Skip to main content
Top
Published in: Advances in Therapy 8/2023

Open Access 18-06-2023 | Original Research

Economic and Clinical Burden of Managing Sickle Cell Disease with Recurrent Vaso-Occlusive Crises in the United States

Authors: Chuka Udeze, Kristin A. Evans, Yoojung Yang, Timothy Lillehaugen, Janna Manjelievskaia, Urvi Mujumdar, Nanxin Li, Biree Andemariam

Published in: Advances in Therapy | Issue 8/2023

Login to get access

Abstract

Introduction

The aim of this study was to describe the clinical complications, treatment use, healthcare resource utilization (HCRU), and costs among patients with sickle cell disease (SCD) with recurrent vaso-occlusive crises (VOCs) in the US.

Methods

Merative MarketScan Databases were used to identify patients with SCD with recurrent VOCs from March 1, 2010, to March 1, 2019. Inclusion criteria were ≥ 1 inpatient or  ≥ 2 outpatient claims for SCD and ≥ 2 VOCs per year in any 2 consecutive years after the first qualifying SCD diagnosis. Individuals without SCD in these databases were used as matched controls. Patients were followed for ≥ 12 months, from their second VOC in the 2nd year (index date) to the earliest of inpatient death, end of continuous enrollment in medical/pharmacy benefits, or March 1, 2020. Outcomes were assessed during follow-up.

Results

In total, 3420 patients with SCD with recurrent VOCs and 16,722 matched controls were identified. Patients with SCD with recurrent VOCs had a mean of 5.0 VOCs (standard deviation [SD] = 6.0), 2.7 inpatient admissions (SD 2.9), and 5.0 emergency department visits (SD 8.0) per patient per year during follow-up. Compared to matched controls, patients with SCD with recurrent VOCs incurred higher annual ($67,282 vs. $4134) and lifetime ($3.8 million vs. $229,000 over 50 years) healthcare costs.

