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State of the Art Management of Acute Vaso-occlusive Pain in Sickle Cell Disease

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Abstract

Acute vaso-occlusive crisis (VOC) is a hallmark of sickle cell disease (SCD). Multiple complex pathophysiological processes can result in pain during a VOC. Despite significant improvements in the understanding and management of SCD, little progress has been made in the management of pain in SCD, although new treatments are being explored. Opioids and non-steroidal anti-inflammatory drugs (NSAIDs) remain the mainstay of treatment of VOC pain, but new classes of drugs are being tested to prevent and treat acute pain. Advancements in the understanding of the pathophysiology of SCD and pain and the pharmacogenomics of opioids have yet to be effectively utilized in the management of VOC. Opioid tolerance and opioid-induced hyperalgesia are significant problems associated with the long-term use of opioids, and better strategies for chronic pain therapy are needed. This report reviews the mechanisms of pain associated with acute VOC, describes the current management of VOC, and describes some of the new therapies under evaluation for the management of acute VOC in SCD.

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Acknowledgements

The authors would like to thank Brandon Stelter, Sr. Graphic Designer, St. Jude Biomedical Communications, for preparation of the figures.

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Authors and Affiliations

  1. Department of Hematology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Latika Puri & Jane S. Hankins

  2. Janssen Research and Development, Raritan, NJ, USA

    Kerri A. Nottage

  3. Division of Anesthesia, Department of Pediatric Medicine, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, MS 130, Memphis, TN, 38105-3678, USA

    Doralina L. Anghelescu

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  1. Latika Puri
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  2. Kerri A. Nottage
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  3. Jane S. Hankins
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  4. Doralina L. Anghelescu
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Correspondence to Doralina L. Anghelescu.

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Latika Puri, Jane S. Hankins, and Doralina L. Anghelescu have no conflicts of interest. Kerri A. Nottage is employed by Janssen Research and Development, Raritan, NJ, USA.

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Puri, L., Nottage, K.A., Hankins, J.S. et al. State of the Art Management of Acute Vaso-occlusive Pain in Sickle Cell Disease. Pediatr Drugs 20, 29–42 (2018). https://doi.org/10.1007/s40272-017-0263-z

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