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Acute pain in children and adults with sickle cell disease: management in the absence of evidence-based guidelines

Field, Joshua Ja; Knight-Perry, Jessica Ea; DeBaun, Michael Rb

Current Opinion in Hematology: May 2009 - Volume 16 - Issue 3 - p 173–178
doi: 10.1097/MOH.0b013e328329e167
Erythroid system and its diseases: Edited by Narla Mohandas

Purpose of review Acute, vaso-occlusive pain is the most characteristic complication of sickle cell disease (SCD). Although there has been rigorous work examining the pathogenesis of vaso-occlusion, fewer studies have focused on approaches to the clinical management of acute pain. In this review, we will examine the epidemiology and management strategies of acute pain events and we will identify limitations in the best available studies.

Recent findings Most acute pain events in adults with SCD are managed at home without physician contact. Prior descriptions of the natural history of pain episodes from the Cooperative Study of Sickle Cell Disease relied on physician contact, limiting the generalizability of these findings to current practice. Patient-controlled analgesia has replaced on-demand therapy to become the standard for management of severe pain events in children and adults with SCD requiring hospital admission.

Summary Unfortunately, most clinical practice guidelines for the management of acute pain are not based on randomized clinical trials. As a result, our practice of pain management is primarily limited to expert opinion and inferences from observational studies. Additional clinical trials in management of acute pain in children and adults with SCD are critical for the development of evidence-based guidelines.

aDepartment of Internal Medicine, Washington University School of Medicine, USA

bDepartments of Pediatrics, Neurology and Biostatistics, Washington University School of Medicine, St. Louis, Missouri, USA

Correspondence to Joshua J. Field, MD, Division of Hematology, Department of Internal Medicine, 660 South Euclid Avenue, Campus Box 8125, St. Louis, Missouri, USA Tel: +1 314 286 1215; fax: +1 314 286 1185; e-mail:

© 2009 Lippincott Williams & Wilkins, Inc.