Deadly Gift From Parents 

A seminar reveals the growing 
menace of thalassaemia, reports Biplab Das 

Six-year-old Arati has to depend on blood donors for her life. She is suffering from thalassaemia, a disease inherited from her parents. Her parents are apparently normal, but they carry the thalassaemia genes. "About 3.5 per cent of college students and 4.7 per cent of pregnant mothers in West Bengal carry the defective genes," said Prof. S. N. Banerjee, special secretary (medical education), department of health and family welfare, government of West Bengal, at a seminar, 'Awareness of School Children on Thalassaemia'. 

The seminar, organised by the Institute of Haematology and Transfusion Medicine (IHTM), was held at the Calcutta Medical College. According to Prof. Banerjee, there are around 25,000 thalassaemia patients in West Bengal. "As many as 2000 children are born with thalassaemia every year," he said. 

To check the rising number of thalassaemia patients, premarital blood tests should be made compulsory, said Dr. Sharmila Chandra, a haematologist at IHTM. The practice could prevent transmission of defective genes from one generation to the next one. Talks and health camps can play a vital role in creating awareness about premarital testing, she said. "Prevention is the only option to combat the incurable disease," said Prof. Utpal Chaudhuri, director of IHTM. 

Dr. Prantar Chakrabarti, resident medical officer, IHTM, explained the cause of thalassaemia. In thalassaemics, the shape of haemoglobin, a protein in red blood cells which carries oxygen, is distorted. Haemoglobin has two components - haem and globin. Haem is an organic molecule containing iron. Globin is the protein part containing two protein chains - alpha and beta. "In thalassaemia, the genes that make globin chains get mutated and the molecule takes on an abnormal shape," said Chakrabarti. The disease is called alpha or beta depending upon the chain that is mutated. 

"It is beta thalassaemia which dominates in West Bengal," Chakrabarti said. Apart from beta thalassaemia, there is another blood disease known as haemoglobin E disease that is prevalent here. 

In beta thalassaemia, red blood cells mature abnormally. Also, they have a very short life span and die in the bone marrow soon after they are formed. The spleen has the function of destroying abnormal red blood cells. In patients of these blood diseases, it becomes abnormally enlarged, because of the excessive workload. All these lead to anaemia. The patient has to live on donated blood. Further, destruction of large numbers of red blood cells releases lots of iron, which is deposited in the spleen, liver and skin. Medicines are needed to remove this excess iron. Patients are also advised not to eat iron-rich food. 

According to Chakrabarti, there is a 25 per cent chance of a child getting the disease when both parents carry a copy of the thalassaemia gene. At the end of his speech, Chakrabarti appealed to the young generation, especially school children to carry out awareness campaigns against thalassaemia.