How Long Can You Live With Thalassemia?

Thalassemia is an inherited blood disorder (hemoglobinopathy). Thalassemia occurs when a gene or genes related to the alpha or beta globin chains in the hemoglobin molecule are missing or mutated and this cause decrease or absent production of hemoglobin. There are two main types of thalassemia according to what globin chain that is defected: alpha thalassemia and beta thalassemia.^[1]

Alpha thalassemia silent carriers, trait and hemoglobin constant spring has a normal life span. Alpha thalassemia major people usually have a normal life span with proper treatment and management of thalassemia.^[2] Hemoglobin Bart’s is a severe form of alpha thalassemia, usually most of the fetus dies in the womb or soon after birth due to severe anemia.^[3] Beta thalassemia traits have a normal life span. Beta thalassemia intermedia also have a normal life span with proper treatment and management of beta thalassemia. The average life expectancy of a person with beta thalassemia major is about 17 years, most of the time by the age of 30 they die due to iron overload causing cardiac complications.^[4]

About 1.7% of world’s population has alpha or beta thalassemia and roughly about 5% of world’s population has some kind of a globin change. Males and females are equally affected by thalassemia and it occurs in 4.4 of every 10,000 live births. Alpha thalassemia is seen more commonly in African and Southeast Asian people, and beta thalassemia is seen more commonly in Mediterranean, African and Southeast Asian people. In these ethnic groups’ thalassemia trait is seen in 5 to 30% of people.^[5]

There are different types of alpha thalassemia according to the number of gene deletions and symptoms.

Alpha thalassemia silent carriers, trait and hemoglobin constant spring usually are asymptomatic or sometimes can have mild anemia, therefore the thalassemia does not affect their normal life span.

Alpha thalassemia major people are symptomatic and they need few blood transfusions. If the anemia becomes severe splenectomy is needed. However, they usually have a normal life span with proper treatment and management of thalassemia.^[6]

Hemoglobin Bart’s is a severe form of alpha thalassemia, usually most of the fetus dies in the womb or soon after birth due to severe anemia.

Beta thalassemia is also divided into sub types according to the gene defects and symptoms.

Beta thalassemia trait people are asymptomatic or have mild anemia. They have a normal life span. Beta thalassemia intermedia also have a normal life span with proper treatment and management of beta thalassemia

The average life expectancy of a person with beta thalassemia major is about 17 years, most of the time by the age of 30, they die due to iron overload causing cardiac complications. Iron tends to accumulate in the body with blood transfusions and increased absorption from the diet. This excess iron is deposited in many organs and cause problems in the function of the organ. Iron deposits in the pituitary gland, eyes, thyroid gland, heart, liver and pancreas. A study done on 240 thalassemia major patients showed that 71% died due to cardiac causes, 12% died due to infections (mainly from blood transfusion and low immunity), 6% from liver disease and 11% due to other causes. All of these patients died young before the age of 30 years.^[7]

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