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The management of painful crisis in sickle cell disease

Wright, Josh; Ahmedzai, Sam H

Current Opinion in Supportive and Palliative Care: June 2010 - Volume 4 - Issue 2 - p 97–106
doi: 10.1097/SPC.0b013e328339429a
Pain: non-malignant disease: Edited by Richard Langford

Purpose of review Until recently management of sickle pain was the province of haematologists. However, a recent National Confidential Enquiry into Patient Outcome and Death report highlighted problems with the management of pain and opioid analgesia in this group and suggested a multiagency approach similar to that used in palliative care.

Recent findings Pain is the most frequent complication of this haemoglobin disorder. Sickle cell disease is very variable with many patients leading full lives with long periods with little or no pain. At the other end of the spectrum there are those who exist in a sea of pain. The mechanisms of sickle pain are poorly understood and evidence for the best treatment modalities sparse. Historically there has been a dearth of clinical trials in sickle cell; however, this is starting to be addressed.

Summary In this review we will give a brief overview of the disease and its pathogenesis before examining the epidemiology, management of pain in sickle cell disease. We will also review recent evidence regarding quality of life and discuss the role of opioid hyperalgesia in sickle cell disease.

Academic Unit of Supportive Care, The University of Sheffield, Royal Hallamshire Hospital, Sheffield, UK

Correspondence to J. Wright, BMedSci, MBChB, FRCP, FRCPath, MD, Consultant Haematologist and Honorary Senior Lecturer, Royal Hallamshire Hospital, Sheffield S10 2JF, UK Tel: +44 1142711731; fax: +44 1142711733; e-mail:

© 2010 Lippincott Williams & Wilkins, Inc.