Hemoglobin the iron gets recycled. This process also produces one molecule of carbon monoxide for every molecule of heme degraded. Heme degradation is one of the few natural sources of carbon monoxide in the human body, and is responsible for the normal blood levels of carbon monoxide even in people breathing pure air. The other major final product of heme degradation is bilirubin. Increased levels of this chemical are detected in the blood if red blood cells are being destroyed more rapidly than usual. Improperly degraded hemoglobin protein or hemoglobin that has been released from the blood cells too rapidly can
Fetal hemoglobin round to sickle-shaped. These defective red blood cells have a greater tendency to stack on top of one another and block blood vessels. These invariably lead to so-called "painful vaso-occlusive episodes", which are a hallmark of the disease. If fetal hemoglobin remains the predominant form of hemoglobin after birth, the number of painful episodes decreases in patients with sickle-cell disease. Hydroxyurea promotes the production of fetal hemoglobin and can thus be used to treat sickle-cell disease. The fetal hemoglobin's reduction in the severity of the disease comes from its ability to inhibit the formation of hemoglobin aggregates within red blood