Sickle cell anaemia is an inherited form of anaemia, in which there are not enough healthy red blood cells to carry adequate oxygen throughout the body. Normally, the red blood cells are flexible and round, moving easily through your blood vessels.
In sickle cell anaemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moon. These irregularly shaped cells can get stuck in small blood vessels and are destroyed early. They also can slow or block blood flow and oxygen to parts of the body, according to Dr. J. Chandrasekhar, consultant, internal medicine, KIMS ICON Hospital.
The cause of Sickle Cell Disease (SCD) is a defective gene, called a sickle cell gene. People with the disease are born with two sickle cell genes, one from each parent.
When a person is born with one sickle cell gene, it's called sickle cell trait. People with sickle cell trait are generally healthy, but they can pass the defective gene on to their children. A blood test can show if one has SCD or sickle cell trait.
If both the parents are either sickle cell or thalassemia trait, then the doctors can also diagnose SCD before a baby is born. That test uses a sample of amniotic fluid of the tissue taken from the placenta, said Dr. Chandrasekhar in connection with the World Sickle Day on Wednesday.
Sickle cells break apart easily and die, leaving the patient without enough red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leading to shortage of red blood cells, which causes fatigue.
Periodic episodes of pain are a major symptom of sickle cell anaemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints.
Thalassemia centre
A Thalassemia Centre has been started at the Paediatrics Ward of the KGH recently. “We are getting one or two cases new cases of SCD and thalassemia every day in addition to the old cases which come for treatment.
A majority of the cases of SCD are from the tribal areas,” says Dr. P. Venugopal, HOD of Paediatrics, Andhra Medical College.
B. Madhu Gopal