Sickle Cell Anemia: A Significant Public Health Challenge Impacting Many From Marginalised Communities

Sickle Cell Anemia (SCA), a genetic blood disorder, poses a significant public health challenge in India, particularly among Scheduled Caste (SC) and Scheduled Tribe (ST) communities. Characterised by abnormal, sickle-shaped red blood cells, SCA leads to severe complications such as chronic anemia, pain crises, organ damage, and reduced life expectancy.

India bears the world’s second-largest burden of sickle cell disease, with over one million affected individuals, predominantly from socioeconomically disadvantaged SC and ST populations. This article explores the spread of SCA in India, its disproportionate impact on SC and ST communities, the Indian government’s response through the National Sickle Cell Anaemia Elimination Mission, and the grassroots efforts of the Smrutishesh Madhukar Rao Tamgadge Trust in Nagpur to combat this disease.

Spread of Sickle Cell Anemia in India

Sickle Cell Anemia is caused by a mutation in the β-globin gene, resulting in the production of abnormal hemoglobin (HbS). When inherited from both parents, it manifests as sickle cell disease (SCD), while inheriting the gene from one parent results in the sickle cell trait (SCT).

In India, the prevalence of the sickle cell gene varies widely, with heterozygote (SCT) frequencies ranging from 1% to 40% across different regions and communities. The disease is particularly prevalent in central, western, and southern India, with high concentrations in states like Madhya Pradesh, Chhattisgarh, Maharashtra, Gujarat, Odisha, and Kerala.

The sickle cell gene is widespread among tribal populations, with carrier frequencies reaching up to 35% in certain tribes such as the Bhils, Madias, Pawaras, and Pardhans in Maharashtra, and the Garasiya tribe in Rajasthan. In a systematic review, the prevalence of SCD was estimated at 1.17%, SCT at 5.9%, and HbS-beta-thalassemia at 0.37%, with Madhya Pradesh, Chhattisgarh, and Maharashtra reporting the highest burdens. Studies in Nagpur, a key region for SCA research, have shown that 94% of SCD cases are linked to the Arab-Indian haplotype, indicating a genetic predisposition in central India.

The disease’s prevalence is exacerbated by socioeconomic factors such as endogamy (marrying within the same community), limited healthcare access, and low awareness, particularly in rural and tribal areas. These factors contribute to higher rates of undiagnosed cases and severe complications, including vaso-occlusive crises, severe anemia, and infections.

Impact on Scheduled Caste and Scheduled Tribe Communities

Scheduled Castes and Scheduled Tribes, constituting about 25% of India’s population as per the 2011 Census, are disproportionately affected by SCA due to genetic, socioeconomic, and environmental factors. These communities, often residing in malaria-endemic regions, have a higher prevalence of the sickle cell gene, likely due to historical selection pressure from malaria, as the sickle cell trait confers some resistance to the disease.

In central India, community screening efforts have reported SCD prevalence rates of 13% in SC populations and up to 24% in ST populations, compared to 3.4% in Other Backward Classes (OBCs).

The impact of SCA on SC and ST communities is profound:

1. Health burden: Individuals with SCD experience recurrent pain crises, severe anemia, and complications such as splenic sequestration, stroke, and organ damage. A study in Nagpur found that children with SCD had higher rates of vaso-occlusive crises and severe anemia compared to global cohorts, despite high fetal hemoglobin levels, which typically mitigate severity. Co-inheritance of α-thalassemia, common in tribal populations, may reduce some complications, but many cases still present with severe phenotypes.

2. Socioeconomic challenges: SC and ST communities often lack access to quality healthcare, leading to delayed diagnoses and inadequate management. Low literacy, seasonal migration for work, and poverty exacerbate these issues, with many families unable to afford treatments like hydroxyurea or blood transfusions. The stigma associated with SCA can also discourage individuals from seeking care or disclosing their carrier status, increasing the risk of passing the gene to offspring.

3. Reduced life expectancy and quality of life: The lifespan of SCD patients in India is significantly shortened, averaging around 40 years, due to complications and limited access to advanced care. Chronic pain and frequent hospitalizations impair education, employment, and overall well-being, perpetuating cycles of poverty in these communities.

Government of India's (GOI's) efforts: National Sickle Cell Anaemia Elimination Mission

Recognising the significant burden of SCA, particularly among tribal populations, the Government of India (GOI) launched the National Sickle Cell Anaemia Elimination Mission (NSCAEM) in July 2023, with the ambitious goal of eliminating SCD as a public health issue by 2047.

Announced in the Union Budget 2023, the mission targets 70 million individuals aged 0–40 in high-prevalence areas over its initial three years, focusing on screening, awareness, and comprehensive care. The mission prioritizes 17 high-prevalence states, including Gujarat, Maharashtra, Madhya Pradesh, Chhattisgarh, and Odisha, with a phased approach to expand coverage nationwide.

Key components of the NSCAEM include:

1. Mass screening: The mission employs solubility sickling tests for initial screening, followed by high-performance liquid chromatography (HPLC) for confirmation. Rapid point-of-care testing (POCT) devices are also being introduced to enhance accessibility in remote areas. Newborn screening programs have been initiated in states like Maharashtra, Gujarat, and Odisha to enable early detection.

