What causes the pain in Sickle Cell Disease?
At times, sickle cells become stuck in tiny blood vessels. When they get stuck, the cells pile up and block the blood vessels. This cuts off the blood supply to nearby tissues so that no cells can get through to bring oxygen to them. Without oxygen, the area becomes inflamed and starts to hurt. This blockage of blood vessels is known as a sickle cell pain episode or “crisis.” Pain episodes may be very severe and need to be treated in the hospital with pain-killing drugs or, more often, they are milder and can be treated at home. If the blockage is long-lasting, it can destroy areas of tissue. Sickle cell pain occurs most frequently in bones. Until recently, there was no effective treatment to prevent the sickling that causes a pain crisis. A 1995 study reported that treatment with a drug called hydroxyurea reduced the number of pain crisis in severely affected adults by about 50 percent. However, this drug is not yet recommended for affected children. Studies looking into the safety of
