For US health care professionals only.

The Burden of SCD

Sickle cell disease (SCD) can affect many areas, including daily life, and some of the effects can be lifelong

The Burden of SCD

Sickle cell disease (SCD) can affect many areas, including daily life, and some of the effects can be lifelong

The Burden of SCD

Sickle cell disease (SCD) can affect many areas, including daily life, and some of the effects can be lifelong

In SCD, VOCs/Pain Crises Are the...

Main Cause of ER Visits

~197,000 ER visits per year, averaging nearly 2 visits per patient¹

4 times higher than for those with congestive heart disease²

13 times higher than for those with HIV²

VOCs are the reason for >70% of ER visits for adults with SCD, despite the majority being managed at home^2,3

and Hospital Admissions

~95% of hospitalizations for SCD are due to VOCs⁴

50% of patients discharged prematurely while still experiencing severe pain⁴*

~50% risk of rehospitalization within 30 days for patients with SCD⁴

Frequent and prolonged hospitalizations disrupt daily life and are associated with increased risk of mortality

*Severe pain was defined as pain with an intensity, score ≥6.0. The mean pain score on discharge was in the same range (>6), suggesting premature discharge, with 50% of the patients having a pain score of 7.0.⁴

SCD, sickle cell disease; VOCs, vaso-occlusive crises; ER, emergency room.

SCD May Also Impact Patients in Many Other Ways, Causing Problems With...

Executive Function

SCD may impact areas of the brain involved in response inhibition and higher-order executive function^5,6

Younger patients without a history of stroke have shown reductions in concentration, attention span, visual-spatial working memory, initiation, and organization
~20% lower IQ scores have been reported in patients with SCD due to disease-related complications, including stroke and silent cerebral infarct*

Depression

35% of patients with SCD are diagnosed with depression^{7,8, †}

A higher number of VOCs is associated with depression^‡

Anxiety

13% of adult patients with SCD experience anxiety^8-10

VOCs are associated with a poor overall quality of life, including increased risk of anxiety

The Burden of SCD Can Affect Many Aspects of Daily Life, Such as...

Work

< 50% of patients with SCD are employed^11,12

Of those, 17% of their missed work days are caused by VOCs
24% of caregivers miss 2 or more days of work or school every time their loved one is treated at a hospital

School

~18.2 school days/year are missed by students with SCD, on average, accounting for ~10% of the school year¹¹

Often due to challenges in managing the frequency of VOC-related hospitalizations

Relationships

65% of patients with SCD report impaired sexual function^13,14,¶

Of those individuals, 92% report VOCs as the main cause
Patients also report inability to engage in social outings or travel due to VOCs

*Eg, verbal and performance. Comparing 64 patients with sickle cell anemia and 64 subjects without SCD (control group).
^†A total of 142 patients completed the study.
^‡20 youth ranging in age from 8 to 16 years with SCD without known prior neurologic injury participated in the study.
^§Based on 253 adult patients with SCD.
¶Based on a descriptive study of 20 adult patients with SCD (HbSS).¹³

The Impact of VO and VOCs

References

1. Lanzkron S, Carroll CP, Haywood C. The burden of emergency department use for sickle cell disease: an analysis of the national emergency department sample database. Am J Hematol. 2010;85(10):797-799. 2. Yusuf HR, Atrash HK, Grosse SD, Parker CS, Grant AM. Emergency department visits made by patients with sickle cell disease: a descriptive study, 1999-2007. Am J Prev Med. 2010;38(4 Suppl):S536-S541. 3. 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. 4. 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. 5. Daly BP, Kral MC, Brown RT. Cognitive and academic problems associated with childhood cancers and sickle cell disease. School Psychology Quarterly. 2008;23(2):230-242. 6. Castro IPS, Viana MB. Cognitive profile of children with sickle cell anemia compared to healthy controls. J Pediatr (Rio J). 2017;1-7. doi.org/10.1016/j.jped.2018.04.012. 7. 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. 8. Chetcha Chemegni B, Kamga Olen JPO, Um NYobe LJ, et al. Anxiety, depression and quality of life in adults with sickle cell disease. Arch Med. 2018;10(1):1-7. 9. 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. 10. Wallen G, Minnti C, Krumlauf M, et al. Sleep disturbance, depression and pain in adults with sickle cell disease. BMC Psychiatry. 2014;14:207. doi.10.1186/1471-244X-14-207. 11. Swanson ME, Grosse SD, Kulkarni R. Disability among individuals with sickle cell disease. Am J Prev Med. 2011;41(6S4):S390-S397. 12. 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. 13. Côbo Vde A, Chapadeiro CA, Ribeiro JB, Moraes-Souza H, Martins PR. Sexuality and sickle cell anemia. Rev Bras Hematol Hemoter. 2013;35(2):89-93. 14. Chakravorty S, Tallett A, Witwicki C, et al. Patient-reported experience measure in sickle cell disease. Arch Dis Child. 2018;103(12):1104-1109.