Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Jul 5;14(7):e0214355.
doi: 10.1371/journal.pone.0214355. eCollection 2019.

Sickle cell disease complications: Prevalence and resource utilization

Nirmish Shah  1 , Menaka Bhor  2 , Lin Xie  3 , Jincy Paulose  4 , Huseyin Yuce  5
Affiliations

Sickle cell disease complications: Prevalence and resource utilization

Nirmish Shah et al. PLoS One. .

Abstract

Objectives: This study evaluated the prevalence rate of vaso-occlusive crisis (VOC) episodes, rates of uncomplicated and complicated VOC episodes, and the primary reasons for emergency room (ER) visits and inpatient admissions for sickle cell disease (SCD) patients.

Methods: The Medicaid Analytic extracts database was used to identify adult SCD patients using claims from 01JUL2009-31DEC2012. The date of the first observed SCD claim was designated as the index date. Patients were required to have continuous medical and pharmacy benefits for ≥6 months baseline and ≥12 months follow-up period. Patient demographics, baseline clinical characteristics, the rate of uncomplicated and complicated VOC (VOC with concomitant SCD complications) episodes, and reasons for ER visits and inpatient stays were analyzed descriptively.

Results: A total of 8,521 patients were included in the analysis, with a median age of 30 years. The average follow-up period was 2.7 years. The rate of VOC episodes anytime in the follow-up was 3.31 in person-years. During the first-year follow-up period, an average of 2.79 VOC episodes were identified per SCD patients, with 1.06 VOC episodes treated in inpatient setting and 0.90 VOC episodes in ER without admission. A total of 76,154 VOC episodes were identified during the entire follow-up period for the overall SCD patients. Most of the VOC episodes (70.3% [n = 53,523]) were uncomplicated episodes, and 29.7% were complicated episodes. Using primary diagnosis claims only, the most frequent complications during the VOC episode were infectious diseases (25.9%), fever (21.8%), and pulmonary disorders (16.2%). Among ER and hospitalizations related to VOC or SCD complication, ~85.0% had VOCs as the primary reason for admission; 15.0% had SCD complications as the primary reason.

Conclusion: In summary, SCD and its related comorbidities and complications result in high acute health care utilization. In addition, VOC remains the primary reason for SCD patients' ER visits and inpatient admissions.

PubMed Disclaimer

Conflict of interest statement

I have read the journal's policy and the authors of this manuscript have the following competing interests: This study was funded by Novartis Pharmaceuticals Corporation which is a commercial company. The funder provided support in the form of salaries for authors JP and MB. The funders also provided consultation fees to STATinMED Research, employer of LX, which is a paid consultant in connection with this study and the development of this manuscript. The funder provided support to NS in the form of research funding, consultant fees, and speaker’s fees. HY has nothing to disclose. However, the funder did not have any additional role in the study design, data collection and analysis, decision to publish, or preparation of the manuscript. There are no patents, products in development, or marketed products associated with this research to declare. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Figures

Fig 1
Fig 1. Flow chart for patients selection criteria.
SCD: Sickle cell disease.
Fig 2
Fig 2. The frequency of VOC during the first year of follow-up.
VOC: Vaso-occlusive crisis.
Fig 3
Fig 3. VOC per patients in different settings during the first year of follow-up.
ER: Emergency room; VOC: Vaso-occlusive crisis; #: Number; Other setting includes pharmacy, school, homeless shelter, prison/correctional facility, patient’s home, assisted living facility, independent laboratory and unknown setting.
Fig 4
Fig 4. SCD-related complications and concomitant comorbidities using primary and secondary diagnosis during the entire follow-up period.
DVT: Deep vein thrombosis; SCD: Sickle cell disease.
Fig 5
Fig 5. Primary reason for ER visits and inpatient stay during the first year of follow-up.
SCD: Sickle cell disease; VOC: Vaso-occlusive crisis.
See this image and copyright information in PMC

References

    1. Brousseau DC, Panepinto JA, Nimmer M, Hoffmann RG. The number of people with sickle‐cell disease in the United States: national and state estimates. Am J Hematol. 2010;85(1):77–8. 10.1002/ajh.21570 - DOI - PubMed
    1. Hassell KL. Population estimates of sickle cell disease in the U.S. Am J Prev Med. 2010;38(4 Suppl):S512–21. - PubMed
    1. Piel FB, Steinberg MH, Rees, DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561–73. 10.1056/NEJMra1510865 - DOI - PubMed
    1. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018–31. 10.1016/S0140-6736(10)61029-X - DOI - PubMed
    1. Habara A, Steinberg MH. Minireview: genetic basis of heterogeneity and severity in sickle cell disease. Exp Biol Med (Maywood). 2016;241(7):689‐96. - PMC - PubMed

Publication types