Sickle cell disease was a nightmare for the Tharu community of Nepal. What is its status now?

Sickle cell disease is an inherited blood disease, which causes an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells, leading it to a rigid, sickle-like shape. Under certain circumstances, it can result in multi-organ damage, severe pain, increased risk of infection, and even death if not managed appropriately.

Sickle cell disease is seen when a person inherits two abnormal copies of the β-globin gene (HBB) that makes haemoglobin, one from each parent. The person is termed as a “carrier” if he has a single abnormal copy of HBB and is said to have sickle cell trait but usually no symptoms. The problems in sickle cell disease typically begin around five months of age and an average life expectancy for them in the developed world is 40 to 60 years.