The association of treatment adherence with quality of life (QOL) and the role of sickle cell disease complications were explored in children with sickle cell disease. Primary caregivers of 43 children, ages 5 years and older, and 21 children, ages 8 years and older, completed a standardized measure of QOL during an admission for pain or fever to the hematology acute care unit. Adherence was measured through medical staff ratings, caregiver-report of sickle cell disease-related care activities, and matching of medical staff standard recommendations for treatment of pain and fever with sickle cell disease-related care activities. Sickle cell disease complications were assessed via medical file review. Pearson correlation coefficients indicated that better adherence was associated with poorer overall QOL. In follow-up analyses, although sickle cell disease complications were associated with adherence, it did not explain the negative correlation of adherence with QOL. Higher treatment adherence may interfere with activities that contribute to QOL for some children. Further research to investigate the role of sickle cell disease complications, as well as psychosocial factors, in determining both treatment adherence and QOL is suggested.

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to determine the effects of coping style and family functioning on children's adjustment to sickle cell disease (SCD). Participants: A sample of 73 caregivers and 23 children (ages birth to 18 years) admitted to a hematology acute care unit for pain or fever associated with SCD. Setting: A children's hospital on the East Coast of the United States. Outcome Measures: Disability stress, disease severity, child and parent coping, family functioning, and child adjustment were assessed with standard paper-and-pencil measures. Results: Results provide partial support for J. L. Wallander et al.'s (1989) risk-resistance model. Disability stress did not mediate the relation between disease severity and child adjustment, and severity of medical condition and medical stress did not predict adjustment. Child gender and child age predicted family functioning and child adjustment to SCD. Child or parent coping strategies did not moderate the association of disability stress and child adjustment. Conclusions: Individuals working with patients should be aware of other factors that may affect child outcome above disease severity; specifically, concerns of boys and girls with SCD and their caregivers should be assessed.Sickle cell disease (SCD) affects predominantly the African American population, with 1 in every 400 to 500 African Americans becoming infected (Hurtig, Koepke, & Park, 1989). In SCD abnormal hemoglobin molecules cluster together during deoxygenation, resulting in sickle-shaped red blood cells that obstruct blood flow. SCD produces a number of symptoms and can lead to early mortality (Lemanek, Buckloh, Woods, & Butler, 1995).Individuals with SCD vary in severity with some requiring 3 to 4 months of hospitalization a year, whereas others never use hospital services. Clinical presentation includes vaso-occlusive crises, infection, cerebrovascular accidents, anemic episodes, retarded growth, deterioration in vision, cardiac concerns, abdominal pain, hyperbilirubinemia, and delayed puberty (Charache, Lubin, & Reid, 1984;Lemanek et al., 1995). Pain episodes are one of the most reported symptoms noted in SCD and can result in multiple hospitalizations and repeated absence from school. Traditional treatment for SCD includes hydration, warmth, transfusion therapy, and analgesic medication, although nonpharmacologic meth-ods to reduce pain, including cognitive-behavioral and guided imagery intervention, have been evaluated (Gil, 1989;Gil et al., 2001). Improved medical care has led to an increased life span for individuals with SCD, with females living approximately 48 years and males approximately 42 years (Lemanek et al., 1995). With this increase in the life span of children with SCD, research has begun to focus on how individuals are affected by, and continue to live with, factors associated with chronic illness.Recent reports indicate wide variations in the adjustment of children with SCD (

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