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SECTION 3
Thalassemia Intermedia
Chapter I
The General Picture
 
 
Can people with thalassemia intermedia lead a normal life?
         We can give a very general answer, YES. People with Thalassemia Intermedia should be able to lead a normal life.


What happenes to people with thalassemia intermedia?
         The very big differences between individual patients make it hard to describe thalassemia intermedia as clearly as we can describe thalassemia major. Here we give only a very general description. which may not exactly fit your case.


How severe is the anemia?
         Children with thalassemia intermedia usually become pale and ill only after their second year of life. They are moderately anemia, i.e. their hemoglobin level is usually between 8 and 9 g/dl. However, in some special cases it may be as low as 7 g/dl, and in a few others, as high as 10 or 11 g/dl. In any case, you cannot really judge how severe your thalassemia is from your hemoglobin level alone. What really counts is how you feel in yourself, how strong you are, how well you grow, and whether you have any other problems.
 

 
Do people with thalassemia intermedia ever need blood transfusions?
         Most people with thalassemia Intermedia have occasional times when their anemia gets rather suddenly worse. This is usually a result of an infection, though sometimes it is hard to see a cause, if this makes you feel ill and stops you leading a normal life. Your doctor will suggest that you should have a "top-up" transfusion, or even a course of transfusion. It is sensible to accept a transfusion when you need it. to give yout bone-marrow a short rest, and help both you and it to recover. Having a few top-up transfusions will not make your thalassemia worse, and will not make you go on needing transfusions in the future.


What happens to the spleen?
         In thalassemia intermedia, the spleen usually enlarges only rather slowly, and hypersplenism develops late, if at all. But finally, many people with thalassemia intermedia need to have their spleen taken out.


Are there any bone changes in thalassemia intermedia?
         Most People with thalassemia intermedia have some changes in their bones, and their face. The changes resembling those in children with thalassemia major who are not adequately transfused. But in thalassemia intermedia, the changes are usually quite mild, and do not spoil your looks.


Is there any problem with growth and puberty?
         Children with thalassemia intermedia usually grow nearly normally in height, though they are often a bit thin. they go through normal puberty, though often two or three years later than their "normal" friends. Adults with thalassemia intermedia can usually have children quite normally, though women may need blood transfusions during pregnancy.


Can People with thalassemia Intermedia have children?
         Yes, they can. Usually a woman will need regular transfusions during pregnancy, not so much for herself, but to help the baby to grow normally. Desferal treatment should be stopped during Pregnancy, and probably so should other drugs. But pregnant women should take "Folic Acid" specially regularly.
 

 
Why do people with thalassemia intermedia sometimes need desferal treatment?
         People with thalassemia intermedia quite often have too much iron in their body, even if they are never transfused. This is because they usually more iron from their food than the rest of us do. It is part of the body's attempt to cope with the anemia, it thinks that because you are anemic. You must be short of iron. So iron usually does build up slowly in your body, but it takes a long time (usually more than 10 years) for it to reach an undesirable level.
         To be honest, we are not certain what an undesirable level really is. To be absolutely safe, we usually say you should have some Desferal if your serum ferritin is more than 1000 ng/ml. When it does reach this level, it can usually be brought down again by a relatively short course of Desferal. About 6 weeks of subcutaneous infusions from a pump, or a course of intramuscular injections, twice a year (or an infusion once or twice a week) is usually adequate. it is important for you to have you serum ferritin measured at least once a year, to keep an eye on the iron build-up in your body, and to decide when to control it.

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