Sickle Cell Disease (SCD) is the most common severe monogenic disorder in the world. India accounts for 14.5 per cent of the global SCD newborns, roughly over 42,000 a year, second to sub-Saharan Africa.
We spoke to Dr Rahul Bhargava , the principal director & chief of Bone Marrow Transplant (BMT) department at Fortis Memorial Research Institute, Gurugram, Haryana. Excerpts: What is sickle cell disease (SCD) and how does it affect the body? Sickle Cell Disease (SCD) is a hereditary blood disorder where the body produces an abnormal form of haemoglobin called haemoglobin S (HbS). Generally, red blood cells are circular and flexible, allowing them to move easily through blood vessels.
However, in SCD, these cells become stiff and crescent-shaped. This irregular shape can cause the cells to cluster and obstruct blood flow, leading to a range of health issues. Pain crises (vaso-occlusive crises): Blocked blood flow can cause intense pain episodes in the chest, abdomen, and joints.
Chronic haemolytic anaemia: The sickle cells break apart easily and die, leading to a constant shortage of red blood cells. Infections: SCD weakens the spleen, making the body more vulnerable to infections. Organ damage: Persistent blockages and anaemia can cause damage to organs such as the spleen, liver, kidneys, lungs, and brain.
Stroke: Blocked blood flow to the brain can result in a stroke. It is considered the most common severe monogenic disorder in the world. What makes it such an illness? SCD i.
