Psychosocial and behavioral outcomes in children with sickle cell disease and their healthy siblings Jeffrey I. Gold Æ Nicole E. Mahrer Æ Marsha Treadwell Æ Lina Weissman Æ Elliott Vichinsky Accepted: August 27, 2008 / Published online: September 11, 2008 Ó Springer Science+Business Media, LLC 2008 Abstract The current study aims to compare positive and negative measures of psychosocial functioning among children with sickle cell disease (SCD) and their healthy siblings. Participants were 41 African-American children with SCD, 97 healthy siblings, and their primary caregivers. Primary caregivers completed self-report questionnaires assessing child behavioral problems, while children with SCD and siblings completed self-report questionnaires assessing coping, self-efficacy, and perceived social sup- port. No significant differences were noted between children with SCD and their siblings on all measures. Both groups reported self-efficacy and perceived social support within the normative range, and endorsed significantly greater use of Positive/Approach coping. In general, both groups of children do not have clinically significant behavioral prob- lems. However, secondary exploratory analyses identified that a greater percentage of children from both groups scored above the established clinical cutoff on the behavioral summary scores. Number of visits to the emergency room was related to behavioral problems in children with SCD. While previous reports have been mixed in their findings that children with SCD are at greater risk for psychosocial and other behavioral problems, the current report finds that children with SCD and their healthy siblings endorse posi- tive psychosocial functioning and as a group do not have clinically significant behavioral problems. Nonetheless, ongoing psychosocial evaluation for children receiving treatment for SCD is vital. Keywords Sickle cell disease Á Coping Á Self-efficacy Á Social support Á Behavioral problems Á Psychosocial adjustment Introduction Sickle cell disease (SCD) is a chronic, autosomal recessive disorder characterized by the predominance of the protein hemoglobin S (HbS) in red blood cells. Hemoglobin, which is critical for the transport of oxygen throughout the body, is composed of two alpha globulin chains and two beta globulin chains. In sickle hemoglobin, the beta globulin differs from normal globulin by the substitution of valine J. I. Gold (&) Keck School of Medicine, University of Southern California, Los Angeles, CA 90087, USA e-mail: jgold@chla.usc.edu J. I. Gold Á N. E. Mahrer Department of Anesthesiology Critical Care Medicine, Childrens Hospital Los Angeles, 4650 Sunset Blvd. MS#12, Los Angeles, CA 90027, USA N. E. Mahrer e-mail: nmahrer@chla.usc.edu M. Treadwell Northern California Comprehensive Sickle Cell Center, Children’s Hospital & Research Center Oakland, 747 52nd St., Oakland, CA 94609, USA e-mail: mtreadwell@mail.cho.org L. Weissman Edgewood Center for Children and Families, 1801 Vicente St., San Francisco, CA 94116, USA e-mail: linaw@edgewoodcenter.org E. Vichinsky Hematology/Oncology Department, Children’s Hospital & Research Center Oakland, 747 52nd St., Oakland, CA 94609, USA e-mail: evichinsky@mail.cho.org 123 J Behav Med (2008) 31:506–516 DOI 10.1007/s10865-008-9175-2