Sickle cell disease can have a negative effect on both society and individuals^1-3

Increased hospitalisation and cost of care

In England, sickle cell disease (SCD) is one of the most common reasons for hospital admission, with multiple admissions for individual patients⁴

One study found that over a 30-month period:

One third of the patients accounted for

70% of hospitalisations due to a vaso-occlusive crisis (VOC)

One study found that over a 30-month period:

One third of the patients accounted for

70% of hospitalisations due to a (VOC)

In the United States, VOCs are the primary reason for emergency department (ED) visits and hospital admissions amongst patients with SCD

197,000 ED visitsED visits

due to VOCs per year, averaging nearly 2 per patient¹

The ED Rate is:

4 times

Higher than for those with

congestive heart failure²

13 times

Higher than for those with

HIV²

SCD–related complications result in increased cost of care

Sickle Cell Disease Treatment in the United Kingdom⁵

are ~18 times higher for patients with SCD who experience a VOC and comorbidities compared with patients with SCD without these complications

Sickle Cell Disease Treatment Costs in the United States^6,7

~$1 million

Lifetime health care costs

for a 45-year-old with SCD

>$30,000

annual cost for adults

~$1 billion

Total annual health care costs

for all patients with SCD*

*In 2009. Today, the estimated annual amount may be higher due to increases in prevalence and treatment costs.

Sickle cell disease is associated with impaired cognition and psychological disorders⁸

IMPAIRED COGNITION

In Brazil,

~20%

lower IQ scores have been reported in patients with SCD (eg, verbal and performance)^9*

DEPRESSION

In the United States,

35%

of patients with SCD are diagnosed with depression^10†

ANXIETY

In Nigeria,

13%

of adults with SCD experience anxiety^11‡

*Comparing 64 patients with sickle cell anaemia and 64 subjects without SCD (control group).
^†A total of 142 patients completed the study.
^‡Based on 253 patients with SCD.

Other cognitive and psychological effects of sickle cell disease

Cognitive^12,13

Overt and/or silent stroke may affect cognitive function, eg,
- executive skills
- attention span
- memory impairment
- communication skills

Psychological^13,14

SCD-associated stigma
Feeling of worthlessness
Post-traumatic stress disorder

Sickle cell disease can have a negative impact on employment, relationships, and education^15,16

ADULT PATIENTS

In the United Kingdom,

<50%

are employed and of those, 17% of their missed workdays are caused by VOCs¹⁵

In Brazil,

65%

of men and women report impaired sexuality, and 92% of them report that VOCs are the main cause¹⁷

CAREGIVERS

In the United States,

24%

miss 2 or more days of school or work every time their loved one is treated at a hospital¹⁶

Other social and developmental effects of sickle cell disease

Social relationships¹³

Loss of social connections because of long stays at home or in the hospital
Feeling of not fitting in or belonging
Social isolation due to debilitating effects of disease progression
Inability to engage in social outings or travel due to VOCs

Education and employment^12,13,18

Missed school days
Uncompleted education
Diminished or lost employment opportunities
Low or disability income

References: 1. Lentz MB, Kautz DD. Acute vaso-occlusive crisis in patients with sickle cell disease. Nursing2018. 2017;41(1):67-68. 2. 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. 3. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647-3656. 4. Green SA, Aljuburi G, Majeed A, et al. Characterizing emergency admissions of patients with sickle cell crisis in NHS Brent: observational study. J R Soc Med Sh Rep. 2012; 3(37):1-7. 5. Centre for Clinical Practice at NICE (UK). Sickle Cell Acute Painful Episode: Management of an Acute Painful Sickle Cell Episode in Hospital. Manchester (UK): National Institute for Health and Clinical Excellence (UK); 2012 Jun. (NICE Clinical Guidelines, No. 143.) Appendix F, Full health economic report. Available at: https://www.ncbi.nlm.nih.gov/books/NBK126749/. Accessed March 26, 2019. 6. Adams-Graves P, Bronte-Jordan L. Recent treatment guidelines for managing adult patients with sickle cell disease: challenges in access to care, social issues, and adherence. Expert Rev Hematol. 2016;9(6):541-542. 8. Hasan S, Hashmi S, Alhassen M, Lawson W, Castro O. Depression in sickle cell disease. J Natl Med Assoc. 2003;95(5):533-538. 7. American Society of Hematology. State of Sickle Cell Disease: 2016 Report. Washington, DC: 2016. http://www.scdcoalition.org/report.html. 9. 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 10. 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. 11. 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. 12. Kassim AA, Galadanci NA, Pruthi S, DeBaun MR. How I treat and manage strokes in sickle cell disease. Blood 2015.125(22):3401-3410. 13. Chakravorty S, Tallett A, Witwicki C, et al. Patient-reported experience measure in sickle cell disease. Arch Dis Child. 2018;103(12):1104-1109. 14. Alao AO. Sickle cell disease and posttraumatic stress disorder. Int J Psychiatry Med. 2002;32(1):97-101. 15. Swanson ME, Grosse SD, Kulkarni R. Disability among individuals with sickle cell disease. Am J Prev Med. 2011;41(6S4):S390-S397. 16. 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. 17. 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. 18. Brandow AM, Panepinto JA. Hydroxyurea use in sickle cell disease: the battle with low prescription rates, poor patient compliance and fears of toxicities. Exp Rev Hematol. 2010; 3(3):255-260.

Sickle cell disease can have a negative effect on both society and individuals1-3

Increased hospitalisation and cost of care

In England, sickle cell disease (SCD) is one of the most common reasons for hospital admission, with multiple admissions for individual patients4

In the United States, VOCs are the primary reason for emergency department (ED) visits and hospital admissions amongst patients with SCD

SCD–related complications result in increased cost of care

Sickle cell disease is associated with impaired cognition and psychological disorders8

Other cognitive and psychological effects of sickle cell disease

Cognitive12,13

Psychological13,14

Sickle cell disease can have a negative impact on employment, relationships, and education15,16