Answer: In this marriage for every pregnancy, there will be a 25% chance of conceiving a normal baby, 25% of alpha-thalassemia minor, 25% chance of beta- thalassemia minor 25% chance of having a baby with alpha-beta thalassemia which will clinically behave as thalassemia Intermedia (more severe than thalassemia minor but less severe than thalassemia major).
Question 2: Can thalassemia major patients also carry other diseases?
Answer: Having thalassaemia major does not confer immunity against other diseases. These children can contract all those illness which non-thalassemia children may acquire. In fact these children are some what more susceptible to other disease because of their poor nutrition, repeated blood transfusions and generally poor socio-economic conditions.
Increased insceptibility to bacterial infection occurs in all forms of severe thalassaemia, possibly because the relatively high serum iron levels may favor bacterial growth, or because of blockage of the mononuclear phagocyte system due to increased rate of destruction of red cells.
Question 3: What is Blood Cancer?
Answer: These are a group of disorders in which one or the other type of blood cells start to multiply and grow and their behavior becomes uncontrolled and ….. This causes excessive increase in the number of these cells which…… causes the death of the patients. The word ‘blood cancer’ usually refers to an over production and un controlled proliferation of the white cells. A better known and universally accepted name for this disease is leukemia.
Question 4: Is there a complete cure of thalassemia?
Answer: The only curative treatment of thalassemia major is bone marrow transplantation. Where as minor can also be cured by this procedure, it is not done in this condition. It is not necessary to treat patients with thalassemia minor because it is a harmless condition which does not effect the and the functions of these individuals. For such a mid disease bone marrow transplantation which is a serious and costly adventure is NEVER warranted.
Question 5: How does thalassaemia affect the quality of life: can patients with thalassaemia major have children?
Answer: In thalassemia major the blood which is produced is less in amount and poor in quality. These children are severely anemic; they must receive regular blood transfusion. This exposes them to a number of diseases including hepatitis, AIDS and iron overload. Their abnormal facial features, dark complexion, stunted growth, distended abdomen etc. make them handicapped in more than one ways. These children are sexually underdeveloped and many of them die in early childhood or during adolescence. Those who are “perfectly” managed, as in some of the developed countries, reach adulthood and they get married. Children of patients with thalassemia major will all have thalassemia minor provided that the spouse does not have the thalassemia gene. If two “well” treated persons with thalassemia major get married, all of their children will have thalassemia major.
Increased iron absorption causes accumulation of iron, first in the Kupffer cells of the liver and in the RE cells of the spleen and later in the parenchymal cells of the liver.
Iron accumulates not only in liver but also endocrine glands, particularly the parathyroid, adrenals, and pancreas, and most important, in the myocardium. Iron accumulation in the Myocardium leads to death, either by involving the conduction tissue or by intractable cardiac failure.
Other consequences of cardiac failure include diabetes, hyperparathyroidism, and hypogonadism, mainly due to end organ failure.
Question 6: Is there any thing we can do to finish the existing carriers?
Answer: It is not possible to eliminate the carriers of -thalassemia gene. At present there are nearly 8 million (80 laces) carriers of -thalassemia living in Pakistan.
Genetic engineering and gene manipulation some day may provide an outlet, but at present it is a situation which must be accepted as reality of life.
Question 7: What are the symptoms beta-thalassemia major?
Answer: thalassemia is a disease of the red cells, they are less in number and poor in quality. These patients are therefore severely anemic. To correct anemia they need life long support through blood transfusion. The large amount of iron which accumulates in their body effects may glands is secondary to iron deposition, it is not due to the disease.
Question 8: How can thalassemia be categorized?
Answer: thalassemia can be categorized in many ways; it can be that thalassemia is
- A genetic disorders
- A blood disorder
- A disorder of hemoglobin synthesis
Question 9: What is the level of hemoglobin in a normal person?
Answer: Average amount of hemoglobin in a person varies according to the age and sex of the individual. This is shown in a tabulated from below.
Question 10: What is the difference between thalassemia and AIDS?
Answer: thalassemia is a genetic disorder caused by an abnormality of the genes which are responsible for the formation of globin chains in the hemoglobin molecule. The child is born with thalassemia and live with for the rest of his life, it is neither an infectious disease nor it is contagious.
AIDS on the other hand, is an acquired infectious disease caused by a virus called Human Immunodeficiency virus (HIV). It is a contagious disease which spreads through blood and through sexual contact. As there is no treatment of this disease, it ultimately leads to the death of the patient.
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