Sickle Cell Disease

Published: 5th Apr, 2024

Context

In India, the treatment and care for Sickle Cell Disease (SCD) remains grossly inadequate and inaccessible.

What is Sickle Cell Disease?

SCD is an inherited haemoglobin disorder in which red blood cells (RBCs) become crescent- or sickle-shaped due to a genetic mutation.
These RBCs are rigid and impair circulation, often leading to anaemia, organ damage, severe and episodic pain, and premature death.
India has the third highest number of SCD births, after Nigeria and the Democratic Republic of the Congo.
SCD is also one of the 21 “specified” disabilities listed in the Schedule of the Rights of Persons with Disabilities Act 2016.
In 2023, the Government of India launched the National Sickle Cell Anaemia Elimination Mission, to eliminate SCD by 2047.
Treatment and challenges:
- Blood transfusion is another important therapy for SCD, but its availability is limited to district-level facilities.
- Bone marrow transplantation (BMT) is the other cure for SCD. It is out of reach for most SCD patients due to high cost, long waiting time, difficulty in finding matched donors.
- Drug hydroxyurea lessens the severity of pain, reduces hospitalisations, and improves survival rates by increasing the size and flexibility of RBCs and lowering their likelihood of becoming sickle-shaped. However, its availability is questionable.
- The US recently approved two gene therapies, Casgevy and Lyfgenia, to treat SCD in people ages 12 and older.

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