Institutional members access full text with Ovid®

Share this article on:

Widespread Pain Among Youth With Sickle Cell Disease Hospitalized With Vasoocclusive Pain: A Different Clinical Phenotype?

Zempsky, William T. MD, MPH; Wakefield, Emily O. PsyD; Santanelli, James P. MPH; New, Tamara MD, FAAP; Smith-Whitley, Kimberly MD; Casella, James F. MD; Palermo, Tonya M. PhD

doi: 10.1097/AJP.0000000000000403
Original Articles

Objectives: The purpose of this study was to describe the clinical phenotype of widespread pain (WSP) among youth with sickle cell disease (SCD) hospitalized with vasoocclusive pain.

Materials and Methods: One hundred fifty-six youth with SCD, between 7 and 21 years of age hospitalized at 4 children’s hospitals for a vasoocclusive episode were evaluated. Data were collected during 1 day of the hospitalization.

Results: Using the 2010 American College of Rheumatology guidelines, 21.8% of patients were identified as having WSP (pain in 7 or more unique body locations). Patients classified as having WSP had higher pain intensity (6.5 vs. 5.6; t=2.19, P=0.03) higher pain burden (13.0 vs. 9.8; t=3.09, P=0.002), higher acute functional disability (22.1 vs. 16.5; t=2.43, P=0.016), higher chronic functional disability (30.4 vs. 22.2; t=2.31, P=0.02), lower positive affect (22.9 vs. 27.6; t=2.23, P=0.027), and lower quality of life (56.2 vs. 62.9; t=1.99, P=0.049) than those youth with SCD without WSP.

Discussion: Assessment of WSP may identify a unique clinical phenotype of youth with SCD with differing treatment needs.

*Connecticut Children’s Medical Center

Institute of Living at Hartford Hospital, Hartford

University of Connecticut School of Medicine, Farmington, CT

§Children’s Healthcare of Atlanta at Emory University School of Medicine, Atlanta, GA

Children’s Hospital of Philadelphia, Philadelphia, PA

Johns Hopkins School of Medicine, Baltimore, MA

#University of Washington and Seattle Children’s Research Institute, Seattle, WA

Supported by NIH grant K23 HL090832-01A1. The authors declare no conflict of interest.

Reprints: William T. Zempsky, MD, MPH, Connecticut Children’s Medical Center, 282 Washington Street, Hartford, CT 06106 (e-mail:

Received November 13, 2015

Received in revised form August 3, 2016

Accepted June 11, 2016

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.