Thalassemia

Thalassemia is the name of a group of genetic blood disorders. Haemoglobin is the oxygen-carrying component of red blood cells. It consists of two different proteins, an alpha and a beta. If the body do not produce enough of either of these two proteins, the red blood cells do not form properly and cannot carry sufficient oxygen. The result is anaemia that begins in early childhood and lasts throughout life.

Alpha Thalassemia : People whose haemoglobin does not produce enough alpha protein have alpha thalassemia. Most individuals with alpha thalassemia have milder forms of disease with varying degrees of anemia.

Beta Thalassemia : People whose hemoglobin does not produce enough beta protein have beta thalassemia

The Beta thalassemias range from very severe to having no effect on health.

•  Thalassemia major, the most severe form, is also called Cooley’s anemia, named after the doctor who first described it in 1925.
•  Thalassemia intermedia is a mild Cooley’s anemia.
•  Thalassemia minor (also called thalassemia trait) may cause no symptoms, but changes in the blood do occur.

How Does Thalassemia Affect a Child ?

Most children with thalassemia major appear healthy at birth, but during the first year or two of life, they become pale, listless and fussy, and have a poor appetite. They grow slowly and often develop jaundice (yellowing of the skin).

Without treatment, the spleen, liver and heart soon become greatly enlarged. Bones become thin and brittle; face bones become distorted, and children with thalassemia often look alike. Heart failure and infection are the leading causes of death among children with untreated thalassemia major.

Children with thalassemia intermedia develop some of the same complications, although in most cases, course of disease is mild for first two decades of life. Use of frequent blood transfusions and antibiotics has improved the outlook for children with thalassemia major. Children with thalassemia intermedia usually do not require transfusions, although they may be recommended if complications start to develop.

When children with thalassemia major are treated with frequent transfusions (generally every 3 to 4 weeks) aimed at keeping their haemoglobin level near normal, many of the complications of thalassemia can be prevented. This form of treatment, referred to as “hypertransfusion,” enhances child’s growth and well-being, and usually prevents heart failure and bone deformities.

Unfortunately, repeated blood transfusions lead to a buildup of iron in the body, which can damage the heart, liver and other organs. A drug referred to as an iron chelator, an iron binding agent, can help rid body of excess iron, preventing or delaying problems related to iron overload.

Thalassemia is cured using bone marrow transplants. This form of treatment is possible only for a small minority of patients who have a suitable bone marrow donor, and the transplant procedure is still risky and can result in death.

Role of Noni

Autoxidation of globin chains and iron overload are the suggested mechanisms for the increased oxidative stress in â-thalassemia.To find out the extend of lipid peroxidation and antioxidant status in thalassemia, one clinical stud has been done in the thalassemia and healthy blood sample. The aim of this study was to evaluate the extend of lipid peroxidation and antioxidant status of patients with â-thalassemia and iron deficiency anemia (IDA) and compare the results with healthy subjects. Oxidant and antioxidant status of the children with â-thalassemia major and iron deficiency anaemia were studied. Healthy controls were age and sex matched. Conjugated diene (CD) and thiobarbituric acid-reactive (TBARS) substances were analyzed to indicate oxidative parameters, whereas the erythrocyte superoxide dismutase (SOD) and glutathione peroxidase (GPx) were measured to show antioxidant status of children. Plasma TBARS and CD concentrations were elevated in â-thalassemia compared to IDA. In the iron-deficiency group both TBARS and CD levels were decreased compared to the controls. SOD and GPx activities increased in the â-thalassemia group. SOD in â-thalassemia was higher than both IDA and the controls and GPx activity was higher than the IDA group. In vivo lipid peroxidation was increased in children with â-thalassemia major. This leads to a compensatory increase in antioxidant enzymes, whereas IDA does not lead to lipid peroxidation with a normal antioxidant enzyme activity.

The pathophysiology of thalassemia is, to a certain extent, associated with the generation of labile iron in pathological red blood cell (RBC). Appearance of such forms of iron at the inner and outer cell surfaces exposes cell to conditions whereby the labile metal promotes formation of reactive oxygen species (ROS) leading to cumulative cell damage. Nutritional therapies may be considered as adjuvants to drug therapy. Using nutritional therapy to augment a patient’s antioxidant defenses to decrease morbidity and mortality has similarly been advocated in other more dreaded diseases like cancer

Noni is a powerful effective natural nutritional supplement. It contains all the vitamins, lot of trace minerals, 17 amino acids including all essential amino acids, and besides it contains more than 150 phytochemicals. All those ingredients prsent in Noni just make it a super nutritional supplement. It contains all the antioxidant vitamins and trace elements. It contains maximum number of flavonoids, having eficient antioxidant property. Noni also synthesizes more antioxidant enzymes like super oxide dismutase and glutathione peroxidase from the RBC. It not only helps to neutralise the damaging effect of free radicals like lipid peroxidation but also helps to elevate the level of antioxidant enzyme of our body.

Thalassemia paitents who are using Noni regularly are showing improvement in their clinical symptomatic level and their blood transfusion gap is going to increase. Hence it is advisable for all the thalassemia patients to use Noni with all the normal treatment to enahnce their antioxidant level and to enhance their immunity power. 

Recommended Dosage

Divine Noni Concentrate

5ml morning and 5ml evening for 3 days. Then

10ml morning and 10ml evening for next 3 days. Then

15ml morning and 15ml evening for next 8 months.