2. Genetic counseling: Counsellors at primary health centers provide education on the genetic nature of SCD, advising carriers to avoid marrying other carriers to reduce the risk of affected offspring. The Ministry of Tribal Affairs leverages programs like GOAL (Going Online as Leaders) to engage tribal youth as ambassadors for awareness campaigns.

3. Advanced diagnostics and treatment: The mission supports the use of HPLC machines and collaborates with organisations like Sankalp India for prenatal testing. Drugs like hydroxyurea are included in the National Health Mission’s essential medicines list, and specialised labs, such as those at AIIMS Bhopal, facilitate newborn screening.

4. Community engagement and awareness: The mission emphasises intersectoral coordination, involving ministries like Health and Tribal Affairs to implement information, education, and communication (IEC) and behaviour change communication (BCC) strategies. Targeted media campaigns aim to reduce stigma and myths surrounding SCD, drawing on India’s successful experiences with polio and HIV campaigns.

5. Technology integration: A national sickle cell portal and mobile app have been developed for tracking and data reporting, ensuring efficient monitoring of screening and treatment outcomes.

The NSCAEM builds on earlier efforts, such as the 2016 national guidelines on hemoglobinopathies and the establishment of the National Council for SCD by the Ministry of Tribal Affairs. By addressing both prevention and management, the mission aims to improve the quality of life for SCD patients and reduce disease prevalence through informed reproductive choices.

Role of Smrutishesh Madhukar Rao Tamgadge Trust in Nagpur

In Nagpur, a region with a high burden of SCA, the Smrutishesh Madhukar Rao Tamgadge Trust has emerged as a vital grassroots organisation in the fight against sickle cell anemia. Founded in memory of Smrutishesh Tamgadge, who succumbed to SCD, the trust is dedicated to raising awareness, providing medical support, and advocating for better care for SCD patients, particularly among SC and ST communities in Maharashtra’s Vidarbha region.

Key initiatives of the trust include:

1.Screening and Awareness Campaigns: The trust organizes community-based screening camps in rural and tribal areas of Nagpur and surrounding districts, using solubility tests and partnering with local healthcare providers for HPLC confirmation. These camps are coupled with awareness sessions to educate communities about the genetic basis of SCD, the importance of carrier screening, and preventive measures like genetic counselling.

2. Medical Support and Counselling: The trust facilitates access to treatments such as hydroxyurea and blood transfusions for underprivileged SCD patients. It also provides counselling services to help patients and families manage the physical and emotional challenges of the disease, addressing stigma and encouraging early intervention.

3. Advocacy and Collaboration: The trust collaborates with government bodies, NGOs, and healthcare institutions to advocate for improved SCD care and policies. It works closely with the NSCAEM to support screening and awareness efforts, ensuring that national initiatives reach grassroots levels.

4. Support for Education and Livelihood: Recognizing the socioeconomic barriers faced by SC and ST communities, the trust offers support for education and vocational training for SCD patients and their families, aiming to break the cycle of poverty and improve long-term outcomes.

The trust’s efforts have made a tangible impact in Nagpur, where studies have highlighted severe SCD phenotypes among children. By addressing both medical and social determinants of the disease, the Smrutishesh Madhukar Rao Tamgadge Trust serves as a model for community-driven interventions in high-prevalence areas.

Challenges and the way forward

Despite significant efforts, several challenges remain in addressing SCA in India. Limited healthcare infrastructure in rural areas, low awareness, and stigma continue to hinder screening and treatment efforts. The NSCAEM’s ambitious targets require sustained funding, trained personnel, and robust supply chains for diagnostics and medications.

Additionally, ensuring equitable access to advanced treatments like gene therapy, currently in clinical trials, will be critical for long-term success.

To overcome these challenges, the government, NGOs, and community organisations must continue to collaborate. Expanding newborn screening, strengthening primary healthcare systems, and integrating SCD care into existing programs like Anemia Mukt Bharat can enhance impact.

Community engagement, as demonstrated by the Smrutishesh Madhukar Rao Tamgadge Trust, is essential to build trust and ensure culturally sensitive interventions.

Sickle Cell Anemia remains a pressing health issue in India, disproportionately affecting Scheduled Caste and Scheduled Tribe communities due to genetic predisposition and socioeconomic vulnerabilities. The National Sickle Cell Anaemia Elimination Mission represents a bold step toward addressing this burden through screening, counseling, and comprehensive care.

At the grassroots level, organisations like the Smrutishesh Madhukar Rao Tamgadge Trust in Nagpur are making a difference by bridging gaps in awareness, treatment, and support. By combining national policy with community-driven efforts, India can move closer to its goal of eliminating SCA as a public health issue by 2047, improving the lives of millions in its most marginalised communities.

The author of this article is Sandeep Madhukar Tamgadge, IPS, currently posted as Additional Director General of Police, Nagaland. He is also a carrier of sickle cell anemia trait.

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