Blood disorder II - Thalassemia

 

Iron is combined with a protein in our body to form a substance called hemoglobin in our blood cells.

Iron deficient causes anemic condition whereas thalassemia has to do with protein. With lesser amount of oxygen delivered and the high rate of destruction, the red blood cells become smaller and lower in counts, rendering the patient tired easily.

 

Causes

 

There are 2 proteins present in the hemoglobin - Alpha globin and beta globin. For thalassemia to occur, there is a defect in a gene that helps control production of one of these proteins.

• Alpha thalassemia occurs when a gene (s) related to the alpha globin protein are missing or changed (mutated), occurring mostly in persons from Southeast Asia, the Middle East, China and Africa. The most severe form of alpha thalassemia major causes stillbirth.
• Beta thalassemia occurs when similar gene defects affect production of the beta-globin protein, occurring mostly in persons of Mediterranean origin. The reason is that parts of those countries were once full of malaria and this gene afforded protection against malaria, and thus continue to thrive.

Both alpha and beta thalassemia include the following two broad categories:

A 25% chance of having a child without any (with both genes for normal beta chains).

• Thalassemia minor - inherits one copy of the beta thalassemia (defective) gene from a parent together with one perfectly normal beta-chain gene from another. Probability is 50%. The person:

        -  Remains a carrier and usually do not show symptoms.
        -  Suffers only mild anemia with slight lowering of the blood hemoglobin level but usually normal blood iron level. No treatment is necessary for thalassemia minor and can lead normal healthy life.

• Thalassemia major - inherits two genes for beta thalassemia (defective) and no normal beta-chain gene. Probability is 25%.

        -  The foetus/newborn seems entirely normal because of a special form of hemoglobin present at birth. Anemia begins to develop within the first months after birth when this hemoglobin is replaced by defective hemoglobin. As it becomes progressively more and more severe, the infant failed to grow normally and often eat poorly due to easy fatigue from lack of oxygen and shortness of breath.
        -  Symptoms include pale skin, irritability, growth retardation, swelling of the abdomen due to enlargement of the liver and spleen, bouts of fever and heart failure due to infections, jaundice, diarrhea and other intestinal problems. Most full-blown cases occur within the age group 5-8 and can be fatal.
        -  Severe anemia with rupture of the red blood cells is a critical condition as it gives no choice to the child to become dependent on blood transfusions every 3-4 weeks that can create further problems including iron overload, resulting in diabetes, liver and heart failure if not cleared timely.     
        -  Premature death due to heart failure are reported, usually between ages 20 and 30.
        -  Such patients should not take iron supplements, which can worsen the condition. Also, chelation therapy may need to arrange to remove the excess iron from the body for those who receive significant numbers of blood transfusions. 

        -  Folic acid supplementation is often given to relieve the symptoms of the illness.
        -  A bone marrow transplant may help treat the disease in some patients, especially children. Alternative treatment is by insertion of the normal beta-chain gene through gene therapy.

In 1995, Dr Patrick Tan performed a world’s first stem cell transplant in Singapore on a 5 year old boy suffering from Thalassemia Major from an unrelated donor. After the successful procedure, the boy was completely freed from dependence of drugs and blood transfusions. He was cured and leads a normal healthy life.

Tests

Conventional clinical and blood testing, and molecular medical tests can detect both thalassemia major and minor. These tests permit accurate diagnosis to be made at any time, even before birth and formation of beta chains for hemoglobin.     

VIVA Corner

+ VIVA Dailyguard provides our body cells with 27 vitamins and minerals for its normal functioning, all extracts from fruits and vegetables. As our red blood cells have a lifespan of only 120 days, putting all the essential nutrients can help to improve the quality of new cells regenerated. Its high antioxidant contents protect the cellular membrane against the onslaught of free radicals internally (eg. stress) and externally (eg. pollutants).

+ VIVA B-easy contains natural B complex concentrated from yeast helps in healthy blood formation and thus prevent anemia. Taking adequate B12, B6 and folic acid assist in protein uptake and thus accelerate cell maturation, boosting the number of healthy red blood cells in your body. This is crucial as most thalassemia patients have red blood cells that survive only 90 days instead of 120 days.

VIVA Ageguard is a proprietary formulation of amino acids in highly absorbable form derived from natural foods like soy and rice protein. Specially prepared as unchained free form amino acids, they serve as primary building blocks for healthy tissues and rebuild healthy cells.