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Health and Quality of Life Outcomes
Published in: Health and Quality of Life Outcomes 1/2017

Open Access 01-12-2017 | Research

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

Authors: Sherif M. Badawy, Alexis A. Thompson, Jin-Shei Lai, Frank J. Penedo, Karen Rychlik, Robert I. Liem

Published in: Health and Quality of Life Outcomes | Issue 1/2017

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Abstract

Background

Sickle cell disease (SCD) patients have impaired domains of health-related quality of life (HRQOL). Hydroxyurea is safe and efficacious in SCD; however, adherence is suboptimal, and patients’ perceptions are poorly understood amongst adolescents and young adults (AYA). Study objectives were to: (1) examine patients’ perceptions of SCD and hydroxyurea; and (2) explore the relationship of their perceptions to clinical characteristics, HRQOL domains and hydroxyurea adherence.

Methods

Thirty-four SCD patients on hydroxyurea (≥6 months) participated in a single-institution study. Study measures included Brief-Illness Perceptions Questionnaire, ©Modified Morisky Adherence Scale 8-items, and Patient Reported Outcomes Measurement Information System (PROMIS®). We assessed the relationship of patients’ perceptions to hydroxyurea adherence using Wilcoxon rank-sum test, the number of hospitalizations using Kruskal-Wallis test, and the number of ED visits, adherence level, HRQOL domain scores using Spearman’s rho correlations. We conducted a sub-analysis in HbSS patients to evaluate the relationship of patients’ perceptions to laboratory markers of hydroxyurea adherence.

Results

Participants were 59% male and 91% Black, and had a median age of 13.5 (range 12–18) years. Participants with ≥4 hospitalizations over 1-year prior (using electronic medical chart review) reported more negative perceptions of SCD-related symptoms and emotional response, and perceived hydroxyurea as less beneficial; all p-values ≤0.01. Most participants (74%) reported low hydroxyurea adherence. Participants with higher hydroxyurea adherence perceived more hydroxyurea benefits (r s = 0.44, p < 0.01) and had better emotional response to SCD (r s = −0.44, p = 0.01). In a sub-analysis of HbSS patients, perceived benefits of hydroxyurea positively correlated with HbF (r s = 0.37, p = 0.05) and MCV values (r s = 0.35, p = 0.05). Participants with more negative perceptions of SCD-related consequences, concerns, and emotional response, and fewer perceived hydroxyurea benefits reported worse fatigue (r s = 0.68; r s = 0.44; r s = 0.74; r s = −0.60), pain (r s = 0.56; r s = 0.54; r s = 0.63; r s = −0.39), anxiety (r s = 0.55; r s = 0.58; r s = 0.56; r s = −0.47), and depression (r s = 0.64; r s = 0.49; r s = 0.70; r s = −0.62), respectively, all p-values <0.05.

Conclusions

Dynamics influencing hydroxyurea adherence are multifactorial, and understanding patients’ perceptions is critical to overcoming adherence barriers. Patients’ favorable perceptions correlated with greater adherence and better HRQOL domain scores. Prospective evaluation of patients’ perceptions of SCD and hydroxyurea in relation adherence, HRQOL domains and clinical outcomes is warranted.
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Metadata
Title
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
Authors
Sherif M. Badawy
Alexis A. Thompson
Jin-Shei Lai
Frank J. Penedo
Karen Rychlik
Robert I. Liem
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Health and Quality of Life Outcomes / Issue 1/2017
Electronic ISSN: 1477-7525
DOI
https://doi.org/10.1186/s12955-017-0713-x

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