Ref: CHB/Flash/2007/35                                             Date: March 06, 2007

CHB NEWS FLASH

Thalassaemia (Also known as Cooley’s Anaemia or Mediterranean Anaemia) is a blood disorder.  It is important to understand the structure and function of the blood before Thalassaemia can be understood. (Partly reproduced from MAB’s article on Thalassaemia)  

Blood   

Blood is the red liquid being pumped round by the heart.  It is made up by a yellow liquid called plasma and 3 types of cells - white cells, platelets and red cells.  

The white cells defend the body against infections.  The platelets stops blood loss when the body is cut.  There are many more red cells than white cells. The red cells carry oxygen from the lungs to the body tissues. They are full of red pigment called haemoglobin.   

Anaemia   

If there are too few red cells or if there is too little haemoglobin in them, then the diagnosis of anaemia is made.  It simply means shortage of blood.  If the anaemia is mild it does no harm and may not even be noticeable.  

The commonest from of anaemia is Iron deficiency anaemia which is due to lack of Iron in the diet.  It can be cured by taking Iron medication.   

Thalassaemia   

Thalassaemia is quite different.  It is an inherited disorder and cannot be cured by taking medicines.  The red cells are smaller here so the amount of haemoglobin it carries is less.  It therefore carries less oxygen to the body.   

 How is Thalassaemia Passed on   

Every characteristic of the body is controlled by ‘genes’ which is present in every cell.  There are always 2 kinds of genes - one passed from the mother and the other from the father.  Among many other genes there are 2 genes present to control how haemoglobin is made in the red blood cells.   

‘Normal’ people are normal because they have 2 normal genes for haemoglobin from each parent.  Those who have one normal gene from one parent and one altered or abnormal gene from the other parent are healthy because one gene is working well.  They are said to be healthy carriers of Beta-Thalassaemia Trait. Since gene is inherited from each parent, at least one parent must be a carrier.  People with Beta -Thalassaemia Major have 2 altered genes from haemoglobin, one inherited from each parent, so both their parents must be carriers.
 

Symptoms of Thalassaemia

Some common Symptoms of Thalassaemia are:

·         Fatigue (feeling tired) and weakness

·         Pale skin or jaundice (yellowing of the skin)

·         Protruding abdomen, with enlarged spleen and liver

·         Paleness

·         Headaches

·         Shortness of breath

·         Spleen enlargement

·         Reduced appetite

THALASSAEMIA MAJOR   

In Thalassaemia Major a child is quite ill and anaemic.  Their haemoglobin drops to less than 50% so they look quite pale.  They often have a big spleen.  They need regular blood transfusion - often a unit of blood every month.   

If Thalassaemia Major is not treated, the child becomes weaker and weaker.  It stops growing.  The spleen gets bigger and bigger so the stomach gets big.  The cheek bones and bones of the forehead begin to bulge.  Death occurs if no treatment is given.   

TREATMENT OF THALASSAEMIA MAJOR   

1)   Blood transfusion usually every 4 weeks.

2)   Surgical removal of spleen.

3)   Daily injection of drug called Desferal.   

The ultimate cure for Thalassaemia Major is bone marrow transplant.  It involves a brother or sister donating his or her bone marrow.  The whole procedure is extremely painful, dangerous and expensive to the sum of over £50,000.

The World Federation in collaboration with Africa Federation is looking into ways and means to assist CHB to initiate the Thalassaemia Screening Project for the Jamaats in Africa before end of this year (2007).

“Community’s health – CHB’s priority”