Thalassemia & Free Siddha Energy Remedies

Thalassemia Symptoms (थैलेसीमिया के लक्षण)

Iron overload (लोहे का अधिभार)

People with thalassemia can get an overload of iron in their bodies, either from the disease itself or from frequent blood transfusions. Too much iron can result in damage to the heart, liver, and endocrine system, which includes glands that produce hormones that regulate processes throughout the body. Excessive deposits of iron are the cause of damage. Without adequate iron chelation therapy, almost all patients with beta-thalassemia accumulate potentially fatal iron levels.

Infection (संक्रमण)

People with thalassemia have an increased risk of infection, which is especially true if the spleen has been removed.

Bone deformities (अस्थि विकृति)

Thalassemia can make the bone marrow expand, which causes bones to widen. This can result in abnormal bone structure, especially in the face and skull. Bone marrow expansion also makes bones thin and brittle, increasing the risk of broken bones.

Enlarged spleen (बढ़े हुए प्लीहा)

The spleen aids in fighting infection and filters unwanted material, such as old or damaged blood cells. Thalassemia is often accompanied by the destruction of a large number of red blood cells and the task of removing these cells causes the spleen to enlarge. Splenomegaly can make anemia worse, and it can reduce the life of transfused red blood cells. Severe enlargement of the spleen may necessitate its removal.

Slowed growth rates (धीमी विकास दर)

Anemia can cause a child’s growth to slow. Puberty also may be delayed in children with thalassemia.

Heart problems (हृदय की समस्याएं)

Diseases, such as congestive heart failure and abnormal heart rhythms, may be associated with severe thalassemia.

Thalassemia Causes (थैलेसीमिया के कारण)

Both α- and β-thalassemias are often inherited in an autosomal recessive manner. Cases of dominantly inherited α- and β-thalassemias have been reported, the first of which was in an Irish family with two deletions of 4 and 11 bp in exon 3 interrupted by an insertion of 5 bp in the β-globin gene.

For the autosomal recessive forms of the disease, both parents must be carriers for a child to be affected. If both parents carry a hemoglobinopathy trait, the risk is 25% for each pregnancy for an affected child.

Evolution (क्रमागत उन्नति)

Having a single genetic variant for thalassemia may protect against malaria and thus can be an advantage. People diagnosed with heterozygous (carrier) β-thalassemia have some protection against coronary heart disease.

Pathophysiology (पैथोफ्य्सिओलोजी)

Normally, the majority of adult hemoglobin (HbA) is composed of four protein chains, two α and two β globin chains arranged into a heterotetramer. In thalassemia, patients have defects in either the α or β globin chain, causing the production of abnormal red blood cells (In sickle-cell disease, the mutation is specific to β globin).

Alpha-thalassemia (अल्फा-थैलेसीमिया)

The α-thalassemias involve the genes HBA1 and HBA2, inherited in a Mendelian recessive fashion. Two gene loci and so four alleles exist. The excess β chains form unstable tetramers, which have abnormal oxygen dissociation curves. 

Beta-thalassemia (बीटा थैलेसीमिया)

Beta thalassemias are due to mutations in the HBB gene on chromosome 11, also inherited in an autosomal, recessive fashion. The severity of the disease depends on the nature of the mutation and on the presence of mutations in one or both alleles.

Beta thalassemia most often occurs in people of Mediterranean origin. To a lesser extent, Chinese, other Asians, and African American can be affected.

Delta-thalassemia (डेल्टा-थैलेसीमिया)

Just as with beta-thalassemia, mutations that affect the ability of this gene to produce delta chains can occur.

Thalassemia Diagnosis (थैलेसीमिया का निदान)

Thalassemia can be diagnosed via a complete blood count, hemoglobin electrophoresis, and DNA testing.

Thalassemia Prevention (थैलेसीमिया की रोकथाम)

The American College of Obstetricians and Gynecologists recommends all people thinking of becoming pregnant be tested to see if they have thalassemia. Genetic counseling and genetic testing are recommended for families who carry a thalassemia trait.

A screening policy exists in Cyprus to reduce the rate of thalassemia, which, since the program’s implementation in the 1970s, has reduced the number of children born with the disease from one of every 158 births to almost zero. Greece also has a screening program to identify people who are carriers.

Large scale awareness campaigns are being organized in India both by government and non-government organizations in favor of voluntary premarital screening to detect carriers of thalassemia and marriage between both carriers are strongly discouraged.

Thalassemia Management (थैलेसीमिया का प्रबंधन)

People with β-thalassemia trait should be warned that their condition can be misdiagnosed as the more common iron-deficiency anemia.

They should avoid the routine use of iron supplements; iron deficiency can develop, though, during pregnancy or from chronic bleeding. Counseling is indicated in all persons with genetic disorders, especially when the family is at risk of a severe form of the disease that may be prevented.

Thalassemia Anemia (थैलेसीमिया एनीमिया)

People with severe thalassemia require medical treatment. A blood transfusion regimen was the first measure effective in prolonging life.

Iron overload (लोहे का अधिभार)

Multiple blood transfusions can result in iron overload. The iron overload related to thalassemia may be treated by chelation therapy with the medications deferoxamine, deferiprone, or deferasirox. These treatments have resulted in improving life expectancy in those with thalassemia major.

Deferoxamine is only effective via daily injections which makes its long-term use more difficult. It has the benefit of being inexpensive and decent long-term safety. Adverse effects are primary skin reactions around the injection site and hearing loss.

Deferasirox has the benefit of being an oral medication. Common side effects include:

• Nausea
• Vomiting
• Diarrhea

It, however, is not effective in everyone and is probably not suitable in those with significant cardiac issues related to iron overload. The cost is also significant.

Nausea, vomiting, and diarrhea are relatively common with the use of Deferiprone use. It is available in both Europe and the United States. It appears to be the most effective agent when the heart is significantly involved.

Bone marrow transplantation (बोन मैरो प्रत्यारोपण)

Bone Marrow Transplantation (BMT) may offer the possibility of a cure in young people who have an HLA-matched donor. Success rates have been in the 80–90% range. Mortality from the procedure is about 3%. There are no randomized controlled trials which have tested the safety and efficacy of non-identical donor bone marrow transplantation in persons with β- thalassemia who are dependent on blood transfusion.

In a study of 31 people, the thalassemia-free survival rate 70%, rejection 23%, and mortality 7%. The best results are with very young people.

Thalassemia Research (थैलेसीमिया रिसर्च)

Gene therapy (जीन थेरेपी)

Gene therapy is being studied for thalassemia. The procedure involves collecting Hematopoietic Stem Cells (HSCs) from the affected person’s blood. The HSCs then have a beta-globin gene added using a lentiviral vector. This potentially results in a progressive increase in hemoglobin A2 synthesis in all subsequent developing red blood cells, with the resultant resolution of the anemia.

While one person with beta-thalassemia has no longer required blood transfusions following treatment within a research trial, it is not an approved treatment as of 2018.