thalassemia: causes and treatments

THALASSEMIA: CAUSES AND TREATMENTS

Thalassemia is an inherited blood disorder, a type of anemia that affects the production of hemoglobin.

Hemoglobin allows red blood cells to transport oxygen throughout the body.

Mild cases can often go untreated but more severe cases require medical attention as well as lifestyle changes.

Symptoms of Thalassemia include irritability, jaundice, dark urine, trouble breathing and slow growth in children.

What is Hemoglobin?

Hb is an iron-containing metalloprotein. In normal adult humans, the metalloprotein is composed of four globular subunits of Hb (two alpha subunits and two beta subunits), each carrying a heme group.

In turn, each heme group carries one iron atom that binds one oxygen molecule. In total, one adult human Hb protein typically carries four oxygen molecules.

ROLE OF HEMOGLOBIN

Hb transports oxygen from the lungs to body tissues, releases oxygen for cell use and then returns the carbon dioxide produced in the tissues to the lungs, to be exhaled.

Alterations in the structure and function of Hb can destroy RBCs, causing anemia.

CAUSES

GENETIC MUTATIONS

Four genes (two from each parent) are vital for normal levels of alpha-globulin, and two genes (one from each parent) are vital for normal levels of beta-globulin. Any mutation in a globular subunit gene can alter the protein’s structure and function. The two categories of thalassemia are alpha-thalassemia and beta-thalassemia, described by the globulin chain that is underproduced because of the mutation.

Normally, when the metalloprotein folds into its unique shape, each alpha subunit pairs with a beta subunit. In thalassemia, the excess of normally produced component accumulates and destroys the red blood cell. The severity of the disease depends on the number of genes that are mutated.

TREATMENTS

MEDICAL HELP

Consult doctor 1st.

Administer a blood test and look at your symptoms to determine the severity of your thalassemia.

If the test shows low levels of hemoglobin and your symptoms occur regularly, the doctor will recommend for treatment.

Get regular blood transfusions if your doctor recommends it. Severe cases may require eight or more transfusions per year.

Take iron chelators to decrease the amount of iron in your blood. Blood transfusions increase iron levels, which puts you at risk for heart, kidney and liver disease.

Get a bone marrow transplant if your doctor feels it is necessary. The risks are high in this type of operation.

LIFESTYLE CHANGES

Change your diet so you consume low amounts of iron and adequate amounts of calcium, vitamin D and zinc.

Drink at least one cup of green tea per day. The tannins in the tea help prevent your body from absorbing excess iron.

Avoid infections by practicing good hygiene, avoiding people with contagious illnesses and properly caring for injuries. Cover open wounds with bandages.

FOOD FOR THALASSEMIA PATIENTS

A person who has thalassemia must stick to a specific diet. Foods that are considered healthy for most people can cause serious complications for thalassemia patients.

Thalassemia is a group of genetic blood disorders that can cause life long health problems.

That’s why it’s important to know which food a thalassemia patient can and cannot eat.

Instructions

Avoid foods that contain iron. Some thalassemia patients must get regular blood transfusions. This causes an excess build up of iron in the body which can damage the heart and other organs.

Proteins that are high in iron and should be avoided include liver, oysters, pork, beans, beef, peanut butter and tofu.

Grains that are iron rich include flour tortillas, infant cereal, and cream of wheat.

Fruits and vegetables that contain iron and should be avoided include prunes, watermelon, peas, broccoli, raisins, spinach, and leafy green vegetables.

Eat plenty of foods that contain calcium. This is extremely important for keeping the bones strong and healthy.

This is crucial for people who have thalassemia since frail and brittle bones can be a complication of the disease.

Dairy products are a good source of calcium. An added benefit is that dairy products reduce the body’s ability to absorb iron.

Include vitamin D in your diet. Vitamin D is needed so that the body can absorb calcium. Vitamin D is contained in eggs, dairy products and fish.

(N.B.- ITS BETTER AND SAFE TO CONSULT A DOCTER BEFORE TREATMENT)