Hydroxyurea use 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:10.1002/pbc.27721

Cited by 21 publications

(35 citation statements)

References 13 publications

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“…In analyses conducted using Medicaid claims data, fewer than 50% of adults were ever prescribed or initiated hydroxyurea, and only about 30% of those who initiated treatment achieved adequate adherence levels [ 16 - 19 ]. Among children, adherence was higher; however, the number of children who were prescribed hydroxyurea was low [ 20 - 22 ]. Barriers to prescribing hydroxyurea include providers’ reluctance due to lack of knowledge about the drug and appropriate dosing, low patient acceptance due to insufficient knowledge or misconceptions about risks and benefits, and forgetfulness leading to poor adherence [ 14 , 15 , 20 , 23 - 28 ].…”

Section: Introductionmentioning

confidence: 99%

Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Protocol for an Efficacy and Implementation Study

et al. 2020

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Background Hydroxyurea prevents disease complications among patients with sickle cell disease (SCD). Although its efficacy has been endorsed by the National Health Lung and Blood Institute evidence-based guidelines, its adoption is low, both by patients with SCD and providers. Mobile health (mHealth) apps provide benefits in improving medication adherence and self-efficacy among patients with chronic diseases and have facilitated prescription among medical providers. However, mHealth has not been systematically tested as a tool to increase hydroxyurea adherence nor has the combination of mHealth been assessed at both patient and provider levels to increase hydroxyurea utilization. Objective This study aims to increase hydroxyurea utilization through a combined two-level mHealth intervention for both patients with SCD and their providers with the goals of increasing adherence to hydroxyurea among patients and improve hydroxyurea prescribing behavior among providers. Methods We will test the efficacy of 2 mHealth interventions to increase both patient and provider utilization and knowledge of hydroxyurea in 8 clinical sites of the NHLBI-funded Sickle Cell Disease Implementation Consortium (SCDIC). The patient mHealth intervention, InCharge Health, includes multiple components that address memory, motivation, and knowledge barriers to hydroxyurea use. The provider mHealth intervention, Hydroxyurea Toolbox (HU Toolbox), addresses the clinical knowledge barriers in prescribing and monitoring hydroxyurea. The primary hypothesis is that among adolescents and adults with SCD, adherence to hydroxyurea, as measured by the proportion of days covered (the ratio of the number of days the patient is covered by the medication to the number of days in the treatment period), will increase by at least 20% after 24 weeks of receiving the InCharge Health app, compared with their adherence at baseline. As secondary objectives, we will (1) examine the change in health-related quality of life, acute disease complications, perceived health literacy, and perceived self-efficacy in taking hydroxyurea among patients who use InCharge Health and (2) examine potential increases in the awareness of hydroxyurea benefits and risks, appropriate prescribing, and perceived self-efficacy to correctly administer hydroxyurea therapy among SCD providers between baseline and 9 months of using the HU Toolbox app. We will measure the reach, adoption, implementation, and maintenance of both the InCharge Health and the HU Toolbox apps using the reach, effectiveness, adoption, implementation, and maintenance framework and qualitatively evaluate the implementation of both mHealth interventions. Results The study is currently enrolling study participants. Recruitment is anticipated to be completed by mid-2021. Conclusions If this two-level intervention, that is, the combined use of InCharge Health and HU Toolbox apps, demonstrates efficacy in increasing adherence to hydroxyurea and prescribing behavior in patients with SCD and their providers, respectively, both apps will be offered to other institutions outside the SCDIC through a future large-scale implementation-effectiveness study. Trial Registration ClinicalTrials.gov NCT04080167; https://clinicaltrials.gov/ct2/show/NCT04080167 International Registered Report Identifier (IRRID) DERR1-10.2196/16319

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Section: Introductionmentioning

confidence: 99%

Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Protocol for an Efficacy and Implementation Study

et al. 2020

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“…Hydroxyurea is an oral medication, administered once/day. Despite its clinical benefits, adherence in pediatric and adult patients is relatively low, ranging from 18%–35% 35, 36 . L‐glutamine is a twice/day oral drug, and adherence is usually lower than a once/day dosing regimen 37 .…”

