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Treatment of Thalassaemia
Treatment for Beta thalassaemia major involves you having regular blood transfusions, which take place every six to eight weeks, depending on the severity of the anaemia. Excess iron builds up in your body from these regular transfusions. This is called iron overload. If this is left untreated, iron will build up in your body, leading to a condition called haemosiderosis. This can cause serious long-term damage such as heart failure and liver failure.
Iron overload is kept under control by treatment with a medicine called desferrioxamine, to achieve a more normal level of iron in the body. Desferrioxamine works by binding (the chemical term is “chelating”) with the iron in your blood and the chelated iron is then removed by the kidneys (excretion) from the body. Desferrioxamine has to be given by injection, usually by a slow injection under the skin via a small device or pump over eight to twelve hours. You may be taught how to do this yourself at home. The amount of desferrioxamine and how often it is given depends on the how much iron you have in your body i.e. the amount of iron overload.
As you will be receiving this medicine regularly (often daily), it can become tiresome for you or your carer. So, it is important to understand why desferrioxamine doses should not be missed. Sticking to desferrioxamine treatment routines helps protect against serious complications in later life, such as diabetes and heart disease. There are also more immediate benefits such as prevention of nausea and sickness caused by iron overload.
Deferipone is another medicine used to treat iron overload. It works in a similar way to desferrioxamine but is given by mouth. This medicine is only given to patients in whom desferrioxamine is not suitable or is not tolerated.
Like all medicines, both desferrioxamine and deferipone may have side effects. You should refer to the manufacturer’s patient information leaflet for further information as well as talk to your doctor or pharmacist if you have any particular concerns. If you are receiving either of these medicines you need to be closely monitored by having regular blood tests and eye and ear examinations. Checks on body weight and height every three months may be carried out in children because their growth can be affected by the condition and, in some cases, by the medicines.
Beta thalassaemia intermedia treatment may be the same as for Beta major but as the anaemia is less severe, the need for transfusions or the frequency of tranfusions and need for iron overload treatment will be different according to the severity of the anaemia.
Supplementary treatment
The time taken for iron to be removed from your body by desferrioxamine is improved by taking a daily dose of ascorbic acid (vitamin C). This is prescribed by your doctor according to your age and is usually started one month after starting desferrioxamine treatment. Ascorbic acid prescribed for this purpose should be taken separately from food because it also increases the absorption of iron in food, which you must avoid.
If you have heart problems, you will not be given ascorbic acid because its combined effect with the iron in the blood may make the heart problem worse. Any other multivitamin and mineral supplements should not be taken unless prescribed by a doctor because they can increase or decrease absorption of iron and other minerals.
Other Treatments
Venesection - therapeutic removal of blood from the body - to help deal with excess iron build -up
Surgery – removal of the spleen (splenectomy) is sometimes required if the spleen is damaged because it has to remove a larger number of abnormal red cells from the circulation.
Management and reduction of iron in the diet - usually only a small amount of iron is absorbed from the diet, however the absorption is increased when haemoglobin in the blood is low, as in beta Thalassaemia major. This is especially true between transfusions. Therefore iron in the diet should be low (red meat is especially high in iron). You should be referred to a dietician for advice on diet.
Bone marrow transplantation – this can provide a cure for Beta thalassaemia major. Bone marrow is transplanted from a matched unaffected sibling or unrelated donor. It is best done when the child is very young. However, the procedure is painful and, although success rates are improving, they are unpredictable.
There are many emotional, psychological and social effects for the person with thalassaemia and their family, particularly as self- management is so important. Psychological support is important in managing chelation therapy and other aspects of the condition. You may find getting involved with local support groups and voluntary organisations helpful.