Conclusion

Patients with SCD with recurrent VOCs experience substantial clinical and economic burden driven by inpatient costs and frequent VOCs. There is a major unmet need for treatments that alleviate or eliminate clinical complications, including VOCs, and reduce healthcare costs in this patient population.
Appendix
Available only for authorised users
Literature
1.
go back to reference Hassell KL. Population estimates of sickle cell disease in the US. Am J Prev Med. 2010;38:S512–21. CrossRefPubMed Hassell KL. Population estimates of sickle cell disease in the US. Am J Prev Med. 2010;38:S512–21. CrossRefPubMed
2.
3.
go back to reference Finch JT, Perutz MF, Bertles JF, Dobler J. Structure of sickled erythrocytes and of sickle-cell hemoglobin fibers. Proc Natl Acad Sci USA. 1973;70:718–22. CrossRefPubMedPubMedCentral Finch JT, Perutz MF, Bertles JF, Dobler J. Structure of sickled erythrocytes and of sickle-cell hemoglobin fibers. Proc Natl Acad Sci USA. 1973;70:718–22. CrossRefPubMedPubMedCentral
4.
go back to reference Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019;14:263–92. CrossRefPubMed Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019;14:263–92. CrossRefPubMed
5.
go back to reference Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010. CrossRefPubMed Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010. CrossRefPubMed
6.
go back to reference Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390:311–23. CrossRefPubMed Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390:311–23. CrossRefPubMed
7.
go back to reference Darbari DS, Sheehan VA, Ballas SK. The vaso-occlusive pain crisis in sickle cell disease: definition, pathophysiology, and management. Eur J Haematol. 2020;105:237–46. CrossRefPubMed Darbari DS, Sheehan VA, Ballas SK. The vaso-occlusive pain crisis in sickle cell disease: definition, pathophysiology, and management. Eur J Haematol. 2020;105:237–46. CrossRefPubMed
8.
go back to reference Evensen CT, Treadwell MJ, Keller S, Levine R, Hassell KL, Werner EM, et al. Quality of care in sickle cell disease: cross-sectional study and development of a measure for adults reporting on ambulatory and emergency department care. Medicine (Baltimore). 2016;95: e4528. CrossRefPubMed Evensen CT, Treadwell MJ, Keller S, Levine R, Hassell KL, Werner EM, et al. Quality of care in sickle cell disease: cross-sectional study and development of a measure for adults reporting on ambulatory and emergency department care. Medicine (Baltimore). 2016;95: e4528. CrossRefPubMed
9.
go back to reference Zaidi AU, Glaros AK, Lee S, Wang T, Bhojwani R, Morris E, et al. A systematic literature review of frequency of vaso-occlusive crises in sickle cell disease. Orphanet J Rare Dis. 2021;16:460. CrossRefPubMedPubMedCentral Zaidi AU, Glaros AK, Lee S, Wang T, Bhojwani R, Morris E, et al. A systematic literature review of frequency of vaso-occlusive crises in sickle cell disease. Orphanet J Rare Dis. 2021;16:460. CrossRefPubMedPubMedCentral
10.
go back to reference 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:S536–41. CrossRefPubMedPubMedCentral 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:S536–41. CrossRefPubMedPubMedCentral
11.
go back to reference Ballas SK, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol. 2005;79:17–25. CrossRefPubMed Ballas SK, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol. 2005;79:17–25. CrossRefPubMed
12.
go back to reference Shah N, Bhor M, Xie L, Paulose J, Yuce H. Medical resource use and costs of treating sickle cell-related vaso-occlusive crisis episodes: a retrospective claims study. J Health Econ Outcomes Res. 2020;7:52–60. CrossRefPubMedPubMedCentral Shah N, Bhor M, Xie L, Paulose J, Yuce H. Medical resource use and costs of treating sickle cell-related vaso-occlusive crisis episodes: a retrospective claims study. J Health Econ Outcomes Res. 2020;7:52–60. CrossRefPubMedPubMedCentral
13.
go back to reference Johnson KM, Jiao B, Ramsey SD, Bender MA, Devine B, Basu A. Lifetime medical costs attributable to sickle cell disease among nonelderly individuals with commercial insurance. Blood Adv. 2023;7:365–74. CrossRefPubMed Johnson KM, Jiao B, Ramsey SD, Bender MA, Devine B, Basu A. Lifetime medical costs attributable to sickle cell disease among nonelderly individuals with commercial insurance. Blood Adv. 2023;7:365–74. CrossRefPubMed
14.
go back to reference Shah NR, Bhor M, Latremouille-Viau D, Kumar Sharma V, Puckrein GA, Gagnon-Sanschagrin P, et al. Vaso-occlusive crises and costs of sickle cell disease in patients with commercial, Medicaid, and Medicare insurance - the perspective of private and public payers. J Med Econ. 2020;23:1345–55. CrossRefPubMed Shah NR, Bhor M, Latremouille-Viau D, Kumar Sharma V, Puckrein GA, Gagnon-Sanschagrin P, et al. Vaso-occlusive crises and costs of sickle cell disease in patients with commercial, Medicaid, and Medicare insurance - the perspective of private and public payers. J Med Econ. 2020;23:1345–55. CrossRefPubMed
15.
go back to reference Desai RJ, Mahesri M, Globe D, Mutebi A, Bohn R, Achebe M, et al. Clinical outcomes and healthcare utilization in patients with sickle cell disease: a nationwide cohort study of Medicaid beneficiaries. Ann Hematol. 2020;99:2497–505. CrossRefPubMed Desai RJ, Mahesri M, Globe D, Mutebi A, Bohn R, Achebe M, et al. Clinical outcomes and healthcare utilization in patients with sickle cell disease: a nationwide cohort study of Medicaid beneficiaries. Ann Hematol. 2020;99:2497–505. CrossRefPubMed
