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Thalassemia

Indu Thalessemia Centre was started in the year 1997 with enrollment of 10 patients which has now increased to about approximate 50 patients a month. Blood is supplied as per the requirement from single unit to 3-4 units a month to each patients. The center is sometimes favoured for financial support from Donors for which they adopt a child for a year and bear total expenditure or either give funds to the Institution and thereby free blood is issued to the child according to their suggestion.

Thalessemia being a disease where blood is required to be transfused at a regular interval, data of the voluntary donor are maintained who all come when ever asked for. A group of donors also adopt a child so they come turn by turn throughout the year and fulfill the patient’s blood requirement. The Blood Bank washed RBC’s when required.

Thalassemia is a group of inherited blood disorders that affect the body's ability to produce hemoglobin and red blood cells - patients have a lower-than-normal number of red blood cells in their bodies and too little hemoglobin. In many cases the red blood cells are too small. The bone marrow of people with Thalassemia does not produce enough healthy hemoglobin or red blood cells, which causes anemia and fatigue, because the body is short of oxygen. In more severe Thalassemia cases, the patient's organs may be damaged, there is restricted growth, heart failure, liver damage, and even death.

Alpha Thalassemia
The alpha thalassemia patient's hemoglobin does not produce enough alpha protein. This type is commonly found in southern China, Southeast Asia, India, the Middle East, and Africa.
To make alpha globin protein chains we need four genes, two on each chromosome 16. We get two from each parent. If at least one of these genes is missing, it produces alpha thalassemia. The severity of thalassemia depends on how many genes are faulty.

One faulty (mutated) gene - there are either no symptoms at all, or they are very mild. A person who is apparently "healthy" and has a child with symptoms of thalassemia is known as a Silent Carrier. This type is also known as alpha thalassemia minima, or 2 trait.

Two mutated genes - the patient will have mild anemia. Also known as alpha thalassemia minor, or 1 trait.

Three mutated genes - the patient will have hemoglobin H disease, i.e. chronic anemia. A person with hemoglobin H disease needs regular blood transfusions throughout his/her life.

Beta Thalassemia We need two globin genes to make beta globin chains. We get one from each parent. If one or two of these genes are faulty, it produces beta thalassemia.

Severity of beta thalassemia also depends on how many genes are mutated:
If one globin gene is mutated - the patient may have Beta thalassemia minor.
If both globin genes are mutated - the patient may have either moderate or severe symptoms (Colley's anemia).patiebt may have Beta thalassemia major or intermedia
Beta thalassemia is much more common among people of Mediterranean ancestry, hence its other name, Mediterranean anemia. It is also more prevalent in North Africa and West Asia. Sixteen percent of the people in the Maldives, some islands in the Indian Ocean, are carriers.

The treatment options for thalassemia:

Blood transfusions
this is done to replenish hemoglobin and red blood cell levels. Patients with moderate to severe thalassemia will have repeat transfusions every 4 months, while those with more severe disease may require transfusions every two to four weeks. Patients with mild symptoms may require occasional transfusions when they are ill or have an infection.

Iron chelation
involves removing excess iron from the bloodstream. Sometimes blood transfusions can cause iron overload. Iron overload is bad for the heart and some other organs. Patients may be prescribed subcutaneous (injected under the skin) deferoxamine or oral (taken by mouth) deferasirox.
Folic acid supplements may be prescribed for patients who are administered blood transfusions and chelation.

Bone marrow transplant - also called a stem cell transplant. Bone marrow is a spongy tissue that exists in the hollow centers of flat bones. Bone marrow cells produce red and white blood cells, hemoglobin and platelets.
Bone marrow transplant (from a compatible donor) is the most effective treatment. However, HLA matched sibling is the best allogenic stem cell donor for “the thalassemia major” patient.
This treatment was first performed on thalassemia patient in 1981.
Current evidence suggests >90% patients with Thalassemia major survive stem cell transplantation and >80% patient with thalassemia major become free from disease following stem cell transfusion.