Pain Management of Sickle Cell Disease

Acute and chronic pain episodes are common experiences for many individuals living with sickle cell disease (SCD). The management of pain related to SCD is primary palliative and includes pharmacological, nonpharmacological, and preventive therapies (Ballas, 2002). This chapter focuses on issues relevant to the assessment and treatment of sickle cell pain in primarily pediatric populations, although some of the adult literature is reviewed, particularly as it relates to children and adolescents. SCD is a hereditary disorder. The disorder primarily affects people of Caribbean and African origin in addition to a small percentage of people of Indian, Mediterranean and Middle Eastern descent (National Association of Health Authorities and Trusts–NAHAT, 1991). SCD is also prevalent in Hispanic and Latino populations. Red blood cells containing sickle hemoglobin become rigid. These cells then become elongated and “sickled” in shape, which makes it very difficult for the sickle cells to move smoothly and flow through microcirculation (Morrison & Vedro, 1989). Symptoms of SCD include chronic anemia, susceptibility to infection, and vaso-occlusive crises or vaso-occlusive episodes (VOEs), resulting in severe pain that can last hours to weeks (Wang, George, & Wilimas, 1988). This vaso-occlusion is caused when sickle cells are unable to flow through arteries, capillaries, arterioles, and other blood vessels and as a result obstruct blood flow. This sickling can occur anywhere in the body, including fingers, arms, ribs, abdomen, and organs such as the brain and eyes (Morrison & Vedro, 1989), but it most commonly occurs in the spleen, bones, and joints (Elander & Midence, 1996). These restrictions in blood flow result in severe pain. In addition, other major clinical problems arise from SCD, including stroke, acute chest syndrome, aseptic necrosis of the hips and shoulders, infections, anemia, leg ulcers, and priapism (Serjeant, 1992). Acute chest syndrome is a significant concern and is the leading cause of mortality in both children and adults with SCD (Platt et al., 1994). Stroke tends to be the most disabling condition and is also a significant cause of death. Stroke in SCD patients accounts for approximately 6% of deaths related to sickle cell disease (Platt et al., 1994).