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Hematology Am Soc Hematol Educ Program. 2017 Dec 8;2017(1):534-541. doi: 10.1182/asheducation-2017.1.534.

Pain-measurement tools in sickle cell disease: where are we now?

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Division of Hematology, Center for Cancer and Blood Disorders, Children's National Health System, Washington, DC.
Department of Pediatrics, School of Medicine and Health Sciences, The George Washington University, Washington, DC.
Section of Pediatric Hematology/Oncology, Medical College of Wisconsin, Milwaukee, WI; and.
Children's Research Institute, Children's Hospital of Wisconsin, Milwaukee, WI.


Pain is a complex multidimensional experience and the most common morbidity in patients with sickle cell disease (SCD). Tools to assess pain can be of use not only to guide pain treatment but also to provide insight into underlying pain neurobiology. Mechanisms of pain in SCD are multifactorial and are not completely elucidated. Although vaso-occlusion of microcirculation by sickled red cells is believed to be the underlying mechanism of acute vaso-occlusive pain, mechanisms for chronic pain and the transition from acute to chronic pain are under investigation. A number of modalities can be used in clinical practice and/or research to capture various dimensions of pain. Selection of a pain-assessment tool should be directed by the purpose of the assessment. Pain-assessment tools, many of which are currently in the early stages of validation, are discussed here. Development and validation of these multimodal tools is crucial for developing improved understanding of SCD pain and its management.

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