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20 - Sickle Cell Pain: Biology, Etiology, and Treatment

from SECTION FIVE - SICKLE CELL DISEASE

Published online by Cambridge University Press:  03 May 2010

Martin H. Steinberg ,
Bernard G. Forget ,
Douglas R. Higgs  and
David J. Weatherall
By
Samir K. Ballas  and
James R. Eckman
Martin H. Steinberg
Affiliation:
Boston University
Bernard G. Forget
Affiliation:
Yale University, Connecticut
Douglas R. Higgs
Affiliation:
MRC Institute of Molecular Medicine, University of Oxford
David J. Weatherall
Affiliation:
Albert Einstein College of Medicine, New York
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Summary

INTRODUCTION

Definition of Acute Pain Episodes

Pain is an unpleasant sensory and emotional experience. Acute pain episodes in sickle cell disease are mildly to excruciatingly unpleasant sensory and emotional experiences that occur episodically and unpredictably in one or many parts of the body, lasting from hours to weeks for which there is no other pathological explanation. Episodes of acute pain are the most common and distressing manifestations of sickle cell disease for the affected individual and their families. Their onset is early in life and unpredictable recurrences cause life-long suffering; they are disruptive to social and psychological functioning; they result in frequent utilization of healthcare resources, and they are a predictor of reduced survival.

Pain can occur anywhere, be localized to one or a few areas, be migratory, or be generalized. Back, chest, extremities, and joints are most often involved. Swelling and tenderness over affected areas and low-grade fever are common. The pain episode occurs in phases with an average length of approximately 10 days in adults.

The pathophysiology of painful episodes is poorly understood despite years of investigation. Observations during acute painful events suggest they result from diffuse ischemia of bones and muscles caused by disruption of blood flow in small and large blood vessels. The pathogenesis of the disruption of blood flow is complex and not well understood and is likely to be related to sickle cell–related vasoocclusion and vasculopathy, whose proximate cause is polymerization of deoxygenated sickle hemoglobin (HbS).

Type
Chapter
Information
Disorders of Hemoglobin
Genetics, Pathophysiology, and Clinical Management
 , pp. 497 - 524
Publisher: Cambridge University Press
Print publication year: 2009

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