A FORMULATION FOR IRON CHELATION IN THALASSEMIA PATIENTS
 

Thalassemia is a dreaded disease among children because of genetic disorder. The disease is caused due to hereditary disorders connected with defective haemoglobin synthesis, characterised by hypochromia, microcytosis, haemolysis and a variable degree of anaemia. Thalassemia involves a heterogeneous group of molecular defects and present with a wide spectrum of clinical expressions.

Patients suffering from Thalassemia suffer anaemias with decreased or absence of synthesis of a globulin chain of a normal haemoglobin. The patients of thalassemia are broadly classified into two major groups according to the affected globulin chain. The patients suffering from Alpha (a) Thalassemia are associated with decreased or absence of a-chain synthesis. The patients suffering from Beta (b) Thalassemia are associated with decreased or absence of b-chain synthesis.

The disease Thalassemia occurs worldwide with a particular high incidence in the Mediterranean basin and in the South-East Asia. Malaria is also endemic in these areas- a significant fact since indirect evidence suggests that - Thalassemia (major) heterozygosity confers protection against malaria.

The b-Thalassemia or Thalassemia major type of the disease comprises a heterogeneous group of disorder usually characterised by absence of (bo) or decreased (b+) globulin synthesis. The type of Thalassemia is also classified according to the severity of the anaemia. These clinical classification serves to differentiate homozygous (Thalassemia intermedia or Thalassemia major) from heterozygous state (Thalassemia minima or Thalassemia minor). Though it does not reflect genetic mutations, Thalassemia (minor) is a reduced rate of b-globulin synthesis, with an increased a-b globulin chains, but it not threat to a normal life.

 

 

 

 

 

 

 

 

In the case of Thalassemia (major), iron in the haemoglobin breaks down and gets deposited in the vital organs of the body of the patients e.g. in liver, kidney, spleen etc and make them rigid. This is also known as iron overloading in the body and the life span of the child suffering from Thalassemia (major) becomes unpredictable.

Under the prevailing present day conditions, regular blood transfusion and use of the drug 'Desferal' by injection as explained above, is the best way of treatment of this dreaded disease. Since a decade, efforts are being made by medical professions throughout the world to find a treatment of this disease by a drug, which should be low cost and can be administered orally without side effect.

Keeping in mind the above objective a formulation has been developed which acts as follows:

i) The iron present in the body forms a complex with the formulation (b-diskette type complex) in the presence of a catalyst which reduces consumption of the formulation and helps control fever and the body ache problem associated with such patients.

ii) The complex so produced are soluble in body fluids and the fluids containing these complexes reaches the kidney and it is then excreted from the body of patients through urine and thus reduces pathological iron overload in the organs.

Salient Features

1. The existing drugs are injected daily under the skin of the patients in the controlled manner to avoid side reaction causing allergic conditions. The existing treatment is very painful. While this newly developed formulation is taken orally so consumes less time and no pain at all.

2. The cost is negligible in comparison to existing drug.

3. No side effect of the new formulation.

 

 

 

 

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