle cell disease (SCD) is more than a century old disease, but we still do not have an affordable cure for it. Although studies show that the prevalence of the disease is high in tribal areas, awareness and mainstreaming of management of SCD in the Primary Health Cares are still not formalized in the system. Our study aims to provide practical insights for imparting quality services and ensure that the provisions reach at the doors of
patients.

Objectives: The objectives of the study were to study the epidemiological profile and clinical patterns of SCD among affected tribal population.

Materials and Methods: The study was conducted at a tribal area named Sakwar. By applying suitable formula, the sample size was calculated to be 197. After obtaining informed consent, one to one interview method was used for data collection regarding health status, previous diagnosis, complication, etc. Body mass index of the patients was calculated using standard formula. SPSS version 22 was used for statistical analysis.

Results: Majority of the subjects were females (62.44%) belonging to Hindu religion 145 (73.60%) and scheduled tribes (67.01%). The study subjects were predominantly students belonging to lower socioeconomic status. About 67% of patients were having sickle cell trait while 33% had SCD. Among complications 50% had painful crisis, 36% had gallbladder stone, 15.58% had jaundice, and 5.08% study subjects had avascular necrosis. About 18.7% of study subjects had to receive blood transfusion for their disease. About 18.27% of study subjects were underweight. Majority of the study subjects were taking treatment from private practitioner while government hospitals were preferred for inpatient care and blood transfusion. We found SCD occurrence was significantly associated with female sex and consanguineous marriage. Receiving treatment from private practitioners was significantly associated with occurrence of complication among the study subjects.

Conclusion: The study found that the disease and its complication were significantly more among female sex. Occurrence of complication was more common among people taking treatment from general practitioner.

Key words: Anemia; Genetic Diseases; Health Services; Indigenous; Inborn; Rural Health; Sickle Cell