Identification and Development of a High-Risk District Model in the Prevention of β-Thalassemia in Telangana State, India

Abstract

The burden of β-thalassemia (β-thal) is largely underestimated in India with a carrier frequency of 3.0–4.0% in general, whereas highly stratified frequencies of up to 17.0% are reported in local endogamous subpopulations. We have no idea whether β-thal carrier frequencies or β-thal major (β-TM) births are increasing or decreasing in the population. The cross-sectional nature of all carrier screening programs including large-scale task force and micro level, lack of registration of β-TM births and mechanism to modulate knowledge, awareness programs in a long-term perspective, all preempt impact assessment of preventive programs. During the implementation of a Telangana State Government-sponsored program on ‘Micro profiling of β-thalassemia mutations in Telangana,’ we documented extensive in-depth demographic information on each β-TM child of the study sample that included age–sex distributions, parental and grand-parental ethnic affiliations (local endogamous group level), birth places, marital migrations, endogamy and consanguinity to identify high-risk districts as ethno-geographic regions. In Telangana State, we found β-thal is widely prevalent in 31 districts and 48 local endogamous subpopulations. The present study provided a method of identification of four ‘high-risk districts’ and developed a district model for prevention on high priority in Telangana State. The model has the advantage of impact-assessment of all preventive programs in the district.

Keywords:

• β-Thalassemia (β-thal)
• high-risk districts
• India
• prevention
• Telangana State

Acknowledgments

The authors are grateful for the cooperation of the patients and their parents, and the staff at the Thalassemia and Sickle Cell Society, Hyderabad, Telangana State, India.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Additional information

Funding

This study was supported by the Telangana Sate Council of Science and Technology (TSCOST) grant to V. R. Rao [Ref: Lr. No/TSCOST/LSR PROJ/2016-2017 Dt. 17-04-2077] is acknowledged for the successful completion of the project.