Sickle cell disease affects approximately 72,000 Americans with most being of African-American descent. This disease is a genetic disorder that causes the red blood cells to be misshapen and this will interfere with their ability to carry oxygen throughout the body.
Research into sickle cell anemia did not really start until the 1970s. This is when the government passed the National Sickle Cell Anemia Control Act. This act provided for information and education programs for health professionals and the public. It also established voluntary sickle cell anemia screening and counseling programs. (1,2)
The Secretary of the Department of Health, Education and Welfare took the act a step further by establishing a National Sickle Cell Disease Program and assigned the National Heart, Lung and Blood Institute the responsibility of developing a program of research into sickle cell disease. (3)
Great advancements have been made since the establishment of these government programs. People who are born with sickle cell anemia now have access to medications and treatments that can help them avoid a sickle cell crisis.
Two of the organs most susceptible to damage from a sickle cell crisis are the brain and the lung. Sickle cell disease has been known to cause strokes and a condition called acute chest syndrome in which an infection gets trapped in the lungs and causes respiratory distress.
One of the problems facing infants born with sickle cell disease was the high incidence of pneumococcal infections. However, with research it was discovered that penicillin could prevent the occurrence of Streptococcus pneumoniae sepsis in infants and toddlers.
Now, all infants are screened at birth for sickle cell anemia. If the infant is positive for the disease, he or she will be started on penicillin at around two months of age and will continue taking this drug until they are five years old.
Research has found that blood transfusions can increase the number of red blood cells in circulation and help to relieve anemia. More importantly, blood transfusion can decrease the risk of stroke in children with sickle cell anemia.
A bone marrow transplant can be a cure for sickle cell disease, but it is a very risky procedure.. It can be very difficult to find a suitable bone marrow donor and many complications can arise during a bone marrow transplant. Sometimes the body will reject the donated bone marrow. More research is being performed on bone marrow transplants as a cure for sickle cell disease.
As research into sickle cell anemia continues, new discoveries are being made every day. Researchers are finding that certain medications that are used to treat other diseases can be beneficial in the treatment of sickle cell anemia.
Gene therapy is being studied as a way to cure sickle cell anemia. As technology advances, sickle cell anemia may become a thing of the past.
(1) The American Presidency Project
(2) Sickle Cell Association of the National Capital Area: http://www.scancainc.org/
(3) National Heart Lung and Blood Insitute: Report of the National Heart, Lung, and Blood Advisory Council Subcommittee Review of the NHLBI Sickle Cell Disease Program