Barriers to hydroxyurea adherence and health-related quality of life in adolescents and young adults with sickle cell disease

Eur J Haematol. 2017 Jun;98(6):608-614. doi: 10.1111/ejh.12878. Epub 2017 Apr 17.


Objectives: To identify barriers to hydroxyurea adherence (negative beliefs, access, and/or recall barriers), and their relationship to adherence rates and health-related quality of life (HRQOL) among adolescents and young adults (AYA) with sickle cell disease (SCD).

Methods: A cross-sectional survey was administered to 34 AYAs (12-22 years old) in SCD clinics from January to December 2015. Study measures included Brief Medication Questionnaire, Modified Morisky Adherence Scale 8-items, visual analog scale, and Patient Reported Outcomes Measurement Information System.

Results: Participants (59% male; 91% Black) had a median age of 13.5 years (IQR 12-18). Participants reported negative beliefs (32%), recall barriers (44%), and access barriers (32%). Participants with recall barriers reported worse pain (P=.02), fatigue (P=.05), and depression (P=.05). The number of adherence barriers inversely correlated with adherence level using ©MMAS-8 (rs =-.38, P=.02) and VASdose (rs =-.25, P=.14) as well as MCV (rs =-.45, P=.01) and HbF% (rs =-.36, P=.05), suggesting higher hydroxyurea adherence in patients with fewer barriers.

Conclusions: Patients with fewer barriers to hydroxyurea adherence were more likely to have higher adherence rates and better HRQOL scores. Routine assessment of hydroxyurea adherence and its related barriers could provide actionable information to improve adherence rates, HRQOL, and other clinical outcomes.

Keywords: PROMIS ®; adherence; adolescents and young adults; barriers; compliance; health-related quality of life; hydroxyurea; patient reported outcomes; patient reported outcomes measurement information system; sickle cell.

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / drug therapy*
  • Anemia, Sickle Cell / physiopathology
  • Anemia, Sickle Cell / psychology
  • Antisickling Agents / therapeutic use*
  • Child
  • Cross-Sectional Studies
  • Female
  • Humans
  • Hydroxyurea / therapeutic use*
  • Male
  • Medication Adherence / psychology*
  • Medication Adherence / statistics & numerical data
  • Pain Measurement / methods
  • Quality of Life / psychology*
  • Surveys and Questionnaires
  • Young Adult


  • Antisickling Agents
  • Hydroxyurea