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Adherence to Quality of Care Indicators and Location of Sickle Cell Care Within Indiana

  • Emily Riehm MeierEmail author
  • Isaac A. Janson
  • Kisha Hampton
  • Ellen Bloom
  • Natalie Duncan
  • Chris Roberson
  • Angeli Rampersad
Original Paper

Abstract

Newborn screening (NBS) follow-up programs for infants with sickle cell disease (SCD) are highly variable among states. Initiated in 2009, Sickle SAFE, the NBS follow-up program for infants with SCD in Indiana, follows infants through home visits and phone contact. The current study assessed the attainment rates for recently published quality indicators of pediatric SCD care for Sickle SAFE participants. Using retrospective data, we determined the proportion of children who received transcranial Doppler (TCD) screening, influenza, and pneumococcal vaccination and were prescribed hydroxyurea. We calculated the mean age at confirmatory testing, time to receipt of penicillin prophylaxis, and mean age when genetic counseling was offered. One hundred ninety-eight children born with SCD in Indiana between July 1, 2009 and June 30, 2017 were followed for at least 1 year. While 97.5% received at least one dose of conjugated pneumococcal vaccine, vaccination with the 23 valent pneumococcal vaccine varied by location (county) of care (Allen: 14.3%, Lake: 26.7%, St. Joseph: 40.0%, Marion: 73.3%). Overall TCD screening rate for eligible children was 53%; TCD screening rate varied widely by location of care (Lake: 25% vs. Marion: 63.8%). Similarly, hydroxyurea prescribing practices varied significantly by location of care (p < 0.001). Identified gaps in adherence to quality indicators in SCD care will serve as the basis for future quality improvement initiatives.

Keywords

Sickle cell disease Newborn screening Penicillin prophylaxis Hydroxyurea Transcranial Doppler 

Notes

Acknowledgements

The Sickle SAFE program is funded by the Indiana State Department of Health, Maternal Child Health Division’s Genomics and Newborn Screening Program.

Compliance with Ethical Standards

Conflict of interest

Dr. Meier is a consultant for CVS Caremark and receives grant funding from the Indiana State Department of Health. The other authors have no conflicts to disclose.

