Research ArticleOriginal Research

Sickle-Cell Disease Co-Management, Health Care Utilization, and Hydroxyurea Use

Nancy Crego, Christian Douglas, Emily Bonnabeau, Marian Earls, Kern Eason, Elizabeth Merwin, Gary Rains, Paula Tanabe and Nirmish Shah
The Journal of the American Board of Family Medicine January 2020, 33 (1) 91-105; DOI: https://doi.org/10.3122/jabfm.2020.01.190143

References

  1. 1.↵
    1. Hassell KL
    . Population estimates of sickle cell disease in the U.S. Am J Prev Med 2010;38:S512–S521.
    OpenUrlCrossRefPubMedWeb of Science
  2. 2.↵
    1. Lanzkron S,
    2. Carroll CP,
    3. Haywood C Jr.
    . The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database. Am J Hematol 2010;85:797–9.
    OpenUrlCrossRefPubMed
  3. 3.↵
    1. Elixhauser A,
    2. Steiner CA
    . Readmissions to U.S. hospitals by diagnosis, 2010. Rockville, MD: Agency for Healthcare Research and Quality; August 2013. Statistical Brief #153.
  4. 4.↵
    1. Brousseau DC,
    2. Owens PL,
    3. Mosso AL,
    4. Panepinto JA,
    5. Steiner CA
    . Acute care utilization and rehospitalizations for sickle cell disease. JAMA 2010;303:1288–94.
    OpenUrlCrossRefPubMedWeb of Science
  5. 5.↵
    1. Tanabe P,
    2. Artz N,
    3. Mark Courtney D,
    4. et al
    . Adult emergency department patients with sickle cell pain crisis: a learning collaborative model to improve analgesic management. Acad Emerg Med 2010;17:399–407.
    OpenUrlPubMed
  6. 6.↵
    1. Tanabe P,
    2. Hafner JW,
    3. Martinovich Z,
    4. Artz N
    . Adult emergency department patients with sickle cell pain crisis: results from a quality improvement learning collaborative model to improve analgesic management. Acad Emerg Med 2012;19:430–8.
    OpenUrlCrossRefPubMedWeb of Science
  7. 7.↵
    1. Raphael JL,
    2. Oyeku SO
    . Sickle cell disease pain management and the medical home. Washington, DC: Hematology American Society of Hematology Education Program 2013;2013:433–8.
    OpenUrl
  8. 8.↵
    1. Aljuburi G,
    2. Phekoo KJ,
    3. Okoye NO,
    4. et al
    . Patients' views on improving sickle cell disease management in primary care: focus group discussion. JRSM Short Rep 2012;3:84.
    OpenUrlCrossRefPubMed
  9. 9.↵
    1. Lunyera J,
    2. Jonassaint C,
    3. Jonassaint J,
    4. Shah N
    . Attitudes of primary care physicians toward sickle cell disease care, guidelines, and comanaging hydroxyurea with a specialist. J Prim Care Community Health 2017;8:37–40.
    OpenUrlCrossRefPubMed
  10. 10.↵
    1. Yawn BP,
    2. Buchanan GR,
    3. Afenyi-Annan AN,
    4. et al
    . Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014;312:1033–48.
    OpenUrlCrossRefPubMed
  11. 11.↵
    1. Adams-Graves P,
    2. 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;9:541–52.
    OpenUrlCrossRefPubMed
  12. 12.↵
    1. Whiteman LN,
    2. Haywood C Jr.,
    3. Lanzkron S,
    4. Strouse JJ,
    5. Feldman L,
    6. Stewart RW
    . Primary care providers' comfort levels in caring for patients with sickle cell disease. South Med J 2015;108:531–6.
    OpenUrlCrossRef
  13. 13.↵
    1. Mainous AG III.,
    2. Tanner RJ,
    3. Harle CA,
    4. Baker R,
    5. Shokar NK,
    6. Hulihan MM
    . Attitudes toward management of sickle cell disease and its complications: a national survey of academic family physicians. Anemia 2015;853835.
  14. 14.↵
    1. Tilson EC
    . Dissemination and Adoption of guidelines: the experience of community care of North Carolina. N C Med J 2015;76:251–5.
