ICKLE CELL ANAEMIA (SCA)

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Context

• Sickle cell anaemia (SCA), a genetic blood disorder, found mention in the Budget this year.

Sickle cell disease

• Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body.
• People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

Signs and symptoms

• Signs and symptoms of sickle cell disease usually begin in early childhood.Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain.

The disease

• The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells.
• When red blood cells sickle, they break down prematurely, which can lead to anemia.
• Anemia can cause shortness of breath, fatigue, and delayed growth and development in children.
• The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice.
• Painful episodes occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs, such as the lungs, kidneys, spleen, and brain, of oxygen-rich blood and can lead to organ damage.
• A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension), which can lead to heart failure. Pulmonary hypertension occurs in about 10 percent of adults with sickle cell disease.

Treatment

• Sickle cell disease is a lifelong illness. A blood and bone marrow transplant is currently the only cure for sickle cell disease, but there are effective treatments that can reduce symptoms and prolong life.

Way Ahead

Focus on awareness creation, universal screening, support is for capsule hydroxyurea, free blood transfusion for all Sickle cell patients (men & women), and counselling through collaborative efforts of central ministries and state governments can help in eliminating the