title: Medication adherence among pediatric patients with sickle cell disease: a systematic review.
abstract: OBJECTIVE
  Describe rates of adherence for sickle cell disease (SCD) medications, identify patient and medication characteristics associated with nonadherence, and determine the effect of nonadherence and moderate adherence (defined as taking 60%-80% of doses) on clinical outcomes.
  
  
  METHODS
  In February 2012 we systematically searched 6 databases for peer-reviewed articles published after 1940. We identified articles evaluating medication adherence among patients <25 years old with SCD. Two authors reviewed each article to determine whether it should be included. Two authors extracted data, including medication studied, adherence measures used, rates of adherence, and barriers to adherence.
  
  
  RESULTS
  Of 24 articles in the final review, 23 focused on 1 medication type: antibiotic prophylaxis (13 articles), iron chelation (5 articles), or hydroxyurea (5 articles). Adherence rates ranged from 16% to 89%; most reported moderate adherence. Medication factors contributed to adherence. For example, prophylactic antibiotic adherence was better with intramuscular than oral administration. Barriers included fear of side effects, incorrect dosing, and forgetting. Nonadherence was associated with more vaso-occlusive crises and hospitalizations. The limited data available on moderate adherence to iron chelation and hydroxyurea indicates some clinical benefit.
  
  
  CONCLUSIONS
  Moderate adherence is typical among pediatric patients with SCD. Multicomponent interventions are needed to optimally deliver life-changing medications to these children and should include routine monitoring of adherence, support to prevent mistakes, and education to improve understanding of medication risks and benefits.
authors.affiliation: Department of Pediatrics, Cincinnati Children's Hospital, University of Cincinnati School of Medicine, Cincinnati, Ohio; Departments of Pediatrics, and Meyers Primary Care Institute, Worcester, Massachusetts; Kathleen.walsh@cchmc.org.
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Walsh
authors.affiliation: Meyers Primary Care Institute, Worcester, Massachusetts; Medicine, University of Massachusetts, Worcester, Massachusetts;
authors.forename: Sarah L
authors.initials: SL
authors.lastname: Cutrona
authors.affiliation: Department of Pediatrics, Boston University, Boston, Massachusetts;
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Kavanagh
authors.affiliation: Department of Pediatrics, Cincinnati Children's Hospital, University of Cincinnati School of Medicine, Cincinnati, Ohio;
authors.forename: Lori E
authors.initials: LE
authors.lastname: Crosby
authors.affiliation: Meyers Primary Care Institute, Worcester, Massachusetts;
authors.forename: Chris
authors.initials: C
authors.lastname: Malone
authors.affiliation: Welch Medical Library, Johns Hopkins Medical Center, Baltimore, Maryland; and.
authors.forename: Katie
authors.initials: K
authors.lastname: Lobner
authors.affiliation: Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina.
authors.forename: David G
authors.initials: DG
authors.lastname: Bundy
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000523:psychology; D019072:Antibiotic Prophylaxis / Q000009:adverse effects / Q000523:psychology; D002648:Child; D016903:Drug Monitoring; D057915:Drug Substitution; D006801:Humans; D006918:Hydroxyurea / Q000009:adverse effects / Q000627:therapeutic use*; D007502:Iron Chelating Agents / Q000009:adverse effects / Q000627:therapeutic use*; D055118:Medication Adherence / Q000523:psychology*; D010353:Patient Education as Topic; D010406:Penicillins / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007502:Iron Chelating Agents; D010406:Penicillins; D006918:Hydroxyurea
keywords: medication adherence; sickle cell disease