    OpenUrlAbstract/FREE Full Text
  15. 15.↵
    North Carolina Institute of Medicine. Understanding Medicaid in North Carolina Primer. 2018. Available from: http://nciom.org/wp-content/uploads/2018/01/NC_Medicaid_CHIP_Primer.pdf. Accessed September 27, 2018.
  16. 16.↵
    1. Blinder MA,
    2. Duh MS,
    3. Sasane M,
    4. Trahey A,
    5. Paley C,
    6. Vekeman F
    . Age-related emergency department reliance in patients with sickle cell disease. J Emerg Med 2015;49:513–522 e511.
    OpenUrl
  17. 17.↵
    1. Kroner EL,
    2. Hoffmann RG,
    3. Brousseau DC
    . Emergency department reliance: a discriminatory measure of frequent emergency department users. Pediatrics 2010;125:133–8.
    OpenUrlAbstract/FREE Full Text
  18. 18.↵
    Centers for Medicare and Medicaid Services. Crosswalk Medicare Provider/Supplier to Healthcare Provider Taxonomy. 2018. Available from: https://data.cms.gov/Medicare-Enrollment/CROSSWALK-MEDICARE-PROVIDER-SUPPLIER-to-HEALTHCARE/j75i-rw8y.
  19. 19.↵
    1. Walsh KE,
    2. Cutrona SL,
    3. Kavanagh PL,
    4. et al
    . Medication adherence among pediatric patients with sickle cell disease: a systematic review. Pediatrics 2014;134:1175–83.
    OpenUrlAbstract/FREE Full Text
  20. 20.↵
    1. Candrilli SD,
    2. O'Brien SH,
    3. Ware RE,
    4. Nahata MC,
    5. Seiber EE,
    6. Balkrishnan R
    . Hydroxyurea adherence and associated outcomes among Medicaid enrollees with sickle cell disease. Am J Hematol 2011;86:273–7.
    OpenUrlCrossRefPubMed
  21. 21.↵
    1. Andemariam B,
    2. Owarish-Gross J,
    3. Grady J,
    4. Boruchov D,
    5. Thrall RS,
    6. Hagstrom JN
    . Identification of risk factors for an unsuccessful transition from pediatric to adult sickle cell disease care. Pediatr Blood Cancer 2014;61:697–701.
    OpenUrl
  22. 22.↵
    1. Kayle M,
    2. Docherty SL,
    3. Sloane R,
    4. et al
    . Transition to adult care in sickle cell disease: a longitudinal study of clinical characteristics and disease severity. Pediatr Blood Cancer 2019;66:e27463.
    OpenUrl
  23. 23.↵
    1. McClish DK,
    2. Smith WR,
    3. Levenson JL,
    4. et al
    . Comorbidity, pain, utilization, and psychosocial outcomes in older versus younger sickle cell adults: The PiSCES project. Biomed Res Int 2017;2017:4070547.
    OpenUrl
  24. 24.↵
    1. Quinn CT,
    2. Rogers ZR,
    3. McCavit TL,
    4. Buchanan GR
    . Improved survival of children and adolescents with sickle cell disease. Blood 2010;115:3447–52.
    OpenUrlAbstract/FREE Full Text
  25. 25.↵
    1. Brousseau DC,
    2. Panepinto JA,
    3. Nimmer M,
    4. Hoffmann RG
    . The number of people with sickle-cell disease in the United States: national and state estimates. AM J Hematol 2010;85:77–8.
    OpenUrlCrossRefPubMedWeb of Science
  26. 26.↵
    1. Steiner CA,
    2. Miller JL
    . Sickle cell disease patients in U.S. hospitals, 2004. HCUP Statistical Brief #21 2006. Available from: http://www.hcup-us.ahrq.gov/reports/statbriefs/sb21.pdf. Accessed December, 2018.
  27. 27.↵
    1. Fillingim RB,
    2. King CD,
    3. Ribeiro-Dasilva MC,
    4. Rahim-Williams B,
    5. Riley JL 3rd.
    . Sex, gender, and pain: a review of recent clinical and experimental findings. J Pain 2009;10:447–85.
    OpenUrlCrossRefPubMedWeb of Science
  28. 28.↵
    1. Bartley EJ,
    2. Fillingim RB
    . Sex differences in pain: a brief review of clinical and experimental findings. Br J Anaesthesia 2013;111:52–8.
