Sickle cell disease (SCD)

Why in the News? 

The Indian government released updated guidelines under the Rights of Persons with Disabilities (RPWD) Act, 2016, in March 2024. These new rules create a framework for assessing the extent of disability among people affected by serious blood disorders like sickle cell disease (SCD), people who inherit both sickle cell disease and beta-thalassaemia, or those carrying the Hb D variant.

• The move was expected to help patients gain access to welfare measures such as agricultural land and housing allotments, poverty alleviation schemes, education benefits, and healthcare support. However, the government did not include SCD under the 4% reservation quota for public sector employment and higher education, which is currently extended to people with vision and hearing disabilities, locomotor disabilities, and intellectual disabilities.
• This exclusion of SCD patients from the reservation quota has sparked criticism from public health experts, activists, and tribal welfare groups, as SCD disproportionately affects marginalized and tribal communities in India. Critics argue that the decision undermines the principle of social justice and denies equal opportunities to those who face severe health and socio-economic challenges because of this lifelong condition.

Key Points: Sickle cell disease (SCD)

• The revised guidelines aim to standardize how disability caused by SCD and related conditions is measured, ensuring that affected individuals can access various welfare schemes and healthcare services. This is significant because SCD causes chronic pain, severe health complications, and often leads to progressive disability.
• Despite this progress, the lack of reservation benefits under the RPWD Act remains a major issue. 
• Activists point out that this exclusion further marginalizes communities that are already economically and socially disadvantaged. Since tribal populations in states such as Madhya Pradesh, Maharashtra, Odisha, Chhattisgarh, Jharkhand, Gujarat, Andhra Pradesh, and Tamil Nadu are most affected by SCD, not including the disease in reservations worsens inequalities and limits upward mobility for these groups.
• The controversy comes at a time when the central government has also launched the National Sickle Cell Anaemia Elimination Mission (2023-2030), which aims to eliminate SCD as a public health problem by 2047. This mission focuses on universal screening, genetic counseling, and free treatment, especially targeting high-burden tribal districts.

About Sickle Cell Disease (SCD):

• Sickle Cell Disease is a genetic blood disorder where red blood cells lose their normal round shape and instead form a crescent or sickle-like structure. This shape change causes the cells to stick together and block blood flow, leading to severe pain, oxygen deprivation, and damage to organs over time.
• The condition occurs when a child inherits two copies of the defective hemoglobin gene, one from each parent. Individuals who inherit only one copy of the gene are carriers (having the sickle cell trait) and may not show symptoms, but they can pass the gene to their children.

SCD leads to several health problems including:

• Frequent pain crises and fatigue due to anemia.
• Increased risk of infections.
• Stunted growth and delayed puberty in children.
• Vision problems.
• Severe health risks can include stroke, kidney failure, and even early death.

Currently, treatment focuses on managing symptoms through pain relief, blood transfusions, and medication like hydroxyurea. The only curative treatment is a bone marrow transplant, which is expensive and not widely accessible.

Prevalence in India:

• India has one of the highest SCD burdens in the world, with around 20 million carriers and approximately 1.4 million individuals living with the disease. It is most prevalent among Scheduled Tribe (ST) communities and certain rural populations.
• States with the highest prevalence include Madhya Pradesh, Maharashtra, Odisha, Chhattisgarh, Jharkhand, Gujarat, and some southern states like Tamil Nadu and Andhra Pradesh. These regions often face challenges like poor healthcare infrastructure and lack of awareness, making management and treatment of SCD more difficult.

National Sickle Cell Anaemia Elimination Mission (2023-2030):

• To address the growing burden of SCD, the central government launched this mission in July 2023. Its key objective is to screen every individual below 40 years of age in 200 high-prevalence districts spread across 17 states. The mission also focuses on genetic counseling for carriers, free treatment, and large-scale awareness campaigns.
• The long-term goal is to eliminate SCD as a public health problem by 2047, which is also India’s centenary year of independence.