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SECTION 3
Thalassemia Intermedia
Chapter II
Particular Problems in Thalassemia Intermedia
 
 
A wide range of problems can arise in thalassemia intermedia, but they can all be dealt with. When these problems are severe, they may make it necessary to start on regular transfusion, even relatively late in life.
         Mose of the problems that can occur in thalassemia intermedia, happen because the thalassemia is not being controlled by regular blood transfusions. It can affect almost every part of the body. People with thalassemia major would have the same problems in a worse form, if they were not transfused. In fact, some older people with thalassemia major who were kept on low transfusion scheme in the past, when we did not know better, will remember having had some of these problems themselves.
 
The Spleen

         Sometimes the spleen gets gradually bigger and the anemia gets gradually worse as the years pass. Finally some patients with thalassemia intermedia start to need transfusions. When that happens, removing the spleen often brings the situation back to what it was earlier in life. The anemia gets better, so some people can stop transfusions again after splenectomy.
         Sometimes a mild thalassemia intermedia causes very little trouble in the first few years of life, so that no-one notices that there in anything wrong with the child. But then the spleen may get bigger and make the thalassemia worse, and the diagnosis is made rather late. By this time the thalassemia intermedia may look like thalassemia major, because the spleen is making it worse, and the child may be started on regular transfusions. This is why we alays wonder whether it is thalassemia major or thalassemia intermdeia, when a child with a big spleen starts to need transfusions for the first time, after 4 years of age.
 

 
         Ocassionaly, someone who really have thalassemia intermedia with a big spleen, who has been on regular transfusions for years, has their spleen taken out. After the operation, they may find they can manage without transfusions. This does not happen often. You may have heared stories of people who were "cured" by having their spleen taken out. This is the explanation of such cases. Splenectomy on its own does not improve thalassemia. It is helpful only if the spleen is making the thalassemia worse that it would be otherwise.


How do you know if your spleen is causing trouble and needs to come out?
         The best way to follow what the spleen is doing in thalassemia intermedia, is to keep a regular record of your hemoglobin level and growth over the years.
         In thalassemia Intermedia, your hemoglobin level falls gradually below the normal in the first 3 to 5 years of life. After that, in most cases, it remains more or less constant.
         At this point it is important to explain exactly what a hemoglobin level means. Very many things can affect the measurement. Such as the temperature of the room when the sample is taken, details of the method of taking the sample, and whether it is comes from a finger prick or from vien. These variations alone can make a difference of as much as a gram in the result. Also, your real hemoglobin level usually falls about a gram. Therefore, when you talk about your real hemoglobin level, it must be the average of several measurements taken some weeks apart. This is why we say people with thalassemia intermedia must have their hemoglobin measured at least 4 times a year. Then, if you keep a careful record of the measurements, you can see if your average hemoglobin level is changing from one year to the next.
         The commonest cause for a steady fall in your hemoglobin level over the years, is gradually-developing hypersplenism. However, we do not recommend removing the spleen simple because your hemoglobin level is a bit lower than it used to be. It is only worth having the operation if you fele unwell, or there are other problems. For Instance. an over-active spleen can slow down you growth-rate. This is one reason why it is important to keep a growth-chart.
         See "Questions about Splenectomy", for problems associated with the operation.

 
The Bones

         Some people with thalassemia intermedia can have severe bone problems. This is because their bone marrow tries to work too hard to overcome the anemia, so that it expands inside the bones, and can weaken or even deform them. Some bones can be too fragile, and get fairly easy broken in the course of normal life. The growth of some bones can be disturbed, so that for instance, your upper arm may be shorter that other people's. Sometimes one leg bone may be short than the other, so that you develop a limp. This can usually be corrected by surgery after you have finished growing. Sometimes changes in the bones of the face can be severe, and some adults with thalassemia intermedia can get unpleasant, nagging pains in their bones and joints. If you have real problems of this type with your bones, you have a more severe form of thalassemia intermedia, verging on thalassemia Major. Most bone problems can be controlled or cured by starting on regular transfusions.