    OpenUrlCrossRefPubMedWeb of Science
  29. 29.↵
    1. Sorge RE,
    2. Strath LJ
    . Sex differences in pain responses. Curr Opin Physiol 2018;6:75–81.
    OpenUrl
  30. 30.↵
    1. McClish DK,
    2. Levenson JL,
    3. Penberthy LT,
    4. et al
    . Gender differences in pain and healthcare utilization for adult sickle cell patients: The PiSCES Project. J Women's Health 2006;15:146–54.
    OpenUrlCrossRefWeb of Science
  31. 31.↵
    1. Udezue E,
    2. Girshab AM
    . Differences between males and females in adult sickle cell pain crisis in eastern Saudi Arabia. Ann Saudi Med 2004;24:179–82.
    OpenUrlPubMed
  32. 32.↵
    1. Haque A,
    2. Telfair J
    . Socioeconomic distress and health status: the urban-rural dichotomy of services utilization for people with sickle cell disorder in North Carolina. J Rurual Health 2000;16:43–55.
    OpenUrl
  33. 33.↵
    1. Asnani MR,
    2. Knight Madden J,
    3. Reid M,
    4. Greene LG,
    5. Lyew-Ayee P
    . Socio-environmental exposures and health outcomes among persons with sickle cell disease. PLoS One 2017;12:e0175260.
    OpenUrl
  34. 34.↵
    United States Department of Agriculture. Metropolitan statistical areas metropolitan divisions micropolitan statistical areas and combined statistical areas and guidance on uses of the delineations of these areas. 2013. Available from: https://obamawhitehouse.archives.gov/sites/default/files/omb/bulletins/2013/b-13–01.pdf. Accessed September 26, 2018.
  35. 35.↵
    1. Cronin RM,
    2. Hankins JS,
    3. Byrd J,
    4. et al
    . Risk factors for hospitalizations and readmissions among individuals with sickle cell disease: results of a U.S. survey study. Hematology 2019;24:189–98.
    OpenUrl
  36. 36.↵
    1. Benenson I,
    2. Jadotte Y,
    3. Echevarria M
    . Factors influencing utilization of hospital services by adult sickle cell disease patients: a systematic review. JBI Database System Rev Implement Rep 2017;15:765–808.
    OpenUrl
  37. 37.↵
    1. Brodsky MA,
    2. Rodeghier M,
    3. Sanger M,
    4. et al
    . Risk factors for 30-day readmission in adults with sickle cell disease. Am J Med 2017;130:601; e609–e615.
    OpenUrl
  38. 38.↵
    1. Lanzkron S,
    2. Little J,
    3. Field J,
    4. et al
    . Increased acute care utilization in a prospective cohort of adults with sickle cell disease. Blood Advances 2018;2:2412–7.
    OpenUrlAbstract/FREE Full Text
  39. 39.↵
    1. Blinder MA,
    2. Vekeman F,
    3. Sasane M,
    4. et al
    . Age-related emergency department reliance and healthcare resource utilization in patients with sickle cell disease. Blood 2012;120(475).
  40. 40.↵
    1. Leschke J,
    2. Panepinto JA,
    3. Nimmer M,
    4. Hoffmann RG,
    5. Yan K,
    6. Brousseau DC
    . Outpatient follow-up and rehospitalizations for sickle cell disease patients. Pediatr Blood Cancer 2012;58:406–9.
    OpenUrlCrossRefPubMedWeb of Science
  41. 41.↵
    1. Hemker BG,
    2. Brousseau DC,
    3. Yan K,
    4. Hoffman RG,
    5. Panepinto JA
    . When children with sickle-cell disease become adults: lack of outpatient care leads to increased use of the emergency department. Am J Hematology 2011;86:863–5.
    OpenUrlCrossRefPubMed
  42. 42.↵
    1. Frei-Jones MJ,
    2. Field JJ,
    3. DeBaun MR
    . Risk factors for hospital readmission within 30 days: a new quality measure for children with sickle cell disease. Pediatr Blood Cancer 2009;52:481–5.
    OpenUrlCrossRefPubMedWeb of Science
  43. 43.↵
    1. Ballas SK,
    2. Lusardi M
    . Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol 2005;79:17–25.
