Thalessemia - Killing Childrens Find Out Way To Survive Article

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Thalessemia - Killing Childrens Find Out Way to Survive

Thallessimia is a genetic blood disease caused by a defect in the rate of production of hemoglobin. The condition is further classified into thallessimia minor and thallessimia major (Cooley's anemia, beta thallessimia).

Author: Homeherb

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Thalessemia refers to a group of inherited anemias (red blood cell deficiency). It is a disease of hemoglobin, which is the protein which delivers oxygen throughout our bodies. It is estimated that 100,000 babies a year are born with severe forms, about 10,000 in India alone. Geographically the Thalessemia belt includes the Mediterranean passing through West and Central Asian countries like Turkey, Iran, Afghanistan onto Pakistan & India and passes on to the South East Asian countries like Indonesia, Burma & Thailand, Vietnam and Cambodia. This makes it most common in African, Greek, Italian, Middle Eastern and Southern Asian populations.

The two main groups of Thalessemia are called alpha and beta. There are four types of alpha Thalessemia and three types of beta Thalessemia.

Alpha Thalessemia genes are found in Southeast Asians, blacks, and people originally from the Middle East. The disease affects the red blood cells in two ways. First the body cannot make enough cells with normal hemoglobin. Second, the cells have their lifespan shortened from four months to less than one month.

The first type of alpha Thalessemia minor (trait) is a carrier state with no anemia and no symptoms. The second type has slightly abnormal red cells but still no anemia. The third type of alpha Thalessemias produces a mild anemia that generally doesnt lead to serious complications. The red blood cells are slightly abnormal and resemble iron deficiency anemia. These children would most likely have not been diagnosed and may present for adoption.

The fourth type of alpha Thalessemia major is the most severe and affects mostly Southeast Asians, Chinese and Filipinos. It results in death before or soon after birth. This last type is usually seen only in Southeast Asians.

Beta Thalessemias cause a broad range of health symptoms which range from none to very severe. Beta Thalessemia genes are more widespread - found in Africa, the Middle East, India, Southeast Asia, and around the Mediterranean. Someone with only one of the beta genes will have beta Thalessemia trait, a mild anemia in which the red cells are smaller than normal. Because small red cells are also typical of iron deficiency anemia, which is extremely common in women and children, many people with this trait are erroneously diagnosed as iron deficient, and are given iron pills. Iron not only doesnt help this mild anemia, but it can build up in the tissues. The three categories of beta Thalessemia are major, intermedia and minor.

Beta Thalessemia minor (Thalessemia trait) may cause no symptoms, but can be identified by changes in the blood. Children with Thalessemia minor (Thalessemia trait) are considered carriers and lead completely normal, healthy lives. Beta Thalessemia intermedia is a mild form of Cooleys anemia.

Children with Thalessemia intermedia may develop similar complications, but usually the disease is mild until adulthood. These children usually do not require transfusions, although they may be recommended if complications start to develop. However, countries such as Vietnam which permit the adoption of very young infants may not have identified these children prior to adoption.

Cooleys anemia or Thalessemia major is the most severe type. Infants with Thalessemia major appear healthy at birth, but develop symptoms around 8 to 10 months of age. These children appear pale, weak, fussy, and have poor appetites. Their growth is slow and they often become jaundiced.
Left untreated, the spleen, liver, and heart soon begin to fail. Bones become thin and brittle, and facial bones become misshapen. Untreated children experience retarded physical and mental growth. In untreated children, heart failure and infection are the leading causes of death.

Treatment with frequent blood transfusions and antibiotics can alter the course of the illness. Children with Thalessemia major can be treated by hypertransfusion. Transfusions every 3 or 4 weeks help keep their hemoglobin near normal. This helps prevent some of the complications of the disease, resulting in better growth and general health and can prevent heart failure and bone deformities in some cases.
Unfortunately, the treatments have their own drawbacks. Repeated blood transfusions lead to a buildup of iron in the body, damaging the heart, liver and other organs. This requires daily drug treatment with a chelator to eliminate iron, preventing or delaying problems related to iron overload. The chelator drug is requires daily dosing given by pumping the drug underneath the skin while the child is sleeping.

Children treated aggressively with frequent blood transfusions and iron chelation live 20 to 30 years or longer.

Bone marrow transplants (BMT) have cured some cases of Thalessemia. This high risk treatment is possible only for a small minority of patients as a suitable bone marrow donor must be located. The transplant procedure is still risky and can result in death. The success rate of BMT is as high as 95%, if there is no prior serious organ damage such as excess deposition of iron. About one-third of all BMT patients will have a siblings whose bone marrow is a good match. Adopted children lacking an identifiable common gene pool of their birth family would be less likely to find a suitable donor than a child with an intact and identifiable birth family.

Other treatments on the horizon include oral chelating drugs and various types of gene therapy, including stem cell transplants.

All Thalessemias are inherited. People unfamiliar with the disease may mistakenly think it can be caught from another child. Blood tests can show whether an adopted child has Thalessemia or is a carrier. Identification of mild forms of the disease is important as well meaning physicians may prescribe vitamins or food supplements containing additional iron. While this is appropriate for iron deficiency anemia, it can cause iron overload in children with any form of Thalessemia. There is now a simple test for iron in the body called the ferritin level. If someone seems to be iron deficient, but the ferritin level is normal, they probably have the first type of beta Thalessemia. Ask your physician to test your childs ferritin level if they are anemic before prescribing vitamins.

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Thalessemia - Killing Childrens Find Out Way to Survive