Background: Haemoglobinopathies, including sickle-cell disease and thalassaemia syndrome, are a group of blood diseases mostly confined to tropical and subtropical regions of the world. The spectrum of haemoglobin variants is a group of commonly encountered genetic conditions, with an average frequency of 19.32% in Orissa, varying from region to region and from community to community depending upon the type of mating practices.

Aim: For the first time, the infant mortality rate (IMR), i.e. the number of deaths under 1 year of age (in a given year) per thousand live births in a particular area, was studied to find the cause of the high IMR and to relate it to different genotypes of haemoglobinopathies.

Results: IMR was found to be higher in couples with sickle-cell trait (75.9), ß-thalassaemia (184.2), and sickle cell/ß-thalassaemia (70.2) compared to normal couples (26.3). The reproductive wastage (abortions, stillbirths and neonatal deaths) and the number of deaths of offspring below 1 year of age (infant mortality) and below 10 years of age (childhood mortality) among affected couples in such families were also statistically significantly higher compared to normal parents.

Conclusions: The progeny of sickle-cell trait, ß-thalassaemia trait, and sickle cell/ß-thalassaemia couples contributes substantially to the high neonatal/IMR in the coastal state of Orissa in Central-Eastern India. This study has revealed that in comparison to normal couples, couples who were carriers of haemoglobinopathies had a greater reproductive wastage. Screening and genetic counselling could be an important factor in reducing IMR in rural India. The traits/carriers of haemoglobinopathies should, specifically, avoid marriages and mating for the better health of subsequent generations.