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More to the Story of Sickle Cell Disease
How are patients really managing SCD? Often health care providers are not seeing the full picture. Watch to find out more.
When Managing SCD, Some VOCs/Pain Crises...
Will
Require Health Care Visits
On average, patients with SCD experience ~3 VOCs that lead to a health care visit each year1*
But Most Are
Managed at Home
Up to 79% of VOCs are managed at home2†
Even
Many Severe Ones
~76% surveyed report managing episodes of severe pain at home3‡
Often Intentionally
Avoiding the ER
~67% of those surveyed report delaying or avoiding going to the ER even when they know they should seek care
- ~50% of those surveyed report that past negative ER experiences influence their decision not to seek medical care
Vaso-Occlusion Is Ongoing and Is the Cause of Every VOC4,5
- 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-occlusion6-8
Vaso-Occlusive Damage Can Increase Over a Patient’s Lifetime, as Can VOCs6-9
VOCs are the most visible expression of vaso-occlusive damage
VOC, vaso-occlusive crisis; SCD, sickle cell disease; ER, emergency room.
*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.
