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Thalassaemia may have originated over 50,000 years ago. In a valley south of Italy and Greece now covered by the Mediterranean Sea. The name Thalassaemia is derived from a Greek word meaning sea. But Thalassaemia was recognized as a clinical entity by Dr. Thamas Cooley and dr. Pearl lee who described five cases of thalassaemia in 1925.
Thalassaemia is an inherited disorder in which there is an abnormality in one or more of the globin genes. To understand more about thalassaemia you need to know a little about blood and anemia. Blood is made up of a lot of red cells in clear, slightly yellow liquid called plasma. Red blood cells are produced constantly. Blood cells are replaced very quickly. That’s why people can often give blood frequently.
The red cells in a person’s blood contain a substance called Hemoglobin. Hemoglobin is very important because it carries oxygen from the lungs to where ever it is needed in the body. It also contains a lot of iron, and when red blood cells are broken down most of the iron from the hemoglobin is used again to make new Hemoglobin. People lose some iron from their bodies when urine is passed; this is compensated for when eating food which contains iron. The main reason why people need iron in their food is to make hemoglobin.
There are two forms of Thalassaemia
Thalassaemia major is sometimes known as Cooley’s anemia, Homozygous, Beta Thalassaemia or Mediterranean Anemia. It is serious inherited childhood anemia. Children with thalassaemia major cannot make enough hemoglobin, because of this, their bone marrow cannot produce enough red blood cells. The red blood cells that are produced are nearly empty.
People with Thalassaemia Minor, sometimes known as Trait, carry Thalassaemia but they are not ill. They are completely healthy and normal but some of them have slight anemia. Most people with Thalassaemia Minor do not even know that they have it. It is only discovered if the person has a special blood test or if they have a child with Thalassaemia Major. It is important to know if you have Thalassaemia Minor later in life. The reason for this is that it may cause some problems if the person and their partner want to start family. Thalassaemia Minor’s red blood cells are also different from normal blood cells.
Number of people who are carriers of Thalassaemia around the world. People who are likely to carry the gene of Thalassaemia are people with Mediterranean decent, for example Cyprus, Egypt, Greece, India, Pakistan, Lebanon, Malta, Middle East, Turkey and some parts of South east Asia.
Thalassaemia major patients can also carry other illnesses such as Sickle Cells, diabetes, Liver dis-function and other illness that non thalassaemia people can get for example cancer.
There is not a known cause for Thalassaemia except that isinherited through the genes.
Children with Thalassaemia major are normal at birth but become anaemic between the ages of three months and eighteen months. They become pale, do not sleep well, do not want to eat, and may vomit their feeds. If children with Thalassaemia major are not treated, they have miserable lives. They usually die between one and eight years of age.
A chronic illness always causes some limitation of quality of life, especially when it requires frequent and complex treatment, as Thalassaemia dose. The treatment should not interfere with a Thalssaemic’s life. In particular doctors and hospitals should make the effort to arrange out-patient visits and visits for transfusions so they interfere as little as possible with normal life. Treatment should not interrupt schooling or work.
These days most Thalassaemic’s grow up to become adults, and earn their own living. Most also find a partner and get married. Now a number of thalassaemia major patients have their own children. It is very hard to know the answer for Thalassaemics who are well at present. The disorder and its influence are changing almost from day to day, because of advances in treatment. Thalassaemic patients are now living longer. Today it is reasonable to think that people with thalssaemia major, who have been well treated from the beginning, may well live as long as people without Thalassaemia. Only time will tell. Even though thalassaemics live with more risks than non Thalassaemic, because of the amount of medication and treatment they receive.
But all medical treatments include some risk.
People with thalassaemia major can have babies only if their partner does not carry any sort of Thalassaemia. But all thalassaemia major’s patients children will carry thalassaemia minor. If a Thalassaemia major partner does not carry any thalassaemia gene none of the children would have thalassaemia major.
For a woman with Thalassaemia to have children they must have normal sexual development. Many young women with Thalassaemia are not having their periods, or whose periods have started and then stopped. In this case they can be treated medically so that they produce eggs. If they are not physically fit, a pregnancy could risky for them and the baby. An expected mother should be fit, meaning they must use their pump regularly, her serum ferritin level should be around 1000 and her heart and liver should not have been damaged by iron overload. However, even if they are not perfectly fit, there is a chance that they could have a fairly normal pregnancy. Women are advised to stop Desferal when they are trying to become pregnant, or as soon as they are pregnant. There is no evidence that desferal can harm the foetus, but in general it is a good idea for any pregnant woman to not take drugs during pregnancy. Mothers who breast feed can start taking desferal again as soon as the baby is born. Desferal does not pass from the mother’s body into milk and so cannot harm the baby. Before a woman with Thalassaemia decides to have a baby they must take account about the long term future, their own health and survival or whether they will have support from their families. The chance of having a baby with Thalassaemia minor decreases if their partners have Thalassaemia minor.
The only treatment for Thalassaemia major is regular blood transfusions, usually every three or four weeks. Most children have these transfusions, usually every three or four weeks grow normally and live quite happily into their early twenties. but to live longer, they need other treatment as well.
After each blood transfusion the red cells in the new blood are broken down slowly over the next four months. The iron from the red blood cells stays in the body. If it is not removed. It builds up and can damage the liver, the heart and other parts of the body. If this damage is not prevented most people with Thalassaemia major die when they are twenty years old.
At present the only way to remove the extra iron from the body is to give injections of a drug called Desferal(correct name is Deferrioxamine) this medication starts between the ages of 4 to 8 years old. Desferal is injected under the skin. The injections are given using a portable battery operated pump. This slowly empties in a 5/10ml syringe over 10/12.5 hours. The pump is used 5-7 nights of every week; therefore treatment takes place in home. Usually the parents are responsible for this until the child is able to take over. Desferal pick up the iron and carries it out in the urine.
Thalassaemia major patients should try to keep away from high in iron foods as red meat, liver, kidney, greenleafy vegetables such as spinach, some breakfast cereals, wholemeal breads and alcohol. Although this is recommend, patients do not have to stick with this diet.
This treatment is very successful and most children treated with blood transfusions and desferal can now lead fairly normal healthy lives. But the treatment is unpleasant and often upsetting, it also interferes with their desire for an active social life sometimes medication is neglected.