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Thalassemia, treatment, iron chelation therapy

Treatment of Thalassemia

Thalassemia trait

Normally, there are no treatments recommended. However, the doctor may suggest taking iron or folic acid medication if they feel it is necessary.

Thalassemia major

The primary treatment is regular blood transfusions, usually every four weeks. In addition to the blood transfusions, doctors recommend injections of Desferal to help the body flush out the extra iron created by the new blood. The injections are given under the skin from a small pump 5 to 7 nights a week.

Additionally, splenectomy (removal of the spleen), bone marrow transplants and chelation therapy are being researched as possible treatments for thalassemia.

Medical therapy

Medical therapy for beta thalassemia primarily involves iron chelation. Deferoxamine is the intravenously or subcutaneously administered chelation agent. Deferasirox  (Exjade) is an oral iron chelation drug . Deferiprone is an oral iron chelator that has been approved in Europe since 1999 and many other countries.

Standard Treatments

Blood Transfusions

Transfusions of red blood cells are the main treatment for people who have moderate or severe thalassemias. This treatment gives you healthy red blood cells with normal hemoglobin.

During a blood transfusion, a needle is used to insert an intravenous (IV) line into blood vessels. Through this line, healthy blood is received by patient. The procedure usually takes 1 to 4 hours.

Red blood cells live only for about 120 days. So, may need repeated transfusions to maintain a supply of healthy red blood cells.

If patient have hemoglobin H disease or beta thalassemia intermedia, patient may need blood transfusions on occasion. For example, patient may need this treatment when have an infection or other illness, or when anemia is severe enough to cause tiredness.

If patient have beta thalassemia major, or Cooley’s anemia, patient need regular blood transfusions (often every 2 to 4 weeks). These transfusions will help to maintain normal hemoglobin and red blood cell levels.

Blood transfusions allow patient feel better, enjoy normal activities, and live into adulthood. This treatment is lifesaving, but it’s expensive and carries a risk of transmitting infections and viruses (for example, hepatitis). However, the risk is very low in the United States because of careful blood screening. .

Iron Chelation Therapy

Because the hemoglobin in red blood cells is an iron-rich protein, regular blood transfusions can lead to a buildup of iron in the blood. This condition is called iron overload. It damages the liver, heart, and other parts of the body.

To prevent this damage, iron chelation therapy is needed to remove excess iron from the body. Two medicines are used for iron chelation therapy.

  • Deferoxamine is a liquid medicine that’s given slowly under the skin, usually with a small portable pump used overnight. This therapy takes time and can be mildly painful. Side effects include problems with vision and hearing.
  • Deferasirox is a pill taken once daily. Side effects include headache, nausea (feeling sick to the stomach), vomiting, diarrhea, joint pain, and fatigue (tiredness).

Folic Acid Supplements

Folic acid is a vitamin that helps build healthy red blood cells. Patient may need to take folic acid supplements in addition to treatment with blood transfusions and/or iron chelation therapy.

Other Treatments

Other treatments have been developed or are being tested, but they’re used much less often.

Blood and Marrow Stem Cell Transplant

A blood and marrow stem cell transplant replaces faulty stem cells with healthy ones from another person (a donor). Stem cells are the cells inside bone marrow that make red blood cells and other types of blood cells.

A stem cell transplant is the only treatment that can cure thalassemia. But only a small number of people who have severe thalassemias are able to find a good donor match and have the risky procedure.

Possible Future Treatments

Researchers are working to find new treatments for thalassemias. For example, it may be possible someday to insert a normal hemoglobin gene into stem cells in bone marrow. This will allow people who have thalassemias to make their own healthy red blood cells and hemoglobin.

Researchers also are studying ways to trigger a person’s ability to make fetal hemoglobin after birth. This type of hemoglobin is found in fetuses and newborns. After birth, the body switches to making adult hemoglobin. Making more fetal hemoglobin may make up for the lack of healthy adult hemoglobin.

Treating Complications

Better treatments now allow people who have moderate and severe thalassemias to live much longer. As a result, these people must cope with complications that occur over time.

An important part of managing thalassemias is treating complications. Treatment may be needed for heart or liver diseases, infections, osteoporosis, and other health problems.

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.
  • 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.
  • Tea and Red wine contain Flavonoids. Tea also inhibits iron absorption.