Welcome to the Sickle CellWhat is Thalassaemia Major?
Thalassaemia Major is an inherited blood disorder, which leads to severely diminished production of haemoglobin. The disorder requires lifelong blood transfusions and continuous treatment for the removal of extra iron in the body which is known as 'Iron Chelation'.
An individual with thalassaemia major is unable to produce enough mature haemoglobin which is essential for transporting oxygen around the body. Without sufficient oxygen the body will not survive. Individuals become very anaemic, especially if they do not receive blood transfusions. The frequency of the transfusions varies with each person, and could be every four to six weeks for their entire life.
Desferal is the name of the drug used to remove excess iron from the body. This drug has to be injected under the skin at night and the frequency of this varies for each person. It can be for three, four, five or, seven nights each week, from a minimum of six hours to a maximum of twelve hours. In very rare cases the individual may have to infuse the medication for twenty-four hours. Vitamin C is also given to help the Desferal remove the iron from the body.
Blood transfusions have helped increase the life expectancy of individuals with beta thalassaemia even though there are risks involved.