at PENNSYLVANIA STATE UNIV on February 27, 2014jrs.sagepub.comDownloaded from Management of pain in sickle-cell disease

The acutely painful episodes that characterize sickle-cell disease were described in 1872 by Africanus Horton1, though the mechanism remained uncertain until, nearly thirty years later, James Herrick observed the sickling deformity of red cells that causes vaso-occlusion and tissue infarction. Sickle-cell disease (SCD) is the commonest globin gene disorder: across the world, about 300 000 children are born with it each year3. The pain of sickle-cell crisis is excruciating and, in global terms, a major health problem. TYPES OF PAIN IN SICKLE-CELL DISEASE Pain caused by sickle-cell disease can be acute, chronic or a mixture of the two. The acute pain of tissue infarction, in skeletal or soft tissue, tends to be sudden, unpredictable in onset and intense. After resolution of sickle-cell crisis, i