    OpenUrlCrossRefPubMedWeb of Science
  44. 44.↵
    1. Field TS,
    2. Ogarek J,
    3. Garber L,
    4. Reed G,
    5. Gurwitz JH
    . Association of early post-discharge follow-up by a primary care physician and 30-day rehospitalization among older adults. J Gen Intern Med 2015;30:565–71.
    OpenUrl
  45. 45.↵
    1. Rollins M,
    2. Milone F,
    3. Suleman S,
    4. Vojvoda D,
    5. Sgro M,
    6. Barozzino T
    . Patient navigators: mapping the route toward accessibility in health care. Paediatrics Child Health 2019;24:19–22.
    OpenUrl
  46. 46.↵
    1. Rubin DM
    . Community health worker and mobile health programs to help young adults with sickle cell disease transition to using adult healthcare services: the COMETS STudy. 2017. Available from: https://www.pcori.org/research-results/2017/community-health-worker-and-mobile-health-programs-help-young-adults-sickle. Accessed March 23, 2019.
  47. 47.↵
    1. Osunkwo I
    . Comparitive effectiveness of peer mentoring versus structured education-based transition programming for the management of care transitions in emerging adults with sickle cell disease. 2017. Available from: https://www.pcori.org/research-results/2017/comparative-effectiveness-peer-mentoring-versus-structured-education-based. Accessed March 23, 2019.
  48. 48.↵
    1. Mvundura M,
    2. Amendah D,
    3. Kavanagh PL,
    4. Sprinz PG,
    5. 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.
    OpenUrlCrossRefPubMedWeb of Science
  49. 49.↵
    1. Cronin RM,
    2. Hankins JS,
    3. Byrd J,
    4. et al
    . Modifying factors of the health belief model associated with missed clinic appointments among individuals with sickle cell disease. Hematology 2018;23:683–91.
    OpenUrl
  50. 50.↵
    1. Carroll CP,
    2. Cichowitz C,
    3. Yu T,
    4. et al
    . Predictors of acute care utilization and acute pain treatment outcomes in adults with sickle cell disease: The role of non-hematologic characteristics and baseline chronic opioid dose. Am J Hematol 2018;93:1127–35.
    OpenUrl
  51. 51.↵
    1. Raphael JL,
    2. Rattler TL,
    3. Kowalkowski MA,
    4. Mueller BU,
    5. Giordano TP
    . The medical home experience among children with sickle cell disease. Pediatr Blood Cancer 2013;60:275–80.
    OpenUrlCrossRefPubMed
  52. 52.↵
    1. Larochelle JL,
    2. Feldman DE,
    3. Levesque JF
    . The primary-specialty care interface in chronic diseases: patient and practice characteristics associated with co-management. Healthcare Policy 2014;10:52–63.
    OpenUrl
  53. 53.↵
    1. Samal L,
    2. Wright A,
    3. Waikar SS,
    4. Linder JA
    . Nephrology co-management versus primary care solo management for early chronic kidney disease: a retrospective cross-sectional analysis. BMC Nephrol 2015;16:162.
    OpenUrlCrossRefPubMed
  54. 54.↵
    1. Haley WE,
    2. Beckrich AL,
    3. Sayre J,
    4. et al
    . Improving care coordination between nephrology and primary care: a quality improvement initiative using the renal physicians association toolkit. Am J Kidney Dis 2015;65:67–79.
    OpenUrlCrossRefPubMed
  55. 55.↵
    1. Bartels CM,
    2. Roberts TJ,
    3. Hansen KE,
    4. et al
    . Rheumatologist and primary care management of cardiovascular disease risk in rheumatoid arthritis: patient and provider perspectives. Arthritis Care Res (Hoboken) 2016;68:415–23.
    OpenUrl
  56. 56.↵
    1. Brandow AM,
    2. Panepinto JA
    . Hydroxyurea use in sickle cell disease: the battle with low prescription rates, poor patient compliance and fears of toxicities. Expert Rev Hematol 2010;3:255–60.
    OpenUrlCrossRefPubMedWeb of Science
  57. 57.↵
    1. Loiselle K,
    2. Lee JL,
    3. Szulczewski L,
    4. Drake S,
    5. Crosby LE,
    6. Pai AL
    . Systematic and meta-analytic review: medication adherence among pediatric patients with sickle cell disease. J Pediatr Psychol 2016;41:406–18.
