The Journal of Pediatrics
Volume 156, Issue 3 , Pages 415-419, March 2010

Adherence to Hydroxyurea Therapy in Children with Sickle Cell Anemia

  • Courtney D. Thornburg, MD, MS

      Affiliations

    • Department of Pediatrics, Duke University Medical Center, Durham, NC
    • Corresponding Author InformationReprint requests: Courtney D. Thornburg, MD MS, DUMC Box 102382, Durham, NC 27710.
    • ,
  • Agustin Calatroni, MA, MS

      Affiliations

    • Rho, Inc, Chapel Hill, NC
    • ,
  • Marilyn Telen, MD

      Affiliations

    • Department of Medicine, Duke University Medical Center, Durham, NC
    • ,
  • Alex R. Kemper, MD, MPH, MS

      Affiliations

    • Department of Pediatrics, Duke University Medical Center, Durham, NC
    • Duke Clinical Research Institute, Durham, NC

Received 9 February 2009; received in revised form 24 June 2009; accepted 15 September 2009. published online 02 November 2009.

Objectives

To assess adherence to hydroxyurea therapy in children with sickle cell anemia (SCA), evaluate the association between adherence and hematologic profile, and identify barriers and facilitators of adherence.

Study design

Children with SCA (n=75) receiving hydroxyurea were recruited for a single-institution cross-sectional study. The primary outcome was association between treatment adherence and percent fetal hemoglobin (HbF).

Results

Good adherence was estimated at 82% with visual analog scale, 84% with Morisky score, 85% with medical provider report, 77% with clinic visits, and 49% on the basis of pharmacy refills. Increase in HbF was moderately associated with good adherence as measured with the parent/proxy Morisky score (r=−0.39; 95% CI, −0.58–0.17; P < .01) and prescription refills (r=0.39; 95% CI, 0.16–0.57; P < .01). The number of pharmacy refills and the Morisky score explained 23% of the variation in HbF response.

Conclusions

Adherence was ≥75% with 4 of 5 measures. Pharmacy refills and the Modified Morisky Scale may be used to identify children at high risk for poor response because of non-adherence and children with good adherence with poor response because of individual pharmacodynamics. Future research should prospectively compare adherence measures and evaluate methods to improve treatment adherence.

DUMC, Duke University Medical Center, HbF, Percent fetal hemoglobin, MTD, Maximum tolerated dose, SCA, Sickle cell anemia

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 Supported by a National Institutes of Health Comprehensive Sickle Cell Center Investigator Research Supplement U54-HL070769 (C.T.). The NIH did not have a role in the study design; the collection, analysis, and interpretation of data; the writing of report; and the decision to submit the manuscript for publication. The authors declare no conflicts of interest.

PII: S0022-3476(09)00924-X

doi:10.1016/j.jpeds.2009.09.044

Refers to article:

  • Treatment Adherence in Patients with Sickle Cell Anemia , 08 January 2010

    Dennis Drotar
    The Journal of Pediatrics March 2010 (Vol. 156, Issue 3, Pages 350-351)

The Journal of Pediatrics
Volume 156, Issue 3 , Pages 415-419, March 2010

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