An in-depth report on the causes, diagnosis, and treatment of sickle cell disease.
Sickle cell anemia
What is Sickle Cell Disease?
Sickle cell disease is an inherited blood disorder in which the body produces abnormally shaped red blood cells. In sickle cell disease, the hemoglobin in red blood cells clumps together. This causes red blood cells to become stiff and C-shaped. These sickle cells block blood and oxygen flow in blood vessels. Sickle cells break down more rapidly than normal red blood cells, which results in anemia.
What Causes Sickle Cell Disease?
Sickle cell disease is a genetic disorder. People who have sickle cell disease are born with two sickle cell genes, one from each parent. If one normal hemoglobin gene and one sickle cell gene are inherited, a person will have sickle cell trait. People who have sickle cell trait do not develop sickle cell disease, but they are "carriers" who can pass the abnormal gene on to their children.
Complications of Sickle Cell Disease
Sickle cell disease can block the flow of blood in arteries in many parts of the body, causing many complications. The hallmark of sickle cell disease is the sickle cell crisis, which causes sudden attacks of severe pain. An infection or by blockage of blood vessels in the lungs can trigger acute chest syndrome, another common and serious occurrence. Additional medical complications include:
- Pulmonary hypertension (increased pressure in the arteries of the lungs)
- Kidney problems
- Priapism (prolonged and painful erections)
- Liver problems
- Gallbladder disease
- Spleen damage
- Bone and joint problems
- Leg sores and ulcers
- Eye damage in the retina
Recent Findings about Sickle Cell Disease
Hydroxyurea is being studied to replace transfusion therapy in select patients for the prevention of stroke. Sildenafil (Viagra), taken on a daily basis, may actually help reduce the frequency of priapism.