Sickle Cell Disease
Jennifer J. Wilson Schaeffer
University of Kentucky
Karen M. Gil
Laura S. Porter
University of North Carolinaat Chapel Hill
Sickle cell disease (SCD) is an inherited disorder affecting 1 in 500 African Americans, in which the most common complication is variable and unpredictable episodic pain ( Weisman & Schechter, 1992). Since the 1980s, there have been exciting advances made in the medical management and prevention of these painful episodes. Recent studies have demonstrated that the antimetabolite medication, hydroxyurea, is effective in reducing painful episodes in adults and children ( Charache et al., 1995; Scott, Hillery, Brown, Misiewicz, & Labotka, 1996). In addition, medical centers are exploring the use of bone marrow transplant to treat patients with SCD who have multiple medical complications ( Walters et al., 1996). Despite these improvements, there is still no cure for SCD. Children and adults with SCD cope with both acute and chronic complications including painful episodes.
The purpose of this chapter is to present a biopsychosocial perspective on coping with SCD pain. We first describe the clinical features of SCD and outline the current medical approaches to SCD pain. Second, we discuss the relevant psychosocial factors. In the third and fourth sections, we review the research on the assessment and the psychological and cognitive-behavioral treatment of SCD pain. In these two final sections, suggestions for clinical practice are highlighted.
SCD is characterized by the production of primarily hemogloblin S (HbS), which is structurally different from normal hemoglobin (HbA) by the