SCD & VOC Burden

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The Burden of
Sickle Cell Disease

A Silent Process With Widespread Impact

Ongoing vaso-occlusion can culminate in vaso-occlusive crises (VOCs)—the clinical hallmark of sickle cell disease (SCD). VOCs are unpredictable and extremely painful events that can lead to medical intervention. Over time, the physical burden of VOCs often negatively affects patients’ physical, cognitive, emotional, and social well-being, and can be associated with an increased risk of organ damage and death.1-6

Increased Mortality

Historically, sickle cell disease was viewed as a pediatric condition with a life expectancy in the United States of just 14 years. Today, as a result of advances in screening and interventions, people with sickle cell disease typically live into adulthood; the average life span in the United States, however, is still 30 years less than the life span of the general population. Today, the majority of patients with SCD are adults.7-8

 

Life expectancy of patients with sickle cell disease

 

Reduced life expectancy may be attributed to ongoing silent vaso-occlusion.1 Ongoing vaso-occlusion can culminate in vaso-occulsive crises—the clinical hallmark of sickle cell disease—which are unpredictable and extremely painful events that can lead to medical intervention.2,9 VOCs can impact patient quality of life and may increase the risk of organ damage and may be associated with higher mortality.10

 

Recurrent vaso-occlusive crises in sickle cell disease patients

 

Survival data obtained for 542 adult subjects enrolled at 3 university hospitals in the southeastern United States, followed for a median of 9.3 years.10

  • VOCs were defined as crises requiring inpatient admission
  • Median survival was 61 years for patients with 0-4 VOCs per year and 53 years for patients with >4 VOCs per year. Hazard ratio was 3.6982 (P<.0001)

Extensive Physical Impact

Vaso-occlusion and its resulting vaso-occlusive crises may account for a majority of the burden of sickle cell disease. Painful vaso-occlusive crises are the hallmark of sickle cell disease. The majority of these events occur at home and are not reported.2,11,12

 

The self-perpetuating cycle of vaso-occlusion and VOCs may account for the majority of the burden of SCD.10,13-15

Effect of vaso-occlusive crisis on central nervous system

Among patients with sickle cell disease, chronic complications can include:

  • Pain is the number one cause of hospital admissions2
  • Cerebrovascular accident
    • Transient ischemic attack occurs in ~4%10
    • Overt stroke occurs in ~16%10
    • Silent cerebral infarction occurs in ~20% of children4
    • Screen for vascular disease and stroke starting at age 2 years and continuing until at least age 16 years16
  • Retinopathy occurs in ~23%10
  • Seizures occur in ~12%10

Musculoskeletal effects from vaso-occlusive crisis

Among patients with sickle cell disease, chronic complications can include:

  • Bone pain crisis is the most common form of vaso-occlusive pain, and can lead to bone or bone marrow infarction and osteonecrosis17,18

Cardiopulmonary effects from vaso-occlusive crisis

Among patients with sickle cell disease, chronic complications can include:

  • Acute chest syndrome (ACS) occurs in ~75%10
  • Pulmonary hypertension occurs in ~20%4
  • Left-sided heart disease occurs in ~13%4

Reticuloendothelial effects from vaso-occlusive crisis

Among patients with sickle cell disease, chronic complications can include:

  • Splenic sequestration occurs in ~50% of patients who survive their first episode18
  • Splenic sequestration can worsen anemia19

Gastrointestinal and urogenital effects from vaso-occlusive
crisis

Among patients with sickle cell disease, chronic complications can include:

  • Gastrointestinal complications16
    • Cholelithiasis, acute cholecystitis, biliary sludge, and acute choledocholithiasis are common
    • Gallstones occur in ~75%
  • Urogenital complications
    • Renal dysfunction begins at an early age, and ~30% experience chronic renal failure4
    • Priapism occurs in ~35% of men20

Cognitive and Emotional Burden

Ongoing silent vaso-occlusion may lead to central nervous system complications that can impair cognition and mood.4,21


Patients with sickle cell disease who have depression

Patients with SCD may need counseling services from mental health professionals.

 

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Social Burden

The complications of vaso-occlusion impact both patients with sickle cell disease and their caregivers. Vaso-occlusive crises and emergency room visits may cause children, adults, and caregivers to miss school, work, or other activities.6,24



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. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647-3656. 3. Ballas SK, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol. 2005;79(1):17-25. 4. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031. 5. Adam SS, Flahiff CM, Kamble S, et al. Depression, quality of life, and medical resource utilization in sickle cell disease. Blood Adv. 2017;1(23):1983-1992. 6. Swanson ME, Grosse SD, Kulkarni R. Disability among individuals with sickle cell disease. Am J Prev Med. 2011;41(6S4):S390-S397. 7. Niraimathi M, Kar R, Jacob SE, Basu D. Sudden death in sickle cell anaemia: report of three cases with brief review of literature. Indian J Hematol Blood Transfus. 2016;32 (Suppl 1):S258-S261. 8. Thein MS, Igbineweka NE, Thein SL. Sickle cell disease in the older adult. Pathology. 2017;49(1):1-9. 9. 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. 10. Elmariah H, Garrett ME, De Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell disease cohort. Am J Hematol. 2014;89(5):530-535. 11. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-1573. 12. Smith WR, Penberthy LT, Bovbjerg VE. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med. 2008;148:94-101. 13. Belcher JD, Mahaseth H, Welch TE, et al. Critical role of endothelial cell activation in hypoxia-induced vasoocclusion in transgenic sickle mice. Am J Physiol Heart Circ Physiol. 2005;288:H2715-H2725. 14. Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore). 2005;84(6):363-376. 15. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639-1644. 16. Yawn BP, John-Sowah J. Management of sickle cell disease: recommendations from the 2014 expert panel report. Am Fam Physician. 2015;92(12):1069-1076. 17. Alagbe AE, Olaniyi JA, Aworanti OW. Adult sickle cell anaemia patients in bone pain crisis have elevated pro-inflammatory cytokines. Mediterr J Hematol Infect Dis. 2018;10(1):e2018017. doi: 10.4084/MJHID.2018.017. 18. National Institutes of Health. National Heart, Lung, and Blood Institute. The Management of Sickle Cell Disease, 4 ed. NIH Publication No. 02-2117. Available at: http://purl.access.gpo.gov/GPO/LPS22097. 19. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323. 20. Adeyoju AB, Olujohungbe ABK, Morris J. Priapism in sickle-cell disease; incidence, risk factors and complications–an international multicentre study. BJU Int. 2012;90(9):898-902. 21. Hasan SP, Hashmi S, Alhassen M, Lawson W, Castro O. Depression in sickle cell disease. J Natl Med Assoc. 2003;95(7):533-538. 22. Castro IP, Viana MB. Cognitive profile of children with sickle cell anemia compared to healthy controls. J Pediatr (Rio J). 2018;1:1-7. doi: 10.1016/j.jped.2018.04.012. 23. Anie KA, Egunjobi FE, Akinyanju OO. Psychosocial impact of sickle cell disorder: perspectives from a Nigerian setting. Global Health. 2010;6:2. doi: 10.1186/1744-8603-6-2. 24. Brandow AM, Brousseau DC, Panepinto JA. Post-discharge pain, functional limitations, and impact on caregivers of children with sickle cell disease treated for painful events. Br J Haematol. 2009;144(5):782-788.

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