There is much to be done to help alleviate the effects and extent of thalassemia in India. Organizations like Thalassemics India have done pioneering work in offering services for the treatment and wellbeing of thalassemia affected children.

Regional Centres for Blood Donation

For Blood Transfusion

Preeti Tuli Thalassemia Unit at Sir Ganga Ram Hospital Poorvi Marg, New Rajinder Nagar, New Delhi.

Incharge :  Dr. V.K. Khanna
Phone : 42251028

"THALASSEMIA UNIT" at St. Stephen's Hospital,
Tis Hazari, Delhi-54.

Incharge :  Dr. Nirmal
Phone : 23983580; Extn : 452

Other thalassemia centers are at AIIMS,
NDMC Charak Palika, LNJP, DDU & GTB,
Apollo Hospitals of Delhi.

For Prenatal Diagnosis & Screening

Prof. I.C. Verma
Senior Consultant & Head,
Dept. of Medical Genetics, Sir Ganga Ram Hospital,
Poorvi marg, New Rajender Nagar,
New Delhi. Phone : 25762672

Dr. Madhulika Kabra
Genetics Unit, All India Institute of Medical Sciences,
Ansari Nagar, New Delhi - 110029
Phone : 26594585 (Extn.- 3264)

Other centers are at Calcutta, Mumbai, Vellore, Hyderabad, Lucknow & Pune.

For HLA Matching

Dr. N.K. Mehra, Dept. Anatomy
Room No. 75, AIIMS, Ansari Nagar,
New Delhi - 110029
Phone : 26588700 / 500

Dr. Mammen Chandy, CMC Hospital, Vellore-632004.
Phone : 91-0416-2222102 / 2223603 ext. 2169
Fax : 91-0416-2232035 / 2232054
E-mail : mammen@cmcvellore.ac.in

For Bone Marrow Transplantation

Dr. Mammen Chandy, Prof. & Head of Hematology,
Bone Marrow Transplantation Centre,
Christian Medical College Hospital, Vellore-632004.
Phone : 91-0416-2222102 / 2223603 ext. 2169
Fax : 91-0416-2232035 / 2232054
E-mail : mammen@cmcvellore.ac.in

Stem Cell Transplantation
Stem Cells, present in the bone marrow, give rise to red blood cells that produce Hemoglobin. In Thalassemia, it's the defect in the stem cells that leads to deformed red blood cells that produce the abnormal hemoglobin and transfusion - dependent anemia. Stem Cell transplantation provides a thalassemia patient with healthy stem cells that will produce normal blood cells.

Stem Cells can be transplanted into a patient's body through different methods, such as :

Bone Marrow Transplantation (Bmt)

In BMT, chemotherapy is used to kill the patient's stem cells in the bone marrow. They are then replaced with healthy stem cells from a compatible donor. Bone marrow transplants are generally most successful when the donor and host are genetically compatible and when the patient is younger and less transfused.

Cord Blood Transplantation
Instead of harvesting stem cells from a donors bone marrow or blood, stem cells are taken from the "cord blood" found in the umbilical cord/placenta of a newborn sibling. Unlike regular bone marrow transplantation, the stem cells taken from the cord blood do not have to be a perfect genetic match. There appears to be a lower instance of rejection in cord blood transplantation.
Gene Therapy
The objective of GENE therapy is to insert a normal beta - globin gene into the patients stem cells, thus allowing increased production of beta globin and healthy red blood cells

Hydroxyurea
Hemoglobin is composed of two protein chain-alpha globin chain and beta globin chains. Patients with beta-thalassemia do not make beta globin, they often require frequent red blood cell transfusions.

Some drugs, including hydroxyurea, can stimulate production of a third type of protein chain called gamma chains. In the womb, the foetus makes this type of protein instead of beta globin. It is not until after birth, when the foetus no longer produces gamma globin that the beta globin deficiency becomes apparent.

Hydroxyurea has been shown to induce production of fetal hemoglobin. Fetal hemoglobin has a pair of gamma-globin molecules in place of the typical beta-globins of adult hemoglobin. Higher-than-normal levels of fetal hemoglobin can ameliorate some of the symptoms of thalassemia. Effects in patients with -thalassemia major are controversial. We now report a marked elevation of total Hb levels with HU that permitted regular transfusions to be stopped in some children with transfusion-dependent -thalassemia.

Check List :

Cardiac Evaluation - The following tests are standard protocol and should be performed at least once a year Cardiac Echo 24 hour Holter monitor EKG Cardiac Stress Test ( for patients 18years and older) Iron related Heart problems can be determined by T2* (MRI) testing. It determines the degree of iron overload in the heart Liver Function Tests Annual Hepatitis C Surface antibodies (if hep C Ab positive, draw hep C PCR) SGOT and SGPT (every three months) Hepatitis B panel If you have elevated ALT, it is recommended you repeat the test every month. If persistently elevated then you should consider a liver biopsy. Ophthalmology Evaluation: Annual Evaluation for cataracts, night blindness, decreased visual field (recommended for patients on Desferal and those who are diabetic) Audiological Evaluation: Annual Audiological Evaluation (every 6 months if on Desferal) for hearing changes and Tinitus (ringing in the ears). Others Annual HIV

In Thalassemia, although most of the iron overload is due to blood transfusions, a small amount of iron is also absorbed from the diet. The amount absorbed is higher when the hemoglobin is low.

There are two kinds of iron in the diet:

Iron which is present in red meat (meat iron) Iron which is widely distributed in the diet (non-meat).

Intake of Milk, a rich source of calcium, should be at least one pint daily, particularly because it helps to prevent osteoporosis. The absorption of non-meat iron is much less into our body as compared to the meat-iron.

Iron chelators such as Desferal and Deferiprone not only bind iron but also some Zinc and excrete it in the urine. The main nutritional sources of Zinc are animal foods (meat and dairy products) and wholemeal cereals. Dairy products like milk, cheese and yoghurt, eggs etc contain a lot of Zinc.

In thalassemia, because of the excess iron in the body, there is a higher risk of oxidative damage. The four main antioxidants are vitamin E, vitamin C, carotenoids and flavonoids.

Vitamin E is mainly found in vegetable oils such as olive oil and safflower oil Vitamin C is present in fruit and vegetables. It is best not to consume many of those in combination with foods that are high in non-meat iron. Carotenoids are found in carrots, yellow squash, corn, tomatoes, papaya, oranged and dark-green leafy vegetables. As these foods are also high in vitamin C, the above caution applies Tea and Red wine contain Flavonoids. Tea also inhibits iron absorption.