 
The Bone Marrow, and Folic Acid

         In thalassemia intermedia the bone marrow has to work very hard to make enough red blood cells to go round. One of the things it needs to make red cells is a vitamin called Folic Acid. This vitamin is present in meat and in green vegetables. Usually people get as much folic acid as they need from their diet, but this is not always true for people with thalassemia intermedia, since they need considerably more than usual. This is why your doctor advises you to take a folic acid tablet every day.


Are any particular people likely to get short of folic acid?
         Yes. People who have a strict vegetaranian diet can get short of folic acid. For instance, in England, some groupd of people of Indian origin both have a high frequency of thalassemia, and a strict vegeteranian diet. People with thalassemia intermedia on this type of diet can get very ill without additional Folic Acid, and may be thought to have a thalassemia major. when they start on extra folic acid, they get better very quickly.


Can expansion of the bone-marrow cause any other problems?
         Very ocassionaly, the bone marrow expands altogether too much, and makes the bones too thin and fragile, so that they break very easily. Sometimes it may even get into places where it should not be, and can interfere with the smooth working of other organs, for instance by putting pressure on nervers. Such important problems may indicate that, in this case, the thalassemia intermedia is too severe, and the patient should start on regular blood transfusions.
 
The Heart

         In thalassemia intermedia, your heart has to work extra hard. Because it helps to compensate for your anemia by pumping your blood round faster than usual. This does not, as a rule, do it any harm: the heart is rather tough organ! But it does mean that when you have chest X-Ray your heart looks a little larger than usual. In itself, this is normal for you, and should not worry you.


But can't you have problems with your heart in thalassemia intermedia?
         Yes, of course you can, but only under special circumstances. Firstly, if your thalassemia intermedia really is too severe. or if you get hypersplenic, or short of folic acid, you become more anemic than you should be. When anemia is really severe, it does put too much strain on your heart. Then it really is not able to cope. and you can have problems. These things do not happen suddenly: the main thing is that you start to feel unwell and short of breath. If you really have heart problems, they can usually be "cured" by starting an regular blood transfusions.

 
The Kidneys

         Thalassemia can sometimes affect your kidneys, because as your bone marrow is so overactive, they make an unusual amount of wastes to be excreted in your urine. (The most important of these is uric acid.) As a rule this does not do your kidneys any harm, but they are often a bit larget than normal.
         There may be an increased level of uric acid in your blood, and there certainly is in your urine. If the level is very high, the excess of uric acid can damage the kidneys. so it may be necessary to take a drug (Allopurinol) that prevents your body making so much uric acid.


Why do people with thalassemia intermedia often have to get up at night to pass water?
         Quite Often, people with thalassemia intermedia cannot make suc concentrated urine as other people do. This means that you may lose more water than other people, and so may need more to drink. So many people with thalassemia intermedia need to get up once (or even twice) at night to pass water, and to have a drink. This does no harm, but it is important always to have enough to dring, especially if you are ill and have a temprature.

 
Body Heat

Why do some people with thalassemia intermedia sweat alot, and suffer from the heat in summer or at night?
         This is because of the hard work of your bone-marrow does all the time, to make enough good red blood cells for you. For every good red blood cell, it has to make many that are no good, and die in the bone-marrow without getting out into your blood. When they are broke down in the bone-marrow, each cell releases some heat, so altogether the body of a person with thalassemia intermedia generates more heat than other people. People with thalassemia intermedia generates more heat than other people. People with thalassemia intermedia have their own "central heating" that they can't switch off at night or in summer. So it is more comfortable for them not to have too many bed-clothes in night, and to keep cool in warm weather.

 
The Liver

         Your liver may be a bit big, so that the doctor can feel it, but that is harmless and a normal part of thalassemia intermedia.


Is it true that people with thalassemia intermedia are particularly likely to develop gall-stones?
         Yes, it is quite common for older people with thalassemia intermedia to get gall-stones. The stones are made of a material called bile-pigments, that comes from your red-cells that are made and broken down faster than normal. It collects in your gall-bladder, that is attached to your liver.
 

