BENGALURU : Sickle cell anaemia is an inherited form of anaemia — a condition in which there aren’t enough healthy red blood cells to carry adequate oxygen throughout your body. With an aim to spread awareness about the disease, World Sickle Cell Awareness Day is celebrated on June19 every year. It is a genetically inherited disease due to which the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
The sickle cell gene is passed from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means, both the mother and the father must pass on the defective form of the gene for a child to be affected. If only one parent passes the sickle cell gene to the child, he/she will have the sickle cell trait.
Symptoms of sickle cell anaemia include
Due to easily breakable red cells, haemoglobin levels are low and can lead to tiredness and difficulty to feed. In infants, this can usually present as excessive fussiness, sleeping without waking up for feeds, difficulty in taking sufficient feeds. The child will need to receive a blood transfusion to correct the anaemia.
Episodes of pain
Periodic episodes of pain, called crises, are a major symptom of sickle cell anaemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. Infants most often present with swelling in their fingers and toes during a pain crisis.
Sickle cells can damage an organ that fights infection (spleen), leaving the infant more vulnerable to infections. Hence, it is important for infants and children with sickle cell anaemia to have their timely vaccinations and antibiotics.
A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
The best way for a society to tackle sickle cell anaemia is by prevention. It is important for young adults with sickle cell trait to seek medical guidance and counselling to prevent the birth of a child with sickle cell anaemia if possible.The author is consultant, Paediatric Haematology Oncology and BMT Physician, BGS Gleneagles Global Hospital, Bengaluru