It was Shifa’s (not an actual name) 18th birthday. She was trying to put on a bit darker makeup just to hide her pale and ailing skin. What an irony! The meaning of her name is ‘the healing’ — nevertheless; it has no effect on her at all! Shifa has a disease named thalassaemia.
Recently, we took part in a field work and went to different educational institutions of Dhaka. Our ultimate motto was to spread awareness among young people. We also offered them free blood test so that they could know their carrier status of the disease. We found many young boys and girls even do not know about the disease, let alone their knowledge on how it spreads and the consequences.
Some terms should be understood in order to know the type of the disease. They are alpha thalassaemia, beta thalassaemia, thalassaemia major and thalassaemia minor (also called thalassaemia trait). The main component of the red blood cells (RBC) called ‘Haemoglobin’ has two parts, namely globin chains and heme.
There are two types of globin chain — alpha globin and beta globin. A mutation or deletion of the genes that control globin chain production is the main reason of having thalassaemia. Thalassaemia is called ‘alpha’ when this part of the haemoglobin is not being made. Same thing is also applicable for beta thalassaemia.
On the other hand, to mark the severity of the disease, the word major or minor is used. The same way we inherit our parents’ skin or hair colour, this abnormal globin gene is also inherited. A person, may not have any symptoms at all or may have only mild anaemia, if s/he inherits the abnormal gene from his/her parents; No blood transfusion will be needed and the person will have normal blood iron level, meaning he has only the trait. This is called thalassaemia minor. Then again, a person with thalassemia major may have severe symptoms and may need regular blood transfusions.
It is really important to know whether a person has received abnormal trait from his parents. When two persons (both have abnormal trait) marry, there is a 25% chance of having a baby who is definitely going to be a thalassaemia patient. If an abnormal trait marries a normal trait, the parents will not have the diseased child but may transfer the abnormal trait to their children and increase their risk of having thalassaemia.
There is no actual prevention for thalassaemia apart from highly expensive bone marrow transplantation. Yet, there are some precautions, for example, the unmarried people should do the blood test so that they can know about their carrier status and avoid marrying the carrier of the disease and also consanguineous marriage should be ignored.
This is truly unfortunate that there are many children like Shifa who is becoming the victim of our ignorance. The disease not only affects children, it also creates unbearable problems to the family. To get rid of this intolerable situation, all marriageable people must perform a genetic test before planning for marriage so that in future, their child does not have to be silhouetted against the shiny horizon.
Sadia Tasnim Tuba is a Research Fellow at the Institute for developing Science and Health initiatives (ideSHi) and Md Tarikul Islam is a PhD student at Kanazawa University, Japan.