Like sickle cell anemia, thalassemia is another inherited blood problem and refers to a group of blood disorders causing anemia. Anemia refers to low levels of, or poorly functioning hemoglobin. Hemoglobin is a molecule found in red blood cells that is responsible for holding on to oxygen while the blood is circulated around the body and it then releases it to the tissues that need it. In thalassemia, hemoglobin is not produced properly. With abnormal hemoglobin a person feels very tired and weak because their tissues are not getting enough oxygen.

Thalassemia is found mainly in people from the Mediterranean regions of the world. With immigration it is more and more common to see it in Canadians. Thalassemia is most common in people from Cyprus at 16%. In India, Pakistan, and Bangladesh it is present in 3—8% of the population, and in 3—14% of people from Thailand.

Hemoglobin is made up of different types of “chains.” There is an alpha chain, a beta chain, and a delta chain that can go wrong in thalassemia. In alpha-thalassemia the alpha-chain is damaged, and in beta-thalassemia the beta-chain in damaged. Like sickle cell anemia, thalassemia is a recessive condition, meaning that a person must inherit the damaged gene from both parents to have full-blown thalassemia. If they inherit the damaged gene from only one parent, the thalassemia is not as bad and they can live quite normal lives. In parents who are both carrying the gene, each of their children has a 25% chance of inheriting the disease. If the child does not inherit full-blown thalessemia, they have almost a 70% that they are carriers of it and have the less severe form of thalassemia.

Symptoms of thalassemia include fatigue, weakness, light-headedness, pale skin, yellow skin (jaundice), dark urine, decreased appetite and weight-loss, and poor exercise tolerance. People with thalassemia often have protruding stomachs as their spleens and livers are enlarged. They also tend to have poor bone growth.

Diagnosis is made with a special blood test for hemoglobin. A complete blood count is done at first as well as iron studies.

Treatment of thalassemia involves blood transfusions and sometimes a bone marrow transplant. Since people with the disease are prone to infections, often heavy duty antibiotics are required. Iron supplements don’t help like they do in other forms of anemia, because the problem is not that there is low iron. As mentioned, the problem is in the hemoglobin itself. This means that with repetitive blood transfusions, iron can build up in the body and needs to be removed by chelation. Surgery is sometimes needed to deal with the enlarged spleen and at times, the gallbladder needs to be removed as well. Carriers of thalassemia do not need treatment.

There is no way to prevent thalassemia, but it can be diagnosed prenatally, before a baby is born.

People with thalassemia need to be careful with taking multivitamins and minerals as they do not need any extra iron. A folic acid supplement is a good idea to help the body to make new red blood cells. Flu shots and certain vaccinations are important. Please see your family doctor and they can direct you.