Greater number of perceived barriers to hydroxyurea associated with poorer health-related quality of life in youth with sickle cell disease

Arlene Smaldone, Deepa Manwani, Nancy S. Green

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


Background: Despite medical benefits, hydroxyurea adherence in adolescents is often poor. As part of a baseline assessment of 28 youth (10–18 years) parent dyads who participated in a 6-month feasibility trial to improve hydroxyurea adherence, we measured the relationship between greater barriers to adherence and health-related quality of life (HRQL) from youth and parent perspectives. Procedure: Barriers were measured using the Adolescent and Parent Medication Barriers Scales with nine hydroxyurea items added. Barriers reported by ≥25% of the sample were considered common. Generic and disease-specific HRQL were measured by PedsQL and PedsQL Sickle Cell Disease modules. Data were analyzed using descriptive statistics, Cronbach alpha, Spearman correlation coefficients, and paired t tests. Results: Fifty-six subjects (28 dyads) participated. Youth reported greater barriers compared with parents (5.0 ± 3.9 and 3.5 ± 3.2; P = 0.03), with >80% of respondents reporting ≥1 barriers. Twelve barriers were reported by ≥25% of adolescents, whereas six were reported by ≥25% of parents. Of these, only two were common to both dyad members. Approximately one-third of youth had generic and disease-specific HRQL scores that fell at or below cutoff scores, suggesting being at risk for impaired HRQL. Greater barriers were inversely associated with poorer generic (parent r = −0.43, P = 0.03; youth r = −0.44, P < 0.001) and disease-specific HRQL (parent r = −0.53, P = 0.005; youth r = −0.53, P < 0.001). Conclusions: Hydroxyurea barriers were frequently reported but differed by dyad members’ perspective. Greater barriers were associated with poorer generic and disease-specific HRQL. To reduce barriers to hydroxyurea in youth with sickle cell disease, perspectives of both dyad members should be addressed.

Original languageEnglish (US)
Article numbere27740
JournalPediatric Blood and Cancer
Issue number7
StatePublished - Jul 2019


  • hydroxyurea
  • medication barriers
  • quality of life
  • sickle cell disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology


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