Brief Screening Measures Identify Risk for Psychological Difficulties Among Children with Sickle Cell Disease

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Brief Screening Measures Identify Risk for Psychological Difficulties Among Children with Sickle Cell Disease Anna M. Hood1,4   · Ilana Reife2 · Allison A. King3 · Desiree A. White1

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Abstract Children with sickle cell disease (SCD) experience disproportionately high rates of psychological problems. Our goal was to examine the clinical utility of psychological screening measures to identify children with such problems in medical settings. Caregivers completed screening measures assessing social–emotional problems, ADHD symptoms, executive dysfunction, and health-related quality of life for children with SCD (receiving either chronic blood transfusion or hydroxyurea) and their siblings. Our findings demonstrated that screening measures identified clinically elevated symptoms in children with SCD that had not been previously reported. Scores for siblings were for the most part in the normal range. The number of days hospitalized (but not cerebral infarct status) predicted higher scores, emphasizing the challenges associated with SCD complications. Overall, our findings support the notion that screening measures reduce the need for reliance on medical provider judgment for psychological referrals and increase equitability in access to services. Early identification resulting in early intervention has contributed substantially to improved psychological functioning in many contexts, and it is thus likely that such improvements would also be achieved in this uniquely vulnerable population. Keywords  Sickle cell disease · Children · Behavior · Executive dysfunction · Quality of life · Transfusion · Hydroxyurea Sickle cell disease (SCD) is a chronic genetic disorder characterized by the production of abnormal hemoglobin in the red blood cells and affects approximately 100,000 Americans (Rees, Williams, & Gladwin, 2010). Children with SCD face life-threatening medical (Redding-Lallinger & Knoll, 2006) and neurologic (DeBaun & Kirkham, 2016) challenges, but in comparison to other pediatric chronic illness populations, such as childhood cancer or asthma (Bennett, Shafran, Coughtrey, Walker, & Heyman, 2015), there is less research regarding their psychological functioning * Anna M. Hood [email protected] 1



Department of Psychological & Brain Sciences, Washington University, Campus Box 1125, St. Louis, MO, USA

2



Children’s Neuropsychological Services, Andover, MA, USA

3

Program in Occupational Therapy and Pediatrics, Division of Hematology and Oncology, Washington University School of Medicine and St. Louis Children’s Hospital, St. Louis, MO, USA

4

Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, MLC 7039, Cincinnati, OH 45229, USA





(Anie, 2005). This is particularly surprising given the intense chronic treatment regimens, unpredictable painful episodes, and frequent hospitalizations. Children who receive the primary disease-modifying treatments for SCD-related comp