Clinical profile of sickle cell disease in Orissa

Abstract

Children comprised 52% of patients with Sickle Cell Disease (SCD). Types of Sickle Cell Disease encountered were SS (92.7%). SB thalassaemia (6.7%) and SD disease (0.7%). The disease was widespread in almost all castes and communities in the society; largest number of patients (20%) belonging to scheduled castes and only 1.4% were from scheduled tribes. Maximum number of cases were in the age group 2–4 and 4–6 years, many of whom died around this age. Besides attacks of pain, jaundice and anemia, frequent attacks of fever with anemia or only anemia in childhood were a predominant presenting feature. Splenic sequestration was frequent (10.1%). The patients usually had a steady state hemoglobin level of 6–10 g/dl, with which they thrived well. Fetal hemoglobin was 5–30%. Blood transfusion was not a frequent requirement, but prophylactic long acting penicillin was helpful in preventing frequency of crisis.