Background and objectives Sickle cell disease poses a significant healthcare burden across several regions and states in India. We present findings from a sickle cell anaemia registry in the State of Jharkhand, a tribal predominant state situated in Eastern India, to provide an insight into the clinico-epidemiological profile and need-based management of sickle cell anaemia. Methods Sickle cell disease Registry at Rajendra Institute of Medical Sciences (RIMS), Ranchi, Jharkhand was started in the year 2022 and it has 334 sickle cell disease patients recruited over two and a half years. This clinical research provides systematically captured comprehensive data of sickle cell anaemia patients with HbSS genotype, including demographics, clinical presentation, laboratory findings, treatment modalities, complications, and their outcomes. Results Clinical manifestations varied from mild to severe, with the most common presentation being vaso-occlusive crises (n=257, 94.5%). A significant proportion of patients required blood transfusion n=260, (95.6%). Hydroxyurea the mainstay of treatment, was taken regularly by 136, (50%) of patients, whereas n=68, 25% were irregularly taking hydroxyurea medication and n= 68, 25% never took it. Regular intake of hydroxyurea therapy was significantly associated with reduction in pain crises and a decreased need for blood transfusion. Interpretation and conclusions Establishment of hydroxyurea monitoring units, monitoring of blood transfusion through appropriate investigations, increased utilisation of iron chelation therapy, and identification of patients with increased stroke risk can lead to improved patient care, escalated awareness and reduced rate of hospitalisation in sickle cell disease.
Prasad et al. (Sat,) studied this question.