Fever and infection usually precipitate painful episodes in young children, while stress, changes in weather, or the menstrual cycle may cause painful episodes in adults. In many patients there is not an apparent trigger. Sites of pain are predictable. Infants and toddlers experience painful swelling of hands and feet called dactylitis. Children and young adults complain of pain in the long bones, sternum, and lumbar regions.
Some patients have abdominal pain episodes caused by mesenteric sickling.
These episodes can be so severe they mimic an acute, surgical abdomen. Occasionally, acute chest syndrome presents as referred pain to the abdomen prior to manifestation of infiltrate on chest tadiograph. Traditionally, pain management included sporadic dosing of IM or IV Demerol, oxygen supplementation, intravenous fluids, and blood transfusion. Often, adequate analgesia was not provided and this resulted in heightened patient anxiety and a roller coaster effect which was associated with p.r.n. dosing. Blood transfusions increase the risk of allo-immunization in blood borne infections and should not be used for the routine management of painful crisis.