MOST of us have heard the name of the disease Thalassaemia but few know about it. The word Thalassaemia is formed from two Greek words "thalassa" meaning sea and "haima" meaning blood.
This name is given to the disease due to its discovery in the 20th century in countries around the Mediterranean Sea. There are two types of Thalassaemia, Thalassaemia Minor and Thalassaemia Major.
The disease we are talking about here is Thalassaemia Major and when ever you hear or read about the disease Thalassaemia the writer and the talker is referring to Thalassaemia Major, as Thalassaemia Major is the real disease. Whereas Thalassaemia Minor is in fact not a disease, a person who has Thalassaemia Minor is a carrier who can transfer the disease to other persons. We must understand here what a carrier means. A carrier is a person infected with the disease and can pass it on to others but does not itself display any of the symptoms.
Now let us see what Thalassaemia Major is. It is an incurable blood disease, which is present in a child since birth. In this disease blood lacks RBCs (Red Blood Corpuscles) that is, it is a dysfunction of blood to produce Red Blood pigment haemoglobin. This disease results in spleen and liver dysfunction, Red Blood Corpuscles (RBC) and platelets become low in the blood, abundance of White Blood Corpuscles (WBC) in the blood and the defence mechanism of the body becomes very weak.
This disease transmitted from parents to the child and could be fatal. It is common in couples who are cousins in relation. To save your child from this disease as well as yourself from its discomfort and suffering you must have knowledge about it.
A screen test before marriage of both the partners can save future problems a child will face as a result of such marriage. There is always 25 percent chance in every pregnancy that a child with Thalassaemia Major will bear if both parents are Thalassaemia Minor.
The best way to avoid this disease is prevention and to prevent the disease it is necessary that such pregnancy does not occur. A pre-natal diagnosis can prevent the birth of a child suffering from thalassaemia Major. Within 12 weeks of pregnancy a blood test called (Chronic Villous Sampling) CVS test of the baby while in the womb of the mother is done to diagnose the disease.
Altar genes are present in the families but they don't know anything about it, as the disease is only detectable by a Hb electrophoresis test. Or one can only know about the presence of disease when it appears in a person as Thalassaemia Major because then it shows symptoms indicating the presence of the disease.
This disease is more dangerous than cancer, as no cure has so far been discovered for it. In tribal area clan marriages are a norm, which is said to be the basic reason of spreading this disease, especially in those areas.
DIAGNOSIS AND TREATMENT:
Thalassaemia Major is diagnosed when the child is six months to one year of age. Symptoms include pale colour, loss of appetite, cranky forehead and cheekbones become prominent.
Blood transfusion, increased iron discharge and spleen removal operation all are some treatment to provide possible comfort and less pain that a child experiences during this disease. The life long treatment of Thalassaemia is blood transfusion, whereas blood transfusion is actually not a treatment instead it is a relief for the patient from the suffering from the disease as long as possible. The injections and oral drugs are not cost effective as well as not a standard mode of treatment, as there is no curative treatment of the disease has been discovered yet. The best possibility to escape the disease is prevention only.
Every district must have a Thalassaemia centre or blood disorder disease centre and a blood bank to cater to the needs of Thalassaemia Major patients. A child needs blood transfusion twice a month. A patient needs 40 injections of desferol per month.
Thalassaemia Major patients receive Leuko reduced blood as 98 percent blood reactions occur in patients due to White Blood Corpuscles (WBC), which are already in abundance in the blood of the patient. 9 to 10.5 haemoglobin is maintained in children's blood as a normal child haemoglobin is 9 plus.
As it is a genetic disorder of blood other persons who are in contact with the child patient cannot catch it. Like all other normal children such children also need the same kind of love and attention and must be treated as normal human being. The most essential thing for such children is regular blood transfusion. Other than that they lead a normal life.
We must create awareness among people about this fatal disease. Health education for better public awareness of preventive measures for this dreadful disease is badly needed.