Sickle cell trait & disease among tribal communities in Orissa, Madhya Pradesh & Kerala

A study of 2570 tribals comprising 973 from Kerala, 696 from Madhya Pradesh and 901 from Orissa revealed the frequency of sickle cell gene to vary from 0.05 to 0.31 among different communities. High frequency of the gene (0.145 or more) was observed among Chettys, Kurmars and Kondhs, who also had a substantial number of homozygous sicklers. None of those with sickle cell disease (HbSS) were more than 39 yr in age as compared with 9.9-35.3 per cent among heterozygotes (HbAS). Mean foetal haemoglobin in those with sickle cell disease varied between 9.7 to 13.5 per cent, although ti showed a slight positive correlation with total haemoglobin only among the Kondhs. Painful crises were universally observed among all tribals with sickle cell disease, with jaundice being present in 57.5 per cent of cases. Some carriers of sickle cell gene also complained of painful crises. A health plan for identifying homozygotes in infancy with appropriate medical management is highly desirable.