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Vaso-occlusion and vaso-occlusive crises can have a range of clinical complications1-4

Ongoing vaso-occlusion and recurrent vaso-occlusive crises (VOCs) are associated with decreased quality of life, end-organ damage, multiorgan failure, and death.

See how even 1 VOC per year can affect survival in patients with SCD

Take this 3D interactive tour to see how ongoing vaso-occlusion and recurrent vaso-occlusive crises can lead to end-organ damage

Vaso-occlusive crises are the major burden of sickle cell disease1

Vaso-occlusion and resulting VOCs may account for the majority of the burden of sickle cell disease (SCD).1 The majority of these events occur at home and are not reported.5,6 VOCs can affect quality of life and increase the risk of end-organ damage.1,3,4

One or more vaso-occlusive crisis requiring a health care visit in a given year has been associated with an increased risk of death at an earlier age7

Survival in sickle cell anaemia (HbSS) by severe pain crises requiring an emergency department visit/hospitalisation in past year

KM survival curve for patients with sickle cell anaemia (HbSS comparing 0 VOCs/year vs 1 VOCs/year)

This analysis included 264 adult patients with sickle cell anaemia (HbSS) followed for a median of ~5 years in the United States (The Bethesda Sickle Cell Cohort Study, 2001-2007)

  • Vaso-occlusive crises (VOCs) were defined as patient-reported emergency department visit or hospitalisation for pain treatment in the 12 months prior to enrolment
  • VOCs were associated with death at a younger age: 66.2 years for patients with 0 VOCs in the past year and 55.8 years for patients with ≥1 VOCs in the past year (P=0.04)
See how VOCs are the primary cause of hospitalisation in patients with SCD

References: 1. Conran N, Franco-Penteado CF, Costa FF. Newer aspects of the pathophysiology of sickle cell disease vaso-occlusion. Hemoglobin. 2009;33(1):1-16. 2. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031. 3. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-1573. 4. 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. 5. 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. 6. van Tuijn CF, Sins JW, Fijnvandraat K, Biemond BJ. Daily pain in adults with sickle cell disease—a different perspective. Am J Hematol. 2017;92(2):179-186. 7. Darbari DS, Wang Z, Kwak M, et al. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. PLoS One. 2013;8(11):e79923. doi: 10.1371/journal.pone.0079923.

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