 
What other problems can occur in thalassemia intermedia?
         Ankle Ulcers are a frequent and troublesome problem. They often arise just above the inside or outside of your ankle. They often start from a bruise or a bang, that takes a long time to heal. Once an ulcer has started, it can take a very long time to go, or simple refuse to go away at all. These ulcers are important, because they can interfere with your ability to work.
         Ankle ulcers tend to occur in older patients ( Usually After Puberty ), especially if they are tall. They are caused partly by the fact that the blood supply to the skin over your ankles is rather poor. Your anemia also contributes, because when blood does flow to this part of the skin, it may not be able to give it enough oxygen. And finally, when you stand or sit, thhe circulation in your legs is slower than when you are lying down, because gravity keeps too much of the blood in your legs. There may be other reasons as well, why these ulcers happen.


What can you do to avoid ankle ulcers?
         There are several things you can do, to prevent ulcers from starting. but you only need to think about this after you have passed through Puberty, and if the skin on your ankles looks unusually thin and shine, or has brown marks on it.
         Firstly, try to make sure that you do not bang your ankles. Most people bang their ankles accidently from time to time. even with the heel of the opposite shoe. Try wearing a sock with a turned-down top (e.g. a towelling sock.) to protect your shins, or one of those towelling bands that tennis stars use on their wrists.
         Secondly, make sure that the circulation is as free as possible in your ankles, for at least several hours a day. To do this you need to raise your feet above the level of your heart. The easiest way to do this is to try sleeping with your feet slightly raised, by putting a brick under the foot of your bed. It is even easier to raise your feet in a comfortable chair or on a sofa when you are resting, e.g. Watching television. You should try to arrange to sit like this for about 2 hours every day, If possible.
         Thirdly some people who have had ankle ulcers say that they can be prevented by taking Zinc Sulphate tablets daily, by mouth. This has not been proved scientifically, but it is worth a try!
         If you do actually get an ankle ulcer, discuss the best way to treat it with your own doctor.


Is it really better to have thalassemia intermedia than thalassemia major?
         Usually it is better, because you remain well enough to lead a reasonably normal life, without having regular blood transfusions, and daily tedious treatment with the pump.
         However, there are some real disadvantages to having a thalassemia intermedia. For instance, many people with thalassemia intermedia never feel completely well. In addition, you may hear a lot of things about thalassemia, but most of what people say, applies only to thalassemia major. Unless you understand that thalassemia intermedia really is different, and why, you may always feel uncertain about your health, and worried about your future, So sometimes people with thalassemia intermedia, and their families, worry more than families of people with thalassemia major.
         One of the most worrying times is when the doctors think a small child, of one or two years old may have thalassemia intermedia and not thalassemia major. Only time will give the answer. As time passes. If the child's hemoglobin level stays high, you get more and more hopeful that your child will have only a mild disease. But at the same time you get more and more anxious in case he or she finally turns out to have thalassemia major. We do understand that this can be a very trying time for parents.
         Even after you know that you definitely have thalassemia intermedia, you may feelk anxious in case something happens to make your thalassemia worse. We hope our explanation has reassured you that if it does get worse, we can usually find out why, and can correct the problem. It is rare for any sudden change to happen, so you always have time to notice it and bring it to the attention of your doctor.
         It is true that some people with severe thalassemia intermedia can develop quite serious problems, and may have to change over to having regular transfusions. But really, you should not be afraid of this. On the contrary, it is reassuring to know that if you do to have a problem that cannot be solved in any other way. you can start on regular transfusions. But really, you should not be afraid of problem that cannot be solved in any other way, you can start on regular transfusions. This should make you feel secure, and ready to try any form of treatment that your doctor propses - because you know. If it does not work, you always have transfusion to fall back on.
         And, though regular transfusions and daily Desferal are a bore. There is the advantage that you should feel fitter than ever before in your life, and you enjoy your food more!
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