Battling Thalassaemia and its dilemma

A thalassaemic child taking blood transfusion at Blood Centre of Bangladesh Red Crescent Society in the city is seen with his mother.

The first thing you will notice about 8-year-old Abir is the dark circles under his eyes. They stand in stark contrast to the rest of his pale face; at a glance, he might look a pretty boy with his blue eyes, elfin frame and beautiful smile. But the circles under the eyes tell a different story. Abir looks as if he has not slept in a month for a recurring nightmare.
In fact, Abir has thalassaemia — an inherited blood disease that affects thousands of children around the world, specially in Asia. The most regrettable matter is that, people with thalassaemia have to battle life long as the disorder is not cured by existing treatment.
He needs blood in every three weeks. His thalassaemia, called beta thalassaemia major affects both genes involving production of red cell pigment haemoglobin. Abir has severe anaemia (because of early destruction of red blood cells), enlargement of spleen and liver, growth retardation. He often feels poor appetite and sleep difficulties. Thalassaemia stands for him as a recurring nightmare. It forces his poor family to lose most of their savings and properties.
Like Abir, thalassaemia affects near 322,000 children in our country. As many as 10,000 babies are born with this complex disease, every year while the country is facing very insufficient facilities for the treatment. Statistics says that 90 percent of our patients cannot afford the treatment cost and as a result, they quit treatment early. Many patients cannot have regular blood transfusion as the storage and amount of donated blood is inadequate in comparison to their need. Sometimes they face shortage of drugs that is to take regularly. All these factors make them weak in the battle against thalassaemia. Moreover, very often they do not get the proper treatment guideline.
Thalassaemia is usually diagnosed within the first six months of life and can be fatal in early childhood without any treatment. There are two different types of thalassaemia, alpha (a) and beta (b), with the more common being b-thalassaemia. The World Health Organisation (WHO) revealed in a report that 3.6 million people are the carrier of b-thalassaemia and another 4.8 million are the carrier of abnormal heamoglobin called Hb-E.
The concern arises when two carriers of b-thalassaemia wish to start a family. If two carriers conceive a child, the child has 25 per cent risk of developing thalassaemia major because they inherited the thalassaemia gene from both parents; 25 per cent chance of not inheriting the thalassaemia gene at all; 50 per cent chance of inheriting the gene from one parent and becoming a carrier.
Children with thalassaemia major are healthy at birth but become pale, irritable, weak, do not take food properly and fail to thrive. They are unable to make blood properly and become very anaemic.
Treatment
Treatment includes regular transfusions to boost up haemoglobin level in the blood. However, these transfusions can lead to a rise of iron level and cause serious side effects including diabetes, heart failure and liver disease.
Medications like Desferrioxamine can be used to remove excess iron from the blood. This medication is called 'iron chelators'. The most commonly used medication is injected using a slow infusion pump that runs over several hours.
Oral iron chelator tablets defriprone (commonly known as Kelfer) may be prescribed instead of injectable formula or in condition where the infusion treatment is not suitable or ineffective. However, these medications may have severe side effects and must be monitored carefully.
It is possible to cure thalassaemia with a bone marrow transplant, but it is difficult to find a good match and it may carry risks and complications.
Prevention
Head of the department of Haematology of Dhaka Medical College Hospital, Professor Dr M A Khan expressed his suggestions to manage thalassaemia to Star Health.
He said, "All over the world, major efforts are being made to prevent the birth of a thalassaemic child and to improve the quality of life. Thalassamia is becoming a major health concern in our country. Before starting a family, carriers should be detected and marriage between carriers should be inhibited or discouraged. Proper counselling is needed in this regard. Couples who both have thalassaemia should strictly decide not to have any children. They may choose to adopt or consider using donor sperm or eggs. Couples should also seek prenatal diagnosis with the option of terminating the pregnancy, if the foetus is diagnosed with thalassaemia major. Actually prevention of thalassaemia is possible through genetic testing and counselling."
He underscored on massive campaign for increasing awareness of the need for pre-marital blood testing. He called for greater volumes of safe blood donation by the common people to fight thalassaemia better.