Occasionally people say that: “the cure is worse than the illness.” While that is not always true, it is important to be aware of the side effects of treatments and have an open discussion with your doctor to adjust treatment plans as needed. However, some side effects of a treatment may not have any symptoms, so awareness is important. One such side effect is iron overload, which can occur in patients who undergo frequent blood transfusions, like those with sickle cell disease.
Sickle cell disease, also known as sickle cell anemia, is a genetic blood disorder that affects the shape and flexibility of red blood cells, thus interfering with how oxygen is transported in the body. The disease is inherited only when both parents pass down a defective copy of the gene— meaning if two parents have the gene (sickle cell trait), there is a one in four chance the child will get the disease. In the United States, the disease affects 1 in 500 African-Americans, and 1 in 1,000 Hispanic-Americans.
Specifically, it occurs when one of the components of blood, the protein hemoglobin, becomes mutated and changes the round shape of a red blood cell into a sickle, similar to the letter “C”. Hemoglobin carries oxygen from the lungs to the rest of the body, but in this form it cannot carry oxygen to the cells efficiently, causing acute pain, sometimes resulting in hospitalization. Since the sickle cell lives only 10 to 20 days compared to a regular blood cell that lasts about 120 days, the person usually becomes anemic.
One treatment for sickle cell anemia is blood transfusions, however the treatment may have its own risks. If a person has had over 10 blood transfusions in their lifetime, they may develop iron overload. This occurs when the iron is stored in organs such as the liver, pancreas, or heart, resulting in liver disease, diabetes or heart failure. It is important to have the blood tested for iron levels with a serum ferritin test. With this knowledge, the physician can plan the best course of treatment to both manage sickle cell and prevent any complications from iron overload.
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