Introduction:

Sickle Cell Anaemia (SCA) is an inherited blood disorder caused by abnormal haemoglobin S (HbS), which makes red blood cells rigid and sickle-shaped, impairing blood flow.

Major impacts:

• Recurrent pain crises + severe anaemia.
• Blocked blood vessels → organ damage.
• Increased risk of infections and other complications.
• Symptoms generally begin in early childhood.

India-specific severity assessment:

• CSIR–CCMB developed the India-specific Severity Score (ISS1) using clinical data from Indian children.
• ISS1 improved clinical assessment to about 83%.
• ISS2, using easily observable clinical indicators, achieved about 85% concordance.
• Helps doctors assess disease severity and make treatment decisions with fewer laboratory investigations.
• Highlights the need for population-specific medical tools, as disease profiles can vary across populations.

Way Forward:

• Early diagnosis + genetic counselling.
• Accessible treatment and screening.
• Strengthen specialised SCA care.
• Promote research into stem-cell transplantation and gene therapy.

Conclusion:

• India-specific severity tools can enable earlier risk assessment, personalised treatment and better disease management, strengthening India’s response to Sickle Cell Anaemia.