When Managing SCD, Some VOCs/Pain Crises...


Require Health Care Visits
~3 VOCs

But Most Are

Managed at Home


Many Severe Ones

Often Intentionally

Avoiding the ER
On-going, silent vaso-occlusion is the cause of every vaso-occlusive crisis (VOC)
  • VOCs, also referred to as pain crises, are the clinical hallmark of SCD
  • There is no standard definition of a VOC or measurable biomarkers
  • VOCs are generally defined as episodes of vaso-occlusion characterized by extreme pain that can last up to 10 days

Although vaso-occlusion may not always result in a VOC, every VOC is caused by vaso-occlusion3,5,6

Subclinical vaso-occlusion and VOCs leading to increased inflammation, vascular and organ damage over patient's lifetime



*Based on a Medicaid Analytic extracts database of 8521 adult patients with SCD using claims from July 1, 2009, to December 31, 2012.1

Based on a one-time survey study that evaluated pain interference, quality of health care, and self-efficacy of 440 adults and adolescents (aged 15 to 50 years) with SCD of all genotypes. The survey also assessed how these variables were associated with participants' perceptions of outpatient and ER care.3

Participants in the study indicated how much pain interfered with daily activities in the previous 7 days, including work around the home, social activities, and household chores. Items were scored on a 5-point Likert scale. Scores included a T score with a mean (SD) of 50 (10). The T score was used for the analysis, with higher scores indicating more pain interference.3



1. Shah N, Bhor M, Xie L, et al. Sickle cell disease complications: prevalence and resource utilization. PLoS ONE. 2019;14(70;1-12. 2. Smith WR, Pemberthy LT, Bovbjerg VE, et al. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med. 2008;148(2):94-101. 3. Kanter J, Gibson R, Lawrence RH, et al. Perceptions of US adolescents and adults with sickle cell disease on their quality of care. JAMA Network Open. 2020 May 1;3(5):e206016. doi.10.1001/jamanetworkopen.2020.6016. 4. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647-3656. 5. Brandow AM, Zappia KJ, Stucky CL. Sickle cell disease: a natural model of acute and chronic pain. Pain. 2017;158(Suppl 1):S79-S84. doi.10.1097/j. pain.0000000000000824. 6. Conran N, Franco-Penteado CF, Costa FF. Newer aspects of the pathophysiology of sickle cell disease vaso-occlusion. Hemoglobin. 2009;33(1):1-16. 7. Puri L, Nottage KA, Hankins JS, Anghelescu DL. State of the art management of acute vaso-occlusive pain in sickle cell disease. Paediatr Drugs. 2018;20(1):29-42. 8. Zhang D, Xu C, Manwani D, Frenette PS. Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology. Blood. 2016;127(7):801-809. 9. Ansari J, Gavins F. Ischemia-reperfusion injury in sickle cell disease: from basics to therapeutics. Am J Pathol. 2019;189(4):706-718. 

Use of website is governed by the Terms of Use and Privacy Policy.

Copyright © 2021 Novartis Pharmaceuticals Corporation. All rights reserved.

1/21 SDC-1236447