Section: Discussionmentioning

confidence: 99%

Systematic Review of Crizanlizumab: A New Parenteral Option to Reduce Vaso‐occlusive Pain Crises in Patients with Sickle Cell Disease

Han

Saraf

2020

Pharmacotherapy

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Hydroxyurea, indicated for managing sickle cell anemia (SCA), and L-glutamine, indicated for treating sickle cell disease (SCD), were the only pharmacotherapeutic options in this patient population before the approval of crizanlizumab by the U.S. Food and Drug Administration in November 2019 to reduce vaso-occlusive crisis (VOC) frequency. This article reviews the evidence pertaining to crizanlizumab in SCD by searching records in Medline, Embase, and International Pharmaceutical Abstracts. Crizanlizumab, a P-selectin inhibitor, mitigates the microvascular vaso-occlusion in SCD. In the multicenter randomized double-blind SUSTAIN trial, a higher dose of crizanlizumab decreased the incidence of VOCs by 45% and prolonged the median time to the first and second VOC. A post hoc subgroup analysis demonstrated that the proportion of patients who had no VOC incidence during the study period was greater in the crizanlizumab group, and this benefit was consistent regardless of concomitant hydroxyurea use, prior categorized history of VOC frequency, or SCD genotype. Crizanlizumab had a safety profile comparable with placebo. Multiple ongoing clinical trials are trying to establish its roles in pediatric patients with SCD and its effects on alleviating other SCD-related complications. As the first parenteral option for SCD, providers need to formulate administration logistics to improve patients' access to crizanlizumab. Current available data suggest crizanlizumab is a promising agent to reduce VOC in patients with SCD.

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“…This difference likely explains why there was such a high proportion of children at baseline who had been offered HU and referred for a TCD compared to other studies. 5 , 6 …”

Section: Discussionmentioning

confidence: 99%

“…For example, based on Medicaid administrative data from 6 states, only 18% of children with SCA were noted to have consistent HU therapy, based on prescription refill records. 5 Also, annual TCD screening rates for children with SCA are estimated to range from only 22% to 44%. 6 …”

Section: Introductionmentioning

confidence: 99%

Improving Preventive Care for Children With Sickle Cell Anemia: A Quality Improvement Initiative

Cabana

Marsh

Treadwell

et al. 2020

Pediatric Quality &Amp; Safety

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Introduction: Sickle cell disease is a complex chronic disorder associated with increased morbidity and early mortality. The Pediatric Quality Measures Program has developed new sickle cell-specific quality measures focused on hydroxyurea (HU) counseling and annual transcranial Doppler (TCD) screening; however, these measures have not been used in a clinical setting to inform quality improvement (QI) efforts. Methods: From 2017 to 2018, 9 sickle cell subspecialty clinics from the Pacific Sickle Cell Regional Collaborative conducted a year-long QI collaborative focused on improving the percentage of patients with HU counseling and TCD screening based on the new quality measures. After an initial kick-off meeting, the 9 sites participated in monthly conference calls. We used run charts annotated with plan-do-study-act cycle activities to track each site’s monthly progress and the overall mean percentage for the entire collaborative. Results: There was an overall improvement in the aggregate HU counseling from 85% to 98% (P < 0.01). For TCD screening, referral frequency changed from 85% to 90% (P = 0.76). For both measures, the variation in frequencies decreased over the year. Conclusion: Over 1 year, we found that a regional QI collaborative increased HU counseling. Although referral for TCD screening increased, there was no overall change in TCD completion. Overall, this QI report’s findings can help clinicians adopt and implement these quality measures to improve outcomes in children.

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Hydroxyurea use among children with sickle cell anemia

Cited by 21 publications

References 13 publications

Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Protocol for an Efficacy and Implementation Study

Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Protocol for an Efficacy and Implementation Study

Systematic Review of Crizanlizumab: A New Parenteral Option to Reduce Vaso‐occlusive Pain Crises in Patients with Sickle Cell Disease

Improving Preventive Care for Children With Sickle Cell Anemia: A Quality Improvement Initiative

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