GUWAHATI: Tribals in the Northeast with sickle cell disease (SCD) live longer than fellow tribals suffering from the same disease elsewhere in India, a study conducted by Assam’s Bodoland University found.
SCD (also called sickle cell anaemia) is one of the most prevalent blood genetic disorders which is caused by sickle-shaped haemoglobin and is common among tribals.
About 2.3 per cent of the world’s population carries this defective haemoglobin. Around 44,000 children are born every year in India with SCD.
The Indian Council of Medical Research (ICMR) had entrusted different institutions to conduct a multi-centric study on the disease from 2019 to 2022 in six districts – Udalguri in Assam (Northeast), Kandhamal in Odisha (Eastern India), Mysuru in Karnataka (Southern India), Visakhapatnam in Andhra Pradesh (Southern India), Annupur in Madhya Pradesh (Central India) and Chotaudeypur in Gujarat.
In each district, four primary health centre (PHC) areas, predominantly inhabited by tribals, were identified for the study. Two were selected randomly for implementing intervention and the two others were the control area. The intervention was implemented in all villages of two selected PHC areas but the formative research and evaluation surveys were carried out in sampled villages of all four PHC areas.
Prof Jatin Sarmah, Head, Department of Biotechnology, Bodoland University, said 42 people were detected with SCD in Udalguri. He said such patients die before attaining the age of adulthood but four-five patients outlived their expected life expectancy.
“We noticed that even aged SCD patients are still surviving. Ideally, we would like to find out why their foetal haemoglobin survives naturally for long,” Prof Sarmah, who was the principal investigator of the ICMR-sponsored project in the Northeast, said.
Normally, foetal haemoglobin keeps giving support to SCD patients for 18 to 20 years, he said. "In the cases of these four-five people, we don’t know for what factors they are still getting that support."
The biotechnologist also said that no institution in India ever conducted this study but some medical colleges in the US had done so.
“Saudi Arabia is also conducting a study but our people are different. Indians have different origins and ethnicities," Prof Sarmah pointed out while stating that the expression of some genetic factors could be behind the Udalguri story.
The most common acute events of SCD are pain crisis, acute chest syndrome and lung injury syndrome. With increasing age, chronic end-organ complications begin to appear and they include chronic renal failure, haemorrhagic and non-haemorrhagic stroke, necrosis of bone and pulmonary hypertension.
The disease is prevalent among tribal populations. Remoteness, language barrier, financial hardships, poor awareness, and lack of trust in the public health care system are the main challenges in its management.
The Bodoland University research team’s co-principal investigator was Dr Silistina Narzari.
Dr Bontha V Babu, Scientist-G and Head, Division of Socio-behavioural and Health System Research, ICMR, was the national coordinator of the multi-centric project.
The ICMR initiated the country-wide study to develop a model of screening and management of SCD in the primary health care system. The intervention includes increasing awareness and preparing the communities for accessing the government health care system for SCD care and improving the capacity of the primary health care systems.