28 October, 2009
Thalassemia and sickle cell are genetic disorders. Thalassemia is the result of an imbalance in the two globin chains that produce the hemoglobin molecule. In sickle cell, a mutation of the genes causes an abnormal hemoglobin to be produced, which causes the cells to form a crescent shape (sickle). In either case, the production of hemoglobin is disrupted and cannot do its work of delivering oxygen to the body.
In sickle cell, the red blood cells stick together when deoxygenated and form polymers that make the cell membrane rigid and unable to flow through capillaries as needed.
Symptoms of sickle cell do not often appear until at least 4 months of age and can include the following: painful "crises" that last for long periods, fatigue, jaundice and difficulty breathing. Severe cases of thalassemia can cause death at birth. General symptoms of thalassemia include bone deformities, enlarged liver or spleen and stunted growth.
Blood transfusions are usually necessary for both sickle cell anemai and thalassemia. The major difference in treatment between the two is that sickle cell may also require dialysis, kidney transplant, surgery for eye problems or abnormal erections and gallbladder removal. Antibiotics are necessary due to the incidences of infection.
Sickle cell can cause organ failure if not properly treated. Sickle cell patients are also susceptible to infections that can be fatal if not immediately addressed. Thalassemia can lead to death by heart failure if not treated.
Thalassemia is more likely to be present in those of Greek or Italian descent, whereas sickle cell is more often found in people of African or Asian ancestry.