Context

Last year, Prime Minister Narendra Modi initiated the National Sickle Cell Anaemia Elimination Mission with the goal of eradicating sickle cell disease as a public health issue by 2047. The launch took place in Shahdol, Madhya Pradesh. Mr. Modi's firsthand experience with this debilitating condition dates back to his tenure as Chief Minister of Gujarat, where sickle cell disease is a significant public health challenge.

Overview

• First identified in the Nilgiri Hills of northern Tamil Nadu in 1952, the sickle cell gene is now recognized as being prevalent among populations of the Deccan Plateau in central India, with a smaller concentration in northern Kerala and Tamil Nadu.
• Sickle Cell Disease is notably widespread in India, particularly among tribal communities, although it also affects non-tribal populations.
• Each year, around 150,000 to 200,000 children are born with sickle cell disease in India. The country is second in the world in terms of the burden of this disease, and approximately 50-80 percent of affected children do not live past the age of five.

Challenges in India

• With over a million people affected by sickle cell disease: India carries the world’s second-largest burden of this condition. Most of the affected individuals are located in the tribal regions spanning Odisha, Jharkhand, Chhattisgarh, Madhya Pradesh, and Maharashtra.
• Sickle cell disease results from a genetic mutation:
  • If both parents carry the sickle cell trait, their child has a high likelihood of being born with the disease. Unlike healthy individuals who have disc-shaped red blood cells, those with sickle cell disease have red blood cells that are crescent-shaped or sickle-like. This abnormal shape significantly shortens their lifespan to around 40 years and leads to a diminished quality of life due to various health complications, such as sickle cell anemia, recurrent infections, pain, swelling, and damage to vital organs.
  • Beyond health issues, patients often face social stigma, with some being labeled as “genetically inferior” or ostracized. In certain areas, the disease is seen as a “curse from God” or linked to “black magic.” The hereditary nature of sickle cell disease also impacts patients' marital and social opportunities.

Launch of the mission

• Following the launch of the Mission in 2023: the Central government has significantly intensified its focus on sickle cell disease. Among various initiatives, a nationwide large-scale screening program is currently in progress. Hydroxyurea, a crucial medication for treating sickle cell disease, has been added to the essential medicines list, thereby improving its accessibility. Although these measures will aid in the detection and treatment of sickle cell disease, numerous challenges remain.
• Only few receiving treatment :Estimates indicate that only 18% of individuals affected by sickle cell disease in India receive consistent treatment. This low rate of adherence is due to patients discontinuing care at various stages: during screening, diagnosis, initiation of treatment, and throughout the treatment process.
• Challenge in accurate diagnosis: The most significant drop-offs occur at the diagnosis and treatment adherence stages. Obtaining an accurate diagnosis is challenging because many individuals avoid seeking help due to the stigma associated with the condition. Instead, they often turn to traditional healers, who commonly misdiagnose the disease. Although the public healthcare system has better diagnostic capabilities for sickle cell disease, there is a longstanding mistrust of it in tribal areas, leading to a low rate of testing among patients.
• Issue in treatment adherence: While there is no permanent cure for sickle cell disease, ongoing research into gene therapy is promising but likely to be too expensive for most patients. Currently, affordable medications like hydroxyurea are effective if used correctly, but supply is often inconsistent and lacks proper adherence support. Patients frequently face stock shortages and must travel long distances to obtain their medicines; for instance, some travel over 200 kilometres in central India. Additionally, vaccination coverage, which is crucial for reducing infections and improving quality of life, remains insufficient.

Way ahead

The Mission can build on its initial successes by addressing several key challenges. Firstly, reducing the stigma associated with sickle cell disease and increasing trust in public health institutions are crucial. Targeted media campaigns should be implemented to dispel specific myths related to the disease, which vary by region and tribe. India can leverage its experience from polio and HIV campaigns to inform these efforts. As stigma decreases, individuals may be less likely to hide their carrier status, potentially leading to fewer births of children with the condition.

• Firstly, increasing newborn screening could be highly beneficial, given that cases are frequently overlooked and diagnoses are often delayed. This approach is cost-effective and would be particularly useful in regions where the condition is prevalent.
• Secondly, medications and adherence support should be readily accessible at local health and wellness centers. For managing complications, interdisciplinary centers of excellence at the district or division levels should be established.
• Thirdly, ensuring that all patients receive approved vaccinations is essential, which may necessitate catch-up vaccination programs.
• Fourthly, healthcare services in tribal areas should be strengthened by addressing the unique conditions of these regions. Adequate funding for healthcare is also necessary.
• Finally, research should be undertaken to gain a deeper understanding of the disease and its pathways in India, and to develop new treatments. Philanthropists and civil society members should play a proactive role, collaborating with both Central and State governments to support these efforts.

Conclusion

The National Sickle Cell Anaemia Elimination Mission, launched by Prime Minister Narendra Modi, represents a significant step towards tackling sickle cell disease in India. Despite these efforts, numerous challenges remain, including stigma, low treatment adherence, and inconsistent access to medications and vaccinations. To build on the mission's initial successes, India must enhance newborn screening, ensure availability of medications and adherence support, improve vaccination coverage, strengthen healthcare in tribal areas, and support ongoing research. Addressing these areas comprehensively, with the active involvement of both public and private sectors, will be crucial for making substantial progress towards eliminating sickle cell disease as a public health issue by 2047.

Source: The Hindu