Thalassemia is the name of a group of genetic blood disorders. Haemoglobin is the oxygen-carrying component of red blood cells. It consists of two different proteins, an alpha and a beta. If the body do not produce enough of either of these two proteins, the red blood cells do not form properly and cannot carry sufficient oxygen. The result is anaemia that begins in early childhood and lasts throughout life.

Alpha Thalassemia : People whose haemoglobin does not produce enough alpha protein have alpha thalassemia. Most individuals with alpha thalassemia have milder forms of disease with varying degrees of anemia.

Beta Thalassemia : People whose hemoglobin does not produce enough beta protein have beta thalassemia

The Beta thalassemias range from very severe to having no effect on health.

•  Thalassemia major, the most severe form, is also called Cooley’s anemia, named after the doctor who first described it in 1925.
•  Thalassemia intermedia is a mild Cooley’s anemia.
•  Thalassemia minor (also called thalassemia trait) may cause no symptoms, but changes in the blood do occur.

How Does Thalassemia Affect a Child ?

Most children with thalassemia major appear healthy at birth, but during the first year or two of life, they become pale, listless and fussy, and have a poor appetite. They grow slowly and often develop jaundice (yellowing of the skin).

Without treatment, the spleen, liver and heart soon become greatly enlarged. Bones become thin and brittle; face bones become distorted, and children with thalassemia often look alike. Heart failure and infection are the leading causes of death among children with untreated thalassemia major.

Children with thalassemia intermedia develop some of the same complications, although in most cases, course of disease is mild for first two decades of life. Use of frequent blood transfusions and antibiotics has improved the outlook for children with thalassemia major. Children with thalassemia intermedia usually do not require transfusions, although they may be recommended if complications start to develop.

When children with thalassemia major are treated with frequent transfusions (generally every 3 to 4 weeks) aimed at keeping their haemoglobin level near normal, many of the complications of thalassemia can be prevented. This form of treatment, referred to as “hypertransfusion,” enhances child’s growth and well-being, and usually prevents heart failure and bone deformities.

Unfortunately, repeated blood transfusions lead to a buildup of iron in the body, which can damage the heart, liver and other organs. A drug referred to as an iron chelator, an iron binding agent, can help rid body of excess iron, preventing or delaying problems related to iron overload.

Thalassemia is cured using bone marrow transplants. This form of treatment is possible only for a small minority of patients who have a suitable bone marrow donor, and the transplant procedure is still risky and can result in death.

Role of Noni

Autoxidation of globin chains and iron overload are the suggested mechanisms for the increased oxidative stress in â-thalassemia.To find out the extend of lipid peroxidation and antioxidant status in thalassemia, one clinical stud has been done in the thalassemia and healthy blood sample. The aim of this study was to evaluate the extend of lipid peroxidation and antioxidant status of patients with â-thalassemia and iron deficiency anemia (IDA) and compare the results with healthy subjects. Oxidant and antioxidant status of the children with â-thalassemia major and iron deficiency anaemia were studied. Healthy controls were age and sex matched. Conjugated diene (CD) and thiobarbituric acid-reactive (TBARS) substances were analyzed to indicate oxidative parameters, whereas the erythrocyte superoxide dismutase (SOD) and glutathione peroxidase (GPx) were measured to show antioxidant status of children. Plasma TBARS and CD concentrations were elevated in â-thalassemia compared to IDA. In the iron-deficiency group both TBARS and CD levels were decreased compared to the controls. SOD and GPx activities increased in the â-thalassemia group. SOD in â-thalassemia was higher than both IDA and the controls and GPx activity was higher than the IDA group. In vivo lipid peroxidation was increased in children with â-thalassemia major. This leads to a compensatory increase in antioxidant enzymes, whereas IDA does not lead to lipid peroxidation with a normal antioxidant enzyme activity.

The pathophysiology of thalassemia is, to a certain extent, associated with the generation of labile iron in pathological red blood cell (RBC). Appearance of such forms of iron at the inner and outer cell surfaces exposes cell to conditions whereby the labile metal promotes formation of reactive oxygen species (ROS) leading to cumulative cell damage. Nutritional therapies may be considered as adjuvants to drug therapy. Using nutritional therapy to augment a patient’s antioxidant defenses to decrease morbidity and mortality has similarly been advocated in other more dreaded diseases like cancer

Noni is a powerful effective natural nutritional supplement. It contains all the vitamins, lot of trace minerals, 17 amino acids including all essential amino acids, and besides it contains more than 150 phytochemicals. All those ingredients prsent in Noni just make it a super nutritional supplement. It contains all the antioxidant vitamins and trace elements. It contains maximum number of flavonoids, having eficient antioxidant property. Noni also synthesizes more antioxidant enzymes like super oxide dismutase and glutathione peroxidase from the RBC. It not only helps to neutralise the damaging effect of free radicals like lipid peroxidation but also helps to elevate the level of antioxidant enzyme of our body.

Thalassemia paitents who are using Noni regularly are showing improvement in their clinical symptomatic level and their blood transfusion gap is going to increase. Hence it is advisable for all the thalassemia patients to use Noni with all the normal treatment to enahnce their antioxidant level and to enhance their immunity power. 

Recommended Dosage

Divine Noni Concentrate

5ml morning and 5ml evening for 3 days. Then

10ml morning and 10ml evening for next 3 days. Then

15ml morning and 15ml evening for next 8 months.