Hydroxyurea Initiation Among Children With Sickle Cell Anemia

Reeves, Sarah L.; Jary, Hannah K.; Gondhi, Jennifer P.; Raphael, Jean L.; Lisabeth, Lynda D.; Dombkowski, Kevin J.

doi:10.1177/0009922819850476

Cited by 6 publications

(11 citation statements)

References 55 publications

(71 reference statements)

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“…It is also remarkable that our patients who got hydroxyurea experienced lower mental HRQoL compared to those patients who did not. Despite documented benefits of hydroxyurea for pediatrics and young adults with SCD [ 47 , 48 ], Ceveher et al [ 49 ] proposed that quality of life and compliance of SCD patients might be low due to not sufficiently effective hydroxyurea therapy, comorbidities, concomitant medication use, and side effects. This issue can be explained in part by the previous patients' perceptions of treatment benefits versus risks, and their disease severity.…”

Section: Discussionmentioning

confidence: 99%

Sense of coherence or self-efficacy as predictors of health-related quality of life in sickle cell disease patients

et al. 2022

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Patients with sickle cell disease (SCD) suffer from impaired health-related quality of life (HRQoL). This study aimed to determine the level of HRQoL, sense of coherence (SOC), and self-efficacy (SE) in a sample of SCD patients, and to explore predictors of their physical and mental HRQoL. A cross-sectional descriptive study was conducted on 83 SCD patients of one university hospital. The data of the study was collected through Persian versions of the Short-Form Health Survey SF-36 (RAND 36-item), the Sense of Coherence Scale (SOC-13), and the Sickle Cell Self-Efficacy Scale (SCSES). The mean age of the patients was 26.34 ± 8.19 years old. Patients’ mean scores for the Physical Component Summary (PCS), Mental Component Summary (MCS), SOC, and SCSES were 40.57 ± 17.18 (range: 0–100), 50.44 ± 17.95 (range: 0–100), 52.40 ± 15.35 (range: 13–91), 26.40 ± 6.96 (range: 9–45), respectively. Regression models showed that the level of the patients’ SOC, was the main predictor of the MCS (β = 0.37, p < 0.001). However, the level of the patients’ SE was the main predictor of the PCS (β = 0.30, p = 0.004). Also, “blood transfusion history” in patients was a common predictor for both the PCS (β = − 0.28, p = 0.008) and the MCS (β = − 0.29, p = 0.003). These results can assist nurses and clinicians to plan clinical interventions for SCD patients by focusing on increasing the level of the SOC and SE and improving SCD patients’ HRQoL. Furthermore, measuring the level of the SOC and self-efficacy as screening tests are useful to find patients with a greater risk of impaired HRQoL.

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Section: Discussionmentioning

confidence: 99%

Sense of coherence or self-efficacy as predictors of health-related quality of life in sickle cell disease patients

et al. 2022

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“…Pain crises are associated with substantially lower quality of life, as well as inadequate sleep, increased school absences, chronic depression, and impaired peer relationships 7,32–35 . Although hydroxyurea has been recommended, previous studies have indicated both initiation and adherence are low 13,20,21,23 . Our results suggest that without sustained adherence after initiation, improvements in VOC and ACS outcomes will be hampered.…”

Section: Discussionmentioning

confidence: 70%

“…7,[32][33][34][35] Although hydroxyurea has been recommended, previous studies have indicated both initiation and adherence are low. 13,20,21,23 Our results suggest that without sustained adherence after initiation, improvements in VOC and ACS outcomes will be hampered.…”

Section: Discussionmentioning

confidence: 83%

Section: Methodsmentioning

confidence: 99%

“…Among individuals in the study population, we identified filled prescriptions for hydroxyurea using National Drug Codes, which were validated using RxNorm and review of existing lists. [20][21][22] The main exposure was hydroxyurea adherence, as measured by medication possession ratio (MPR). 23,24 We identified the date of hydroxyurea initiation in year 2, and then summed together the number of days' supply in each filled prescription from initiation date until the end of the study period (end of year 3 of observation).…”

