Neuropathic Pain and Sickle Cell Disease: a Review of Pharmacologic Management
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OTHER PAIN (N VADIVELU AND AD KAYE, SECTION EDITORS)
Neuropathic Pain and Sickle Cell Disease: a Review of Pharmacologic Management Mariam Salisu Orhurhu 1 & Robert Chu 2 & Lauren Claus 2 & Jacob Roberts 2 & Bisi Salisu 3 & Ivan Urits 4 & Ejovwoke Orhurhu 5 & Omar Viswanath 6,7,8,9 & Alan D. Kaye 6,7,8,9 & Aaron J. Kaye 10 & Vwaire Orhurhu 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Sickle cell disease (SCD) remains among the most common and severe monogenic disorders present in the world today. Although sickle cell pain has been traditionally characterized as nociceptive, a significant portion of sickle cell patients has reported neuropathic pain symptoms. Our review article will discuss clinical aspects of SCD-related neuropathic pain, epidemiology of neuropathic pain among individuals with SCD, pain mechanisms, and current and future potential pharmacological interventions. Recent Findings Neuropathic pain in SCD is a complicated condition that often has a lifelong and significant negative impact on life; therefore, improved pain management is considered a significant and unmet need. Neuropathic pain mechanisms are heterogeneous, and the difficulty in determining their individual contribution to specific pain types may contribute to poor treatment outcomes in this population. Summary Our review article outlines several pharmacological modalities which may be employed to treat neuropathic pain in SCD patients. Keywords Neuropathic pain . Sickle cell disease . Chronic pain . Pharmacologic management
Introduction Sickle cell disease (SCD) remains among the most common and severe monogenic disorders present in the world today. Caused by a variant of the beta-globin gene called sickle hemoglobin (Hb S), SCD is inherited in an autosomal recessive pattern. As a result, SCD
equally affects both males and females [1••]. Disease expression requires the presence of either two copies of Hb S or one copy of Hb S with another betaglobin variant [1••]. Carriers of Hb S are protected from malaria infection, and this protection has potentially contributed to the high frequency of Hb S in individuals of African and Mediterranean ancestry [1••]. However,
This article is part of the Topical Collection on Other Pain * Vwaire Orhurhu [email protected] 1
Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
2
Johns Hopkins School of Medicine, Baltimore, MD, USA
3
Meharry Medical College, Nashville, TN, USA
4
Department of Anesthesia, Critical Care, and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave, Boston, MA 02215, USA
5
Massachusetts College of Pharmacy and Health Sciences, Boston, MA, USA
6
Valley Anesthesiology and Pain Consultants, Phoenix, AZ, USA
7
Department of Anesthesiology, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA
8
Department of Anesthesiology, Creighton University School of Medicine, Omaha, N
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