References

  1. 1.
    Gaston, M. H., Verter, J. I., Woods, G., et al. (1986). Prophylaxis with oral penicillin in children with sickle cell anemia. New Englan Journal of Medicine, 314(25), 1593–1599.CrossRefGoogle Scholar
  2. 2.
    National Heart Lung and Blood Institute (NHLBI) (2014). Evidence-based management of sickle cell disease: expert panel report, 2014. Retrieved May 15, 2019, from http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines.
  3. 3.
    Adamkiewicz, T. V., Sarnaik, S., Buchanan, G. R., et al. (2003). Invasive pneumococcal infections in children with sickle cell disease in the era of penicillin prophylaxis, antibiotic resistance, and 23-valent pneumococcal polysaccharide vaccination. Journal of Pediatrics, 143(4), 438–444.CrossRefGoogle Scholar
  4. 4.
    La Pean, A., Collins, J. L., Christopher, S. A., et al. (2012). A qualitative secondary evaluation of statewide follow-up interviews for abnormal newborn screening results for cystic fibrosis and sickle cell hemoglobinopathy. Genetics in Medicine, 14(2), 207–214.CrossRefGoogle Scholar
  5. 5.
    Michlitsch, J., Azimi, M., Hoppe, C., et al. (2009). Newborn screening for hemoglobinopathies in California. Pediatric Blood & Cancer, 52(4), 486–490.CrossRefGoogle Scholar
  6. 6.
    Kavanagh, P. L., Wang, C. J., Therrell, B. L., Sprinz, P. G., & Bauchner, H. (2008). Communication of positive newborn screening results for sickle cell disease and sickle cell trait: Variation across states. American Journal of Medical Genetics C, 148(1), 15–22.  https://doi.org/10.1002/ajmg.c.30160.CrossRefGoogle Scholar
  7. 7.
    Wang, C. J., Kavanagh, P. L., Little, A. A., Holliman, J. B., & Sprinz, P. G. (2011). Quality-of-care indicators for children with sickle cell disease. Pediatrics, 128(3), 484–493.Google Scholar
  8. 8.
    Indiana State Department of Health. Indiana Vital Statistics. Retrieved from http://www.stats.indiana.edu/vitals/. Accessed October 25, 2018.
  9. 9.
    United States Census Bureau Quick Facts Indiana, United States. 2018. Washington, DC: Department of Commerce. Retrieved from https://www.census.gov/quickfacts/fact/table/indianapoliscitybalanceindiana,US/PST045218. Accessed October 25, 2018.
  10. 10.
    United States Census Bureau Urban and Rural Classification and Urban Area Criteria (2010). Washington, DC: Department of Commerce. Retrieved from https://www.census.gov/programs-surveys/geography/guidance/geo-areas/urban-rural/2010-urban-rural.html. Accessed October 25, 2018.
  11. 11.
    Streetly, A., Sisodia, R., Dick, M., Latinovic, R., Hounsell, K., & Dormandy, E. (2018). Evaluation of newborn sickle cell screening programme in England: 2010–2016. Archives of Disease in Childhood, 103(7), 648–653.Google Scholar
  12. 12.
    Centers for Disease Control and Prevention Estimates of Flu Vaccination Coverage among Children—United States, 2017–2018 Flu Season. Retrieved from https://www.cdc.gov/flu/fluvaxview/coverage-1718estimates-children.htm. Published 2018. Accessed November 14, 2018.
  13. 13.
    Neunert, C. E., Gibson, R. W., Lane, P. A., et al. (2016). Determining adherence to quality indicators in sickle cell anemia using multiple data sources. American Journal of Preventive Medicine, 51(1 Suppl 1), S24–S30.CrossRefGoogle Scholar
  14. 14.
    Steinberg, M. H., McCarthy, W. F., Castro, O., et al. (2010). The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up. American Journal of Hematology, 85(6), 403–408.Google Scholar
  15. 15.
    Thornburg, C. D., Files, B. A., Luo, Z., et al. (2012). Impact of hydroxyurea on clinical events in the BABY HUG trial. Blood, 120(22), 4304–4310.CrossRefGoogle Scholar
  16. 16.
    Lobo, C. L., Pinto, J. F., Nascimento, E. M., Moura, P. G., Cardoso, G. P., & Hankins, J. S. (2013). The effect of hydroxycarbamide therapy on survival of children with sickle cell disease. British Journal of Haematology, 161(6), 852–860.CrossRefGoogle Scholar
  17. 17.
    Luchtman-Jones, L., Pressel, S., Hilliard, L., et al. (2016). Effects of hydroxyurea treatment for patients with hemoglobin SC disease. American Journal of Hematology, 91(2), 238–242.CrossRefGoogle Scholar
  18. 18.
    Rogers, Z. R. (1997). Hydroxyurea therapy for diverse pediatric populations with sickle cell disease. Seminars in Hematology, 34(3 Suppl 3), 42–47.Google Scholar
  19. 19.
    Lanzkron, S., Haywood, C., Jr., Segal, J. B., & Dover, G. J. (2006). Hospitalization rates and costs of care of patients with sickle-cell anemia in the state of Maryland in the era of hydroxyurea. American Journal of Hematology, 81(12), 927–932.CrossRefGoogle Scholar
  20. 20.
    Lanzkron, S., Haywood, C., Jr., Hassell, K. L., & Rand, C. (2008). Provider barriers to hydroxyurea use in adults with sickle cell disease: A survey of the sickle cell disease adult provider network. Journal of the National Medical Association, 100(8), 968–973.CrossRefGoogle Scholar
  21. 21.
    Brandow, A. M., Jirovec, D. L., & Panepinto, J. A. (2010). Hydroxyurea in children with sickle cell disease: Practice patterns and barriers to utilization. American Journal of Hematology, 85(8), 611–613.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Departments of Pediatric HematologyIndiana Hemophilia and Thrombosis CenterIndianapolisUSA
  2. 2.Community ProgramsIndiana Hemophilia and Thrombosis CenterIndianapolisUSA

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