Section: Exposure: Hydroxyurea Adherence By Medication Possession Ratiomentioning

confidence: 99%

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Adherence to hydroxyurea and clinical outcomes among children with sickle cell anemia

Reeves

Dombkowski

Peng

et al. 2023

Pediatric Blood & Cancer

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Objective Hydroxyurea lowers the incidence of vaso‐occlusive pain crises (VOC) and acute chest syndrome (ACS) among children with sickle cell anemia (SCA). Our objective was to assess the relationship between levels of adherence to hydroxyurea and clinical outcomes among children and adolescents with SCA. Methods This retrospective cohort study included Medicaid data (2005–2012) from Florida, Illinois, Louisiana, Michigan, South Carolina, and Texas. The study population consisted of children 1–17 years old with SCA enrolled in Medicaid for 3 years. Among children that initiated hydroxyurea, the medication possession ratio (MPR) was calculated as the proportion of days covered by hydroxyurea. Six months after initiation of hydroxyurea, clinical outcomes were assessed through the end of the study period: numbers of VOC‐related inpatient admissions and emergency department visits, and encounters for ACS. Multivariable Poisson models were used to predict outcomes by MPR quartile adjusting for previous healthcare utilization, state, and age. Results Hydroxyurea was initiated by 515 children. The median MPR was 0.53 (interquartile range = 0.3–0.8). The annual median number of visits was 0.0 for ACS, 1.3 for VOC‐related emergency department, and 1.4 for VOC‐related inpatient admissions. For each outcome, the highest quartile of MPR had the lowest predicted count; this difference was significant for ACS visits when compared with the lowest quartile of MPR. Conclusion This study demonstrated a high level of adherence (>75%) was essential to achieve a lower incidence of common negative clinical outcomes. Further, moderate and severe hydroxyurea nonadherence may be more common than previously appreciated among children, emphasizing the importance of developing and testing innovative strategies to increase adherence.

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Impact of Medicaid expansion on access and healthcare among individuals with sickle cell disease

Kayle

Valle

Paulukonis

et al. 2020

Pediatric Blood & Cancer

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Purpose Sickle cell disease (SCD) is associated with high acute healthcare utilization. The purpose of this study was to examine whether Medicaid expansion in California increased Medicaid enrollment, increased hydroxyurea prescriptions filled, and decreased acute healthcare utilization in SCD. Methods Individuals with SCD (≤65 years and enrolled in Medicaid for ≥6 total calendar months any year between 2011 and 2016) were identified in a multisource database maintained by the California Sickle Cell Data Collection Program. We describe trends and changes in Medicaid enrollment, hydroxyurea prescriptions filled, and emergency department (ED) visits and hospital admissions before (2011‐2013) and after (2014‐2016) Medicaid expansion in California. Results The cohort included 3635 individuals. Enrollment was highest in 2014 and lowest in 2016 with a 2.8% annual decease postexpansion. Although <20% of the cohort had a hydroxyurea prescription filled, the percentage increased by 5.2% annually after 2014. The ED visit rate was highest in 2014 and decreased slightly in 2016, decreasing by 1.1% annually postexpansion. Hospital admission rates were similar during the pre‐ and postexpansion periods. Young adults and adults had higher ED and hospital admission rates than children and adolescents. Conclusions Medicaid expansion does not appear to have improved enrollment or acute healthcare utilization among individuals with SCD in California. Future studies should explore whether individuals with SCD transitioned to other insurance plans or became uninsured postexpansion, the underlying reasons for low hydroxyurea utilization, and the lack of effect on hospital admissions despite a modest effect on ED visits.

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Hydroxyurea Initiation Among Children With Sickle Cell Anemia

Cited by 6 publications

References 55 publications

Sense of coherence or self-efficacy as predictors of health-related quality of life in sickle cell disease patients

Sense of coherence or self-efficacy as predictors of health-related quality of life in sickle cell disease patients

Adherence to hydroxyurea and clinical outcomes among children with sickle cell anemia

Impact of Medicaid expansion on access and healthcare among individuals with sickle cell disease

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