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Thalassemia Major
Chapter 7
Other Problems in Thalassemia
What are the most frequent problems?
         Problems affecting the heart, the liver and the endocrine glands. Here, we talk about the heart, the live, and SOME endocrine problems.

Are Heart Problems very common
         They were very common in the past, but are much less common today, thanks to Desferal.

What is the explanation for heart problems?
         When a lot of iron gets stored in the heart, it may become much larger, and sometimes it beats irregularly. Finally, if iron goes on being stored there itstops being able to pump the blood around fast enough. This is why, in the past manu patients died in their twenties. People said they had died of "Iron OverLoad". One of the main advantages of Desferal is that is protects your heart from iron. It prevents it getting in, and takes it out again, it it has already got in. This is why heart problems are much less common today then they used to be.
         If a serious heart problem does develop, it can be treated by intensive Desferal treatment. This is done either by increasing the amount of Desferal you take subcutaneously, or by using a "Portacath" This is small reservoir attach to some tubing. The reservoir is implanted under the patients skin at a convenient place (by a surgeon under a general anesthetic). The tubing goes into one of the big viens near the Heart. The whole system is inside the body. A high dose of Desferal can be infused into the reservoir, and so straight into a vein, using the pump in the usual way but without any discomfort from the high dose. This treatment can prevent damage to the heart.

What is the cause of liver problems?
         They are caused by iron overload, and virus infections tranmitted in the blood.

Can liver complications be prevented
         The best preventive measures are the correct use of Desferal, and testing of blood donors to see if they are carriers of hepatitis virus

What about endocrinlogical complications?
         These are still quite common, but we hope they will become less frequent, especiallyas the children grow up who have been on Desferal from the very early years of their lives.

What are the commonest endocrinological complications?
         Slowing of growth and development at puberty, diabetes, hypothyrodism, hypoparathyroidism, and in adults, failure of sexual function.

Can endocrinological problems be prevented?
         Desferal treatment done well is the best preventive measure.

What is the cause of endocrinological complications?
         They are caused by the iron overload. Iron can get into some of the "endocrine glands" that control many aspecs of your body's functioning.
         For instance, some iron may get into the thyroid gland in the neck, that controls how generally active you are. It usually causes no trouble here. Probably your doctor does a blood test from time to time, to check that your thyroid gland is all right is all right. If it is not active enough, you can easily put it right by taking a small amount of thyroxine every day.
         Iron can also get into the parathyroid glands. These are four tiny little glands attached to the thyroid gland, and they control the amount of Calcium in your blood. In some other thalassemics, iron can disturb these glands, and this can make your blood calcium level fall. This can cause tingling and prickling feeling in your arms and legs. and sometimes cramps as well. It can easily be put right by taking a little extra Vitamin D (Do not under any circumstances take extra Vitamin D on your own, without discussing it with your doctor. If you dont need it, it can be harmful).
         Iron can also be stored in the Pancreas. Which is the biggest gland near your stomach that helps to digest your food. Iron very rarely causes any trouble here, However it can damage the parts of the pancreas that produce insulin (they are called the "islets of Langerhans"!). If this happens you may get diabetes.
         Of Course diabetes can be treated, but the treatment is not easy, particularly if you also hava thalassemia, One reason we encourage people to take their Desferal regularly from an early age is because it reduces their chance of getting diabetes.
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