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SECTION 3
Thalassemia Intermedia

 
 
Somewhere between 3 and 10 out of 100 people who inherit b-thalassemia from both parents, have a milder form of thalassemia called Thalassemia Intermedia. Since this can be rather confusing for everyone, patients and doctors alike. AS there is very little written about it. either for patients or for doctors, we decided to spend some time on it here.
         "Thalassemia Intermedia" simple means thalassemia of a milder kind, in which the patient can if necessary survive without regular transufsions. This includes a very wide range of people. A few are completely healthy, most rather "delicate." while a few are chronic invalids. In the same person, the disease is fairly constant. and should not be change from time to time.
         Thalassemia intermedia is usually due to inheriting one of the common severe thalassemia genes and one milder thalassemia gene. (There is quite a large number of mild b-thalassemia genes. but they are all rare, except Hb E, which is described in Chapter 5.
 

 
How do you know if you have thalassemia intermedia, and not thalassemia major?
         Children with thalassemia intermedia start to develop problems a bit later in life than those with thalassemia major. Most are reasonably well untill after 2 years of age, and some with mild thalassemia intermedia may not have the diagnosis made untill they are about 7 years old, or sometimes even older. Very occasionalyy, some with a very mild thalassemia intermedia finds out only in adult life, for example when they become pregnant, or have a routine medical examination for some other reason.
         There is not really a very clear distinction between thalassemia major and thalassemia intermedia. There are many borderline patients, who may have many of the problems mentioned below, but still manage just to survive without transfusions. Naturally enough, in countries where it is hard to find blood and Deferal, most borderline patients are not transfused, while in mediterranean and european countries, most finally start on regular transfusions.
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