Adjustment and coping in adults with sickle cell disease: An assessment of research evidence

This paper considers the findings from psychological studies of adults with sickle cell disease (SCD), an inherited haematological disorder which causes recurrent episodes of acute pain. Studies are reviewed that deal with aspects of the condition's clinical presentation in adulthood, the risk of maladjustment and psychopathology among affected groups, the role played by cognitive factors in mediating psychological distress and facilitating more effective coping, and the implications of the condition for family life and employment. Adults with SCD experience high levels of maladjustment by comparison with other illness groups, and differences in adjustment are predicted better by measures of social support and life stress than by illness variables. The use of coping strategies plays an important role in pain management and the mediation of psychosocial adjustment, with passive and negative responses to painful episodes associated with less favourable outcomes. Existing findings in this rapidly expanding area of research have implications for the management of SCD and the organization of support services, and point to a need for interventions to enhance patients' coping skills. Future research could evaluate specific cognitive and behavioural interventions to promote more effective coping, and look at the effects of SCD on reproductive behaviour, family life, performance in the workplace, and development and adjustment over the life-span.