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Health-related Quality of Life in Children With Sickle Cell Disease Undergoing Chronic Red Cell Transfusion Therapy

Maxwell, Sarah L. MD*,†; Schlenz, Alyssa M. PhD; Kanter, Julie MD

Journal of Pediatric Hematology/Oncology: May 2019 - Volume 41 - Issue 4 - p 307–312
doi: 10.1097/MPH.0000000000001376
Original Articles

Chronic red cell transfusion (CRCT) therapy is one of few disease-modifying treatments for sickle cell disease (SCD). This study evaluated health-related quality of life (HRQL) in children receiving CRCT relative to 2 comparison groups: children with similar, severe SCD and children with milder disease risk defined by SCD genotype. For this study, 67 children with SCD between the ages of 8 and 18 completed the self-report Pediatric Quality of Life Sickle Cell Disease module (PedsQL SCD) as part of a pilot clinical program during routine hematologic visits. A medical chart review was also performed. Linear regression suggested that children in the CRCT group had significantly higher self-reported HRQL ratings for domains related to pain, F2,64=4.07 (P=0.022) and pain-related functioning, F2,64=4.32 (P=0.017), compared with children with similar and milder disease risk. Exploratory analyses implied that children in the CRCT group also had fewer worries about SCD-related complications, F3,63=9.68 (P<0.001). These patient-perceived benefits of CRCT may have important implications for treatment decisions and for providing ancillary support for children with SCD and their families.

*Department of Pediatrics, Brown University, Providence, RI

Department of Pediatrics, Medical University of South Carolina, Charleston, SC

S.L.M. was supported by the Medical University of South Carolina College of Medicine Alumni Loyalty Fund and Medical Student Summer Research Grant.

The authors declare no conflict of interest.

Reprints: Alyssa M. Schlenz, PhD, Department of Pediatrics, Division of Developmental-Behavioral Pediatrics, Medical University of South Carolina Children’s Hospital, 135 Rutledge Avenue, MSC 561, Charleston, SC 29425 (e-mail:

Received June 11, 2018

Accepted October 30, 2018

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