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

Overview

Journal Paediatr Drugs

Specialties Pediatrics
Pharmacology

Date 2017 Aug 31

PMID 28853040

Citations 10

Authors

Latika Puri

Kerri A Nottage

Jane S Hankins

Doralina L Anghelescu

Affiliations

Soon will be listed here.

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.

Citing Articles

Beyond the traditional oncology patient: the role of palliative care in patients with sickle cell disease receiving stem cell transplantation or gene therapy.

Collins G, Levine D, Leonard A, Sharma A, Johnson L Front Oncol. 2025; 15:1535851.

PMID: 40018407 PMC: 11865843. DOI: 10.3389/fonc.2025.1535851.

Advancing life: innovative approaches to enhance survival in sickle cell anemia patients.

Obeagu E, Adias T, Obeagu G Ann Med Surg (Lond). 2024; 86(10):6021-6036.

PMID: 39359845 PMC: 11444627. DOI: 10.1097/MS9.0000000000002534.

Sickle cell disease iPSC-derived sensory neurons exhibit increased excitability and sensitization to patient plasma.

Allison R, Welby E, Ehlers V, Burand A, Isaeva O, Nieves Torres D Blood. 2024; 143(20):2037-2052.

PMID: 38427938 PMC: 11143522. DOI: 10.1182/blood.2023022591.

A comparison of the mixed and sequential use of acetaminophen and dexketoprofen in painful vaso-occlusive crises.

Kacmaz M, Ilhan G Eur J Clin Pharmacol. 2024; 80(3):475-480.

PMID: 38245872 DOI: 10.1007/s00228-024-03630-8.

Pain Control for Sickle Cell Crisis, a Novel Approach? A Retrospective Study.

Rolle A, Vidal E, Laguette P, Garnier Y, Delta D, Martino F Medicina (Kaunas). 2023; 59(12).

PMID: 38138299 PMC: 10744599. DOI: 10.3390/medicina59122196.

References

Goodman E . Use of ketorolac in sickle-cell disease and vaso-occlusive crisis. Lancet. 1991; 338(8767):641-2. DOI: 10.1016/0140-6736(91)90656-a. View

Glassberg J . Evidence-based management of sickle cell disease in the emergency department. Emerg Med Pract. 2011; 13(8):1-20. View

Hoppe C, Jacob E, Styles L, Kuypers F, Larkin S, Vichinsky E . Simvastatin reduces vaso-occlusive pain in sickle cell anaemia: a pilot efficacy trial. Br J Haematol. 2017; 177(4):620-629. PMC: 5435522. DOI: 10.1111/bjh.14580. View

van Beers E, Van Tuijn C, Nieuwkerk P, Friederich P, Vranken J, Biemond B . Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol. 2007; 82(11):955-60. DOI: 10.1002/ajh.20944. View

Hankins J, Ware R, Rogers Z, Wynn L, Lane P, Scott J . Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood. 2005; 106(7):2269-75. PMC: 1895275. DOI: 10.1182/Blood-2004-12-4973. View

Erhan E, Inal M, Aydinok Y, Balkan C, Yegul I . Tramadol infusion for the pain management in sickle cell disease: a case report. Paediatr Anaesth. 2006; 17(1):84-6. DOI: 10.1111/j.1460-9592.2006.02030.x. View

Galeotti C, Courtois E, Carbajal R . How French paediatric emergency departments manage painful vaso-occlusive episodes in sickle cell disease patients. Acta Paediatr. 2014; 103(12):e548-54. DOI: 10.1111/apa.12773. View

Voskaridou E, Christoulas D, Bilalis A, Plata E, Varvagiannis K, Stamatopoulos G . The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2009; 115(12):2354-63. DOI: 10.1182/blood-2009-05-221333. View

Okomo U, Meremikwu M . Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database Syst Rev. 2015; (3):CD005406. DOI: 10.1002/14651858.CD005406.pub4. View

10.

Browne P, Shalev O, Hebbel R . The molecular pathobiology of cell membrane iron: the sickle red cell as a model. Free Radic Biol Med. 1998; 24(6):1040-8. DOI: 10.1016/s0891-5849(97)00391-2. View

11.

Peters-Lawrence M, Bell M, Hsu L, Osunkwo I, Seaman P, Blackwood M . Clinical trial implementation and recruitment: lessons learned from the early closure of a randomized clinical trial. Contemp Clin Trials. 2011; 33(2):291-7. PMC: 3577351. DOI: 10.1016/j.cct.2011.11.018. View

12.

Foster D, Upton R, Christrup L, Popper L . Pharmacokinetics and pharmacodynamics of intranasal versus intravenous fentanyl in patients with pain after oral surgery. Ann Pharmacother. 2008; 42(10):1380-7. DOI: 10.1345/aph.1L168. View

13.

Ballas S, Lyon D . Safety and efficacy of blood exchange transfusion for priapism complicating sickle cell disease. J Clin Apher. 2015; 31(1):5-10. DOI: 10.1002/jca.21394. View

14.

Ho K, Gan T, Habib A . Gabapentin and postoperative pain--a systematic review of randomized controlled trials. Pain. 2006; 126(1-3):91-101. DOI: 10.1016/j.pain.2006.06.018. View

15.

Telen M, Batchvarova M, Shan S, Bovee-Geurts P, Zennadi R, Leitgeb A . Sevuparin binds to multiple adhesive ligands and reduces sickle red blood cell-induced vaso-occlusion. Br J Haematol. 2016; 175(5):935-948. DOI: 10.1111/bjh.14303. View

16.

Miller S, Wright E, Abboud M, Berman B, Files B, Scher C . Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. J Pediatr. 2001; 139(6):785-9. DOI: 10.1067/mpd.2001.119593. View

17.

Hardwick Jr W, Givens T, Monroe K, King W, Lawley D . Effect of ketorolac in pediatric sickle cell vaso-occlusive pain crisis. Pediatr Emerg Care. 1999; 15(3):179-82. DOI: 10.1097/00006565-199906000-00004. View

18.

El Nemer W, Gauthier E, Wautier M, Rahuel C, Gane P, Galacteros F . Role of Lu/BCAM in abnormal adhesion of sickle red blood cells to vascular endothelium. Transfus Clin Biol. 2008; 15(1-2):29-33. DOI: 10.1016/j.tracli.2008.05.002. View

19.

Brookoff D, Polomano R . Treating sickle cell pain like cancer pain. Ann Intern Med. 1992; 116(5):364-8. DOI: 10.7326/0003-4819-116-5-364. View

20.

ROWBOTHAM M, Harden N, Stacey B, Bernstein P . Gabapentin for the treatment of postherpetic neuralgia: a randomized controlled trial. JAMA. 1998; 280(21):1837-42. DOI: 10.1001/jama.280.21.1837. View