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Blood. 2008 Feb 1;111(3):997-1003. Epub 2007 Oct 16.

Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void.

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Adult Sickle Cell Disease Program, Section of Hematology, Department of Medicine, Yale University School of Medicine, New Haven, CT 06520-8021, USA.


Pain due to vaso-occlusive crisis is the major cause of hospital use in sickle cell disease. Although available guidelines provide recommendations for opioid administration in this setting, only 4 (21%) of 19 medical textbooks present treatment regimens that are consistent with them. Moreover, only 7 texts (37%) note that addiction is infrequent in this population, while 11 (92%) of 12 texts provide such reassurance for cancer-related pain (P < .005). Finally, hydroxyurea use to decrease the frequency of vaso-occlusive crises is completely defined only in 2 textbooks. Thus, most medical texts provide neither adequate information for the treatment or prevention of pain due to vaso-occlusive crisis in sickle cell disease nor reassurance of the unlikelihood of addiction in this population. In contrast, treatment recommendations for less common hematologic disorders are consistent with current standards in 53% to 84% of appropriate texts (P < .05). Limited knowledge regarding the principles and appropriateness of opioid therapy; a lack of evidence-based research on pain control; and misconceptions and prejudices about drug abuse and addiction contribute to this educational void. Thus, research and training on pain control in sickle cell disease are needed to parallel studies of environmental and genetic factors contributing to the known clinical heterogeneity of this disorder.

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