• World Sickle Cell Awareness Day is observed annually on June 19. 

• The international awareness day is observed with the goal to increase public knowledge and an understanding of sickle cell disease, and the challenges experienced by patients and their families and caregivers.

• On December 22, 2008, the United Nations General Assembly passed a resolution acknowledging Sickle Cell Disease as a significant public health issue and one of the earliest recognised genetic disorders.

Sickle Cell Disease

• Sickle Cell Disease (SCD) is an inherited blood disorder that profoundly impacts patients throughout their lives, also known as sickle cell anemia.

• It affects hemoglobin, the protein responsible for transporting oxygen in the body, causing a group of genetic disorders affecting red blood cells.

• Normally, red blood cells are flexible and disc-shaped, facilitating smooth blood flow through vessels. In SCD, these cells adopt a crescent or “sickle” shape, hindering their movement and causing blockages in blood flow.

• Blocked blood flow can lead to serious complications such as stroke, eye problems, infections, and painful episodes known as pain crises.

• Sickle cell disease is inherited in an autosomal recessive manner, requiring two copies of the gene for the disease to manifest. A single copy results in sickle cell trait.

•  It is a lifelong condition, with blood and bone marrow transplant being the only known cure, while treatments exist to manage symptoms and prolong life.

• Symptoms typically appear early in life, often by 6 months of age, and include fatigue, irritability, jaundice, swelling in hands and feet, and frequent infections.

There are several types of SCD caused by different mutations in the hemoglobin genes:

i) Hemoglobin SS disease

ii) Hemoglobin SC disease

iii) Hemoglobin SB+ (beta) thalassemia

iv) Hemoglobin SB 0 (Beta-zero) thalassemia

• Less common types like Hemoglobin SD, SE, and SO usually exhibit milder symptoms.

• Populations from malaria-endemic regions such as Africa, India, the Mediterranean, and Saudi Arabia have a higher prevalence of carriers for SCD due to historical genetic adaptations.

Sickle Cell Disease in India

• Sickle Cell Disease is prevalent among tribal populations in India but also affects non-tribal communities. 

• It causes anemia, recurrent pain crises, stunted growth, and affects multiple organs such as the lungs, heart, kidneys, eyes, bones, and brain.

• According to the 2011 Census, 8.6 per cent of India’s population, totaling 67.8 million people, belong to tribal communities spread across various states.

• The Ministry of Health and Family Welfare (MoHFW) identified Sickle Cell Disease as one of the ten significant health challenges faced by tribal communities. This recognition underscores the need for targeted interventions.

• The National Health Mission launched efforts to address hemoglobinopathies, including thalassemia and Sickle Cell Disease. 

• Guidelines were developed to guide prevention, management, and funding provisions for screening and treatment of sickle cell disease.

• There is growing recognition of the necessity for a dedicated scheme or mission aimed at detecting, managing, preventing, and raising awareness about Sickle Cell Disease. 

• This initiative aims to improve outcomes and quality of life for affected individuals, especially among tribal populations in India.

National Sickle Cell Anaemia Elimination Mission

• The National Sickle Cell Anaemia Elimination Mission aims to ensure affordable and quality healthcare for all Sickle Cell Disease (SCD) patients and reduce disease prevalence through enhanced awareness, changed practices, and screening efforts.

• Vision: The mission envisions eliminating Sickle Cell Disease as a public health concern in India by 2047, necessitating heightened community awareness.

Implementation Strategies:

• Conduct mass screening initiatives to detect cases early and establish a robust diagnostic network.

• Implement a rigorous monitoring system to track progress effectively.

• Strengthen primary healthcare to integrate SCD-specific strategies.

• Provide training for healthcare teams at primary, secondary, and tertiary levels.

• Establish cost-effective intensive care interventions at higher-level facilities.

Mission Objectives:

• Ensure accessible and affordable healthcare for all SCD patients.

• Enhance the quality of care provided to individuals with SCD.

• Reduce the prevalence of SCD through proactive measures.

Strategic Pillars:

• Health Promotion: Focus on raising awareness and providing pre-marital genetic counseling.

• Prevention: Implement universal screening programs for early detection.

• Holistic Management & Continuum of Care: Develop comprehensive management protocols across primary, secondary, and tertiary healthcare levels, including patient support systems and community adoption.

Beneficiaries:

• The mission will operate in a phased manner, initially targeting individuals from birth to 18 years old, expanding gradually to cover those up to 40 years old.

• It will prioritise states and union territories with high SCD prevalence and tribal populations, eventually encompassing all states and Union Territories.

Geographical Focus:

• Initial focus states include Gujarat, Maharashtra, Rajasthan, Madhya Pradesh, Jharkhand, Chhattisgarh, West Bengal, Odisha, Tamil Nadu, Telangana, Andhra Pradesh, Karnataka, Assam, Uttar Pradesh, Kerala, Bihar, and Uttarakhand.

Scale and Impact:

• The mission aims to screen and provide counseling and care to approximately 70 million people within three and a half years, marking a significant step towards addressing SCD nationwide.

(The author is a trainer for Civil Services aspirants.)