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SECTION 2
Thalassemia Major
Chapter 8
Problems about Puberty
 
 
Can thalassemics develop normally at puberty?
         Many thalassemics do not develop normally at puberty. This is because an excessive body iron load has damaged either the "endocrine" glands that control Sexual Maturation, or else the sex glands themselves (i.e. The testes in boys, or ovaries in girls). This happens most often in patients who did not have the best treatment in the early days of their lives, because it was not possible at that time. However we now know that thalassemics who have been well treated all their lives can grow normally at Puberty, and have normal Sexual development.


But doesn't failure of puberty contradict what you said about thalassemics being able to lead a normal life?
         Yes, Ofcourse, but we need to take two things into account
         Only some thalassemics have abnormal development at puberty. There are thalassemics who grow quite normally, and have normal sexual development. The better the early treatment has been, the better the chance of having a normal puberty.
         Thalassemics who do not go through a normal puberty can be helped by medical treatment. To be honest, the result is not quite as good as "the real thing". As a rule, the better the treatment was in early life, the better the results of treatment to promote growth and development.


Are there any married thalassemics who have children?
         In the past, there was very little hope that thalassemics could have children - Unless they had Thalassemia Intermedia. Today, there are several thalassemics in the world with their own children, and we are working on ways of helping the rest to have children, as well as a normal sex-life.
 

 
Is there any treatment, if puberty does not come normally?
         Yes, there is treatment to promote sexual development, and also to improve growth. This is rather a complicated subject, but we know that alot of people are really interested in it, so we will do out best to explain.
         Sexual development starts from a part of your brain, when it recognises that you are big enough and old enough to mature. In fact, this part of your brain works without you knowing anything about it all. (Fig 16 shows the series of events)
         First, your brain produces a hormone called LHRH (or GnRH)5 this is carried in your blood to pituitary gland (in the base of your skull just about between your ears).
         Then your pituitary gland produces two harmones, called LH6 and FSH7. That are carried aroung in your blood and stimulate your sex glands to grow. (these are the testes in boys and ovaries in girls.)
         Then, as they grow, the sex glands produce hormones that are carried around in your blood and affect the rest of your body, to give male or female development. Examples are testoterone in boys, and oestrogens in girls.
         Once the whole sequence is running, it normally takes a few years for all the sex changes to be complete.


So What goes wrong with puberty in people with iron overload?
         Well, actually almost anything can go wrong. It seems that iron can interfere with any of the steps - but it does not interfere with all of them in one person. So if you have had problems with puberty, it couuld be your brain that never sent the first message to your pitiuitary gland, or your pituitary gland neven sent that message to your sex glands, or even your sex glands that were not able to respond to the message when they received it. You can see that you may need a complicated set of investigations to pin down the precise problem in your case. It is important to find the problem, so as to choose the best treatment for you.

5These initials stand for "Luteinising Hormone Releasing Hormone" or
                                     "Gonadotrophin Releasing Hormone"
6Luteinising Hormone.
7Follicle Stimulating Hormone.




What is the treatment for failure of puberty then?
         It still needs quite alot of research. You see, we know that most thalassemis would like to be able to have children, as well as to be married and have a normal sex-life.
         The easiest treatment for failure of Puberty is to give the sex-hormones, testosterone or oestrogen. These work very well to make people develop, and usually to grow as well. But they may not help them to have children.
         On the other hand, if you can be given one of the hormones further back in the chain, like GnRH (LHRH) for instance, it may bring on a more natural sort of development, including the ability to have children. We are not yet quite sure about how successfull this treatment is. We do know it works for some people and not for others, depending on where exactly things have gone wrong. Specialists are working on this problem, with the thalassemics.
         In The long run, it is best to be careful and go slowly, so as to be sure to get the best possible result for everybody.


If you have gone through a normal puberty, can you have problems afterwards?
         Yes, we're afraid you can, because of iron builds up in your body, it can still damage your pituitary or sex glands. Then, in women the periods can stop, and in men, there may be sexual impotence. Therefore it is extremely important to keep up Desferal treatment regularly all your adult life.


What else do we need to know about complications?
         You should realise that the possibility of Complications means that you should have regular medical tests. Then, if a complication happens, it is necessary to follow the treatment to deal with it.
         Surprisingly enough iron is not stored in a lot of places, it is not stored in the skin, the muscles, the brain, the bone, the intestines, or the kindeys. So providing the problems for the heart and endocrine glands are controlled, there should not be too many other problems to worry about.
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