    OpenUrlCrossRefPubMed
  58. 58.↵
    1. Patel NG,
    2. Lindsey T,
    3. Strunk RC,
    4. DeBaun MR
    . Prevalence of daily medication adherence among children with sickle cell disease: a 1-year retrospective cohort analysis. Pediatr Blood Cancer 2010;55:554–6.
    OpenUrlCrossRefPubMedWeb of Science
  59. 59.↵
    1. Heeney MM,
    2. Ware RE
    . Hydroxyurea for children with sickle cell disease. Hematol Oncol Clin North Am 2010;24:199–214.
    OpenUrlCrossRefPubMed
  60. 60.↵
    1. Thornburg CD,
    2. Calatroni A,
    3. Telen M,
    4. Kemper AR
    . Adherence to hydroxyurea therapy in children with sickle cell anemia. J Pediatrics 2010;156:415–9.
    OpenUrlCrossRefPubMedWeb of Science
  61. 61.↵
    1. Badawy SM,
    2. Thompson AA,
    3. Lai JS,
    4. Penedo FJ,
    5. Rychlik K,
    6. Liem RI
    . Adherence to hydroxyurea, health-related quality of life domains, and patients' perceptions of sickle cell disease and hydroxyurea: a cross-sectional study in adolescents and young adults. Health Qual Life Outcomes 2017;15:136.
    OpenUrl
  62. 62.↵
    1. Haywood C Jr.,
    2. Beach MC,
    3. Bediako S,
    4. et al
    . Examining the characteristics and beliefs of hydroxyurea users and nonusers among adults with sickle cell disease. AM J Hematol 2011;86:85–7.
    OpenUrlCrossRefPubMed
  63. 63.↵
    1. Carroll CP,
    2. Haywood C Jr.,
    3. Fagan P,
    4. Lanzkron S
    . The course and correlates of high hospital utilization in sickle cell disease: evidence from a large, urban Medicaid managed care organization. Am J Hematol 2009;84:666–70.
    OpenUrlCrossRefPubMed
  64. 64.↵
    1. Mayer ML,
    2. Konrad TR,
    3. Dvorak CC
    . Hospital resource utilization among patients with sickle cell disease. J Health Care Poor Underserved 2003;14:122–35.
    OpenUrl
  65. 65.↵
    1. Yusuf HR,
    2. Atrash HK,
    3. Grosse SD,
    4. Parker CS,
    5. Grant AM
    . Emergency department visits made by patients with sickle cell disease: a descriptive study, 1999–2007. Am J Prev Med 2010;38:S536–S541.
    OpenUrlCrossRefPubMed
  66. 66.↵
    1. Gingold DB,
    2. Pierre-Mathieu R,
    3. Cole B,
    4. Miller AC,
    5. Khaldun JS
    . Impact of the Affordable Care Act Medicaid expansion on emergency department high utilizers with ambulatory care sensitive conditions: a cross-sectional study. Am J Emerg Med 2017;35:737–42.
    OpenUrl
  67. 67.↵
    1. Nikpay S,
    2. Freedman S,
    3. Levy H,
    4. Buchmueller T
    . Effect of the Affordable Care Act Medicaid expansion on emergency department visits: evidence from state-level emergency department databases. Ann Emerg Med 2017;70:215–225; e216.
    OpenUrlPubMed
  68. 68.↵
    1. Sommers BD,
    2. Blendon RJ,
    3. Orav EJ,
    4. Epstein AM
    . Changes in utilization and health among low-income adults after Medicaid expansion or expanded private insurance. JAMA Intern Med 2016;176:1501–9.
    OpenUrl
  69. 69.↵
    1. Ter-Minassian M,
    2. Lanzkron S,
    3. Derus A,
    4. Brown E,
    5. Horberg MA
    . Quality metrics and health care utilization for adult patients with sickle cell disease. J Natl Med Assoc 2019;111:54–61.
    OpenUrl
  70. 70.↵
    1. Jordan L,
    2. Swerdlow P,
    3. Coates TD
    . Systematic review of transition from adolescent to adult care in patients with sickle cell disease. J Ped Hematol Oncol 2013;35:165–9.
    OpenUrlCrossRefPubMed
  71. 71.↵
    1. Minniti CP,
    2. Vichinsky E
    . Lifespan care in SCD: whom to transition, the patients or the health care system? Am J Hematol 2017;92:487–9.
    OpenUrl
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