Systematic and Meta-Analytic Review: Medication Adherence Among Pediatric Patients With Sickle Cell Disease

Loiselle, Kristin A.; Lee, Jennifer L.; Szulczewski, Lauren; Drake, Sarah; Crosby, Lori E.; Pai, Ahna L. H.

doi:10.1093/jpepsy/jsv084

Cited by 64 publications

(71 citation statements)

References 71 publications

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“…A recent meta-analysis reported lower adherence rates in older patients and those with more identified barriers [16], which are numerous and varied in the literature [13–22]. Nevertheless, the relationship between patients’ perceptions of both hydroxyurea and SCD and their adherence to hydroxyurea has not been previously examined.…”

Section: Discussionmentioning

confidence: 99%

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

Badawy

Thompson

Lai

et al. 2017

Health Qual Life Outcomes

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BackgroundSickle 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.MethodsThirty-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.ResultsParticipants 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.ConclusionsDynamics 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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Section: Discussionmentioning

confidence: 99%

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

Badawy

Thompson

Lai

et al. 2017

Health Qual Life Outcomes

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“…Hydroxyurea markedly reduces symptoms, morbidity and mortality, improves HRQL, decreases healthcare cost, and may protect against cumulative disease burden. Despite these therapeutic benefits, adherence in adolescents and young adults with SCD is often poor …”

Section: Introductionmentioning

confidence: 99%

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

Smaldone

Manwani

Green

2019

Pediatric Blood & Cancer

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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.

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“…Hydroxyurea is efficacious and cost‐effective in pediatric and adult patients with SCD . Nevertheless, adherence to hydroxyurea in SCD remains suboptimal, and lower adherence rates have been associated with worse HRQOL, more frequent SCD‐related complications, and increased healthcare utilization . Adherence barriers that have been identified for hydroxyurea include forgetfulness, lack of access to hydroxyurea, lack of knowledge about hydroxyurea, fear of side effects, and concerns about efficacy .…”

Section: Introductionmentioning

confidence: 99%

“…Nevertheless, adherence to hydroxyurea in SCD remains suboptimal, and lower adherence rates have been associated with worse HRQOL, more frequent SCD‐related complications, and increased healthcare utilization . Adherence barriers that have been identified for hydroxyurea include forgetfulness, lack of access to hydroxyurea, lack of knowledge about hydroxyurea, fear of side effects, and concerns about efficacy . A recent meta‐analysis showed a significant negative association between the number of adherence barriers and overall adherence rates to different medications in patients with SCD, including hydroxyurea .…”

Section: Introductionmentioning

confidence: 99%

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

Badawy

Thompson

Penedo

et al. 2017

European J of Haematology

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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 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. K E Y W O R D Sadherence, adolescents and young adults, barriers, compliance, health-related quality of life, hydroxyurea, patient reported outcomes, patient reported outcomes measurement information system, PROMIS ® , sickle cell | INTRODUCTIONSickle cell disease (SCD) is a lifelong debilitating disease that results in significant morbidity, early mortality, and impairment in health-related quality of life (HRQOL) over time. [1][2][3] Hydroxyurea is efficacious and cost-effective in pediatric and adult patients with SCD. [4][5][6][7][8] Nevertheless, adherence to hydroxyurea in SCD remains suboptimal, 4,9-12 and lower adherence rates have been associated with worse HRQOL, 13 more frequent SCD-related complications, and increased healthcare utilization. 4Adherence barriers that have been identified for hydroxyurea include forgetfulness, lack of access to hydroxyurea, lack of knowledge about hydroxyurea, fear of side effects, and concerns about efficacy. [9][10][11][12]14,15 A recent meta-analysis showed a significant negative association between the number of adherence barriers and overall adherence rates

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Systematic and Meta-Analytic Review: Medication Adherence Among Pediatric Patients With Sickle Cell Disease

Abstract: The wide range of adherence rates reported in the literature may be because of, in part, the use of variable assessment strategies. Future studies examining pediatric SCD adherence should incorporate key correlates with the goal of replication.

Cited by 64 publications

References 71 publications

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

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

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

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

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