16.
go back to reference Darbari DS, Wang Z, Kwak M, Hildesheim M, Nichols J, Allen D, et al. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort. PLoS ONE. 2013;5(11): e79923. CrossRef Darbari DS, Wang Z, Kwak M, Hildesheim M, Nichols J, Allen D, et al. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort. PLoS ONE. 2013;5(11): e79923. CrossRef
17.
go back to reference Tan L, Reibman J, Ambrose C, Chung Y, Desai P, Llanos JP, et al. Clinical and economic burden of uncontrolled severe noneosinophilic asthma. Am J Manag Care. 2022;28:e212–20. CrossRefPubMed Tan L, Reibman J, Ambrose C, Chung Y, Desai P, Llanos JP, et al. Clinical and economic burden of uncontrolled severe noneosinophilic asthma. Am J Manag Care. 2022;28:e212–20. CrossRefPubMed
18.
go back to reference Zeiger R, Sullivan P, Chung Y, Kreindler JL, Zimmerman NM, Tkacz J. Systemic corticosteroid-related complications and costs in adults with persistent asthma. J Allergy Clin Immunol Pract. 2020;8:3455-65.e13. CrossRefPubMed Zeiger R, Sullivan P, Chung Y, Kreindler JL, Zimmerman NM, Tkacz J. Systemic corticosteroid-related complications and costs in adults with persistent asthma. J Allergy Clin Immunol Pract. 2020;8:3455-65.e13. CrossRefPubMed
20.
go back to reference Brandow AM, Carroll CP, Creary S, Edwards-Elliott R, Glassberg J, Hurley RW, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020;4:2656–701. CrossRefPubMedPubMedCentral Brandow AM, Carroll CP, Creary S, Edwards-Elliott R, Glassberg J, Hurley RW, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020;4:2656–701. CrossRefPubMedPubMedCentral
22.
go back to reference Mvundura M, Amendah D, Kavanagh PL, Sprinz PG, Grosse SD. Health care utilization and expenditures for privately and publicly insured children with sickle cell disease in the United States. Pediatr Blood Cancer. 2009;53:642–6. CrossRefPubMed Mvundura M, Amendah D, Kavanagh PL, Sprinz PG, Grosse SD. Health care utilization and expenditures for privately and publicly insured children with sickle cell disease in the United States. Pediatr Blood Cancer. 2009;53:642–6. CrossRefPubMed
23.
go back to reference Gallagher ME, Chawla A, Brady BL, Badawy SM. Heterogeneity of the long-term economic burden of severe sickle cell disease: a 5-year longitudinal analysis. J Med Econ. 2022;25:1140–8. CrossRefPubMed Gallagher ME, Chawla A, Brady BL, Badawy SM. Heterogeneity of the long-term economic burden of severe sickle cell disease: a 5-year longitudinal analysis. J Med Econ. 2022;25:1140–8. CrossRefPubMed
26.
go back to reference Lee L, Smith-Whitley K, Banks S, Puckrein G. Reducing health care disparities in sickle cell disease: a review. Public Health Rep. 2019;134:599–607. CrossRefPubMedPubMedCentral Lee L, Smith-Whitley K, Banks S, Puckrein G. Reducing health care disparities in sickle cell disease: a review. Public Health Rep. 2019;134:599–607. CrossRefPubMedPubMedCentral
27.
go back to reference Hemker BG, Brousseau DC, Yan K, Hoffmann RG, Panepinto JA. When children with sickle-cell disease become adults: lack of outpatient care leads to increased use of the emergency department. Am J Hematol. 2011;86:863–5. CrossRefPubMed Hemker BG, Brousseau DC, Yan K, Hoffmann RG, Panepinto JA. When children with sickle-cell disease become adults: lack of outpatient care leads to increased use of the emergency department. Am J Hematol. 2011;86:863–5. CrossRefPubMed
28.
go back to reference Powers-Hays A, McGann P. When actions speak louder than words—racism and sickle cell disease. N Engl J Med. 2020;383:1902–3. CrossRef Powers-Hays A, McGann P. When actions speak louder than words—racism and sickle cell disease. N Engl J Med. 2020;383:1902–3. CrossRef
29.
go back to reference Lubeck D, Agodoa I, Bhakta N, Danese M, Pappu K, Howard R, et al. Estimated life expectancy and income of patients with sickle cell disease compared with those without sickle cell disease. JAMA Netw Open. 2019;2: e1915374. CrossRefPubMedPubMedCentral Lubeck D, Agodoa I, Bhakta N, Danese M, Pappu K, Howard R, et al. Estimated life expectancy and income of patients with sickle cell disease compared with those without sickle cell disease. JAMA Netw Open. 2019;2: e1915374. CrossRefPubMedPubMedCentral
30.
go back to reference Holdford D, Vendetti N, Sop DM, Johnson S, Smith WR. Indirect economic burden of sickle cell disease. Value Health. 2021;24:1095–101. CrossRefPubMed Holdford D, Vendetti N, Sop DM, Johnson S, Smith WR. Indirect economic burden of sickle cell disease. Value Health. 2021;24:1095–101. CrossRefPubMed
Metadata
Title
Economic and Clinical Burden of Managing Sickle Cell Disease with Recurrent Vaso-Occlusive Crises in the United States
Authors
Chuka Udeze
Kristin A. Evans
Yoojung Yang
Timothy Lillehaugen
Janna Manjelievskaia
Urvi Mujumdar
Nanxin Li
Biree Andemariam
Publication date
18-06-2023
Publisher
Springer Healthcare
Published in
Advances in Therapy / Issue 8/2023
Print ISSN: 0741-238X
Electronic ISSN: 1865-8652
DOI
https://doi.org/10.1007/s12325-023-02545-7

Other articles of this Issue 8/2023

Advances in Therapy 8/2023 Go to the issue