Thalassaemia :
   Thalassaemia is one of the commonmost inherited disorder in the world. Amongst the Indian population the carriers range between 3-15%. It is estimated that there are 30 million gene carriers in India. This condition is more prevalent in certain communities like Sindhis, Punjabis, Gujratis, Benngalis, Bhanushalis, Lohanas, Muslims. Thalassaemia is mainly of two types. Thalassaemia Major and

Thalassaemia Minor : Thalassaemia minor is a heterozygous condition in which the person carries the gene of Thalassaemia and can pass it to the next generation. This is also known as Thalassaemia trait or carrier. Persons with Thalassaemia minor are otherwise normal except that during stress situations like pregnancy their haemoglobin may drop. Illustrative Diagram of How the Disorder is transmitted Genetically

Thalassaemia major : is a serious disease in which the affected child is unable to maintain normal hemoglobin level. If left untreated it limits the life span to 3-5 years. The present day treatment available for thalassaemia major is repeated blood transfusions and iron chelation therapy. Overall the treatment of thalassaemia major is very taxing, traumatic and expensive and is beyond the reach of most.

I-Iow to prevent Thalassaemia'?
   Thalassaemia is preventable but not curable. All married women should be screened either before pregnancy or during early pregnancy for Thalassaemia status. * If she is found to be Thalassaemia minor then the husband should also be screened. If both are Thalassaemia minor, antenatal diagnosis is advised between 9 and 11 weeks of pregnancy. In case the fetus is found to be affected by Thalassaemia major, MTP is advised and if the fetus is Thalassaemia minor or normal the pregnancy is allowed to continue.

Definition:
    Thalassaemia intermedia is a clinical state in which thalassaemic Children survive without blood transfusion, maintaining their hemoglobin between 6-9 g/dl. However, the hemoglobin level drops under stress and the children occasionally require blood. The disease is milder than thalassaemia.

Genetic Aspects :
    Genetically in thalassaemia intermedia, the beta chain synthesis is less impaired than in thalassaemia major. The imbalance between the.... And beta chains is less pronounced. The genetic defects in thalassaemia intermedia are variable. There is overlap between thalassaemia intermedia, thalassemia major and thalassaemia minor. This could be the result of reduction in a chain synthesis along with a more severe reduction in beta chain synthesis.

Sign and Symptoms :
    There is no clear cut distinction between thalassaemia major and thalassaemia intermedia. Thalassaemia intermedia presents with a wide spectrum of clinical features. In mild cases, growth and development of the child may be relatively normal, while the severe cases may present with a clinical picture similar to transfusion dependent beta thalassemia major. Pallor and splenomegaly are prominent, as in thalassemia major. Growth and development often get affected if the anaemia persists for a long time and in the absence of blood transfusions. Many borderline patients with severe anaemia manage to survive without transfusions. Most of them present late in life (four to seven years or even later) and maintain their hemoglobin between 6-9 g/dl. They grow normally in height but are under weight. Puberty may be delayed by 2-3 years. Woman may need regular transfusions during pregnancy. The hemoglobin drops during stress such as pubertal growth, pregnancy, associated vitamin deficiency (B' Z and folic acid). In India, iron deficiency may become evident as it is widely prevalent. The decision to start regular transfusion in thalassemia intermedia is much more difficult than in thalassemia major. Patient who can lead a normal life and do not have other problems, should not be started on regular transfusions. Occasional transfusions are given when the haemoglobin falls below 7 g/dl, especially in presence of infections. All patients need regular monitoring for early detection of complications.

New Hope for 'Thalassaemia Majors

The miracle of birth and the lifeline between mother and child could be a lifesaver for those unfortunate children suffering from thalassaemia Major The blood collected from this lifeline has the capacity to continue as a lifesaver Donate your cord blood A lifeline before birth and beyond....

What is Cord Blood?
    Years of medical research have led us to an amazing discovery, cord blood. Cord blood is what remains in the umbilical cord and placenta following birth, which is normally discarded. Collection of cord blood is pain-free, simple and non-invasive. First used in transplant in 1988, umbilical cord is a rich source of stem cells, the building blocks of blood and immune cells, which can be used to treat a variety of life-threatening diseases. In just a few years, hundreds of acutely ill patients have received treatment because of this medical advancement.

How is cord blood donated?
    First, a pregnant mother gives her consent to donate her baby's umbilical cord blood to the Reliance Life Sciences cord blood donor program. After the birth of the child, blood is drawn from the portion of the umbilical cord, which is still attached to the placenta. The blood is collected in a specially designed bag provided by Reliance. As in all other donor programs, your confidentiality is assured.

Is there any harm to either mother or child as a result of these procedures?
    No. Since the baby is delivered before any blood is collected, there is no chance of your baby getting an infection from the collection process. This blood is derived. From the placenta and hence the newborn is not deprived of any blood.

    Donating your baby's cord blood will provide New Hope for children with thalassaemia, cancer and other diseases.

    The stem cells from a single placenta are sufficient to rebuild the blood and immune system of a child with thalassaemia, whose own red blood cells are incapable of carrying oxygen.

    Recognizing the advantages of cord blood, Reliance Life Science has been established providing Relicord: stem cell enriched umbilical cord blood for transplantation, storage facilities for Sibling Donor programs and mobilizing peripheral blood cells programs for aggressive chemo and radiotherapy.

What is blood"?
    Blood is a part of your body. It is pumped around by your heart, and circulates in the blood vessels that distribute it through your whole body. The blood vessels are arteries, capillaries and veins. When your heart pumps blood out, it flows first into the big arteries, and then into smaller arteries to reach the capillaries. The capillaries are so small that you can only see them through a microscope, but they are very important because while the blood is flowing through them, it gives out the air and food it is carrying to the tissues of the body, and picks up wastes to take them away. After this the blood flows into the veins, which finally carry it, back to your heart.

What is blood made of ?
    Blood is made up of a light yellow liquid, called plasma, and of three types of "cells". In fact, your whole body is made up of tiny building blocks called cells, far too small to see. In most tissues they are stuck together, but in the blood they float round freely in the plasma. There are 3 types of blood cells: red cells, white cells and platelets.

What does blood do?
    Each part of the blood has its own special function. The plasma carries water, salts and materials such as food, hormones and drugs to your tissues, and carries away waste, to be got rid of through your lungs (in your breath) and through your kidneys, in your urine. Your white blood cells defend your body against infections. Your platelets stop you from losing blood if you hurt yourself. They stick together and block up your blood vessels when they get damaged, so they stop any more blood escaping. You have many more red cells than white blood cells. The red blood cells are full of hemoglobin, which is red, and this is what makes your blood look red. Hemoglobin picks up oxygen from the air in your lungs, and carries it round to your tissues, where it lets it go. To live, your tissues need to breath, so they need oxygen. New red blood cells are being made all the time in your bone marrow. They only live about 120 days. Then are destroyed in your spleen.

What is "anemia?
    If you have too few red blood cells, or there is too little hemoglobin in them, you have "anemia." This simply means a shortage of blood. If the anemia is mild, it does not harm and you may not even notice it: but if it is severe, you are ill, because your tissues don't have enough oxygen.

    The commonest form of anemia is "iron deficiency anemia." This can happen if you don't have enough iron in your diet. It can be cured by taking iron medicine. Thalassaemia is quite different from iron deficiency anemia. It is an inherited anemia. It cannot be cured by taking any medicines.

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