Sickle Cell Anemia

IT has been estimated that as many as 72,000 people in this country are affected by sickle cell, and as many as 2 million Americans carry the trait. The rates are highest among African- Americans with I in 500 Blacks affected and I in I 2 carrying the trait.

Sickle cell anemia is a blood disorder that is inherited when both parents have the same trait for defective hemoglobin, the molecule in red blood cells that carries oxygen to the body’s organs and brings carbon dioxide back to the lungs to be released. With sickle cell anemia, the hemoglobin molecules may cluster after they release their oxygen and form long, rod-like structures. It is a chronically painful condition that ultimately can lead to the damage of tissue and vital organs. Also, because the sickled red cells die much faster than normal red cells and cannot be replaced quickly, sufferers are chronically short of red blood cells, giving rise to anemia.

During the last 30 years, there has been a great deal of progress among researchers in understanding sickle cell and its effects. This has resulted in some success in treating some of the complications and in developing drugs that can treat symptoms. Bone-marrow transplantation has been helpful in treating severe symptoms in children and some research attention has focused on finding ways to help adults produce more fetal hemoglobin, which has been shown to reduce the severity of the disease and cause red cells to survive longer in the bloodstream. Ultimately, it seems that genetic therapy may hold the cure, according to medical experts.

Now a study for the National Institutes of Health may help health professionals understand why women have fewer sickle cell crises and a longer average lifespan with sickle cell than men. According to the findings of the study, it may be due to the ability of women to manufacture more nitric oxide, the substance that helps dilate blood vessels. That is important because the arteries in sickle cell sufferers can become blocked, due to their misshaped red cells. Women produce more than twice as much nitric oxide as men, probably due to their estrogen levels.

Copyright Johnson Publishing Company Mar 2004