Changes in Hydroxyurea Use Among Youths Enrolled in Medicaid With Sickle Cell Anemia After 2014 Revision of Clinical Guidelines

Reeves, Sarah L.; Peng, Hannah K; Wing, Jeffrey J.; Cogan, Lindsay; Goel, Ankit; Anders, David G.; Green, Nancy; Lisabeth, Lynda D.; Dombkowski, Kevin J.

doi:10.1001/jamanetworkopen.2023.4584

Cited by 5 publications

(1 citation statement)

References 47 publications

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“…12 However, HU adherence, measured using HU proportion of days covered (HU-PDC) typically is better in the early childhood and worsens as children age and enter adolescence. 18 Based on national guidelines, we examined the prevalence of TCD screening in North Carolina (NC) to determine whether TCD screenings are associated with sociodemographic factors (SDF) and rate of HU adherence.…”

Section: Introductionmentioning

confidence: 99%

Association of hydroxyurea adherence with transcranial Doppler screenings in children with sickle cell disease

Knight,

Tanabe,

Blewer

et al. 2024

Pediatric Blood & Cancer

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BackgroundNational sickle cell disease (SCD) guidelines recommend oral hydroxyurea (HU) starting at 9 months of age, and annual transcranial Doppler (TCD) screenings to identify stroke risk in children aged 2–16 years. We examined prevalence and proportion of TCD screenings in North Carolina Medicaid enrollees to identify associations with sociodemographic factors and HU adherence over 3 years.Study designWe conducted a longitudinal study with children ages 2–16 years with SCD enrolled in NC Medicaid from years 2016–2019. Prevalence of TCD screening claims was calculated for 3 years, and proportion was calculated for 12, 24, and 36 months of Medicaid enrollment. Enrollee HU adherence was categorized using HU proportion of days covered. Multivariable Poisson regression assessed for TCD screening rates by HU adherence, controlling for age, sex, and rurality.ResultsThe prevalence of annual TCD screening was between 39.5% and 40.1%. Of those with 12‐month enrollment, 77.8% had no TCD claims, compared to 22.2% who had one or higher TCD claims. Inversely, in children with 36 months of enrollment, 36.7% had no TCD claims compared to 63.3% who had one or higher TCD claims. The proportion of children with two or higher TCD claims increased with longer enrollment (10.5% at 12 months, 33.7% at 24 months, and 52.6% at 36 months). Children with good HU adherence were 2.48 (p < .0001) times more likely to have TCD claims than children with poor HU adherence.ConclusionWhile overall TCD screening prevalence was low, children with better HU adherence and longer Medicaid enrollment had more TCD screenings. Multilevel interventions are needed to engage healthcare providers and families to improve both evidence‐based care and annual TCD screenings in children with SCD.

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Section: Introductionmentioning

confidence: 99%

Association of hydroxyurea adherence with transcranial Doppler screenings in children with sickle cell disease

Knight,

Tanabe,

Blewer

et al. 2024

Pediatric Blood & Cancer

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Social Vulnerability and Sickle Cell Disease Mortality in the US

Tan,

San,

Yeo

et al. 2024

JAMA Netw Open

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ImportanceSocial determinants of health (SDOH) influence health outcomes, including those of sickle cell disease (SCD), despite advancements in treatments like disease-modifying therapies.ObjectiveTo investigate the association of SDOH with SCD mortality rates from 2016 to 2020.Design, Setting, and ParticipantsThis cross-sectional study combined county-level data from the Centers for Disease Control and Prevention and Agency for Toxic Substances and Disease Registry Social Vulnerability Index (SVI) with SCD mortality data from the Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiologic Research database from January 1, 2016, to December 31, 2020. US counties were divided into 4 quartile (Q) models according to their SVI scores. Deaths from SCD in the US among patients of all ages were included. Data analysis occurred from March to April 2024.ExposureSVI score.Main Outcomes and MeasuresAge-adjusted mortality rates (AAMRs) per 1 000 000 individuals were measured. Rate ratios (RRs) were obtained by comparing county-specific AAMRs of SVI-Q4 with SVI-Q1.ResultsFrom 2016 to 2020, among a total population of 1 633 737 771 individuals, there were 2635 deaths from SCD (1289 male [49.1%] and 1336 female [50.9%]). There were 1480 deaths in Q4, 687 deaths in Q3, 344 deaths in Q2, and 114 deaths in Q1. Higher SVI was associated with 2.11 excess deaths per 1 000 000 individuals (RR, 4.90; 95% CI, 4.81-5.00). Similar trends were seen for both males (RR, 4.56; 95% CI, 4.44-4.69) and females (RR, 5.85; 95% CI, 5.68-6.03). Middle-aged patients with SCD had the highest mortality rate in Q4, with 3.45 excess deaths per 1 000 000 individuals (RR, 4.97; 95% CI, 4.85-5.09). Higher SVI was associated with 2.29 excess deaths per 1 000 000 individuals in African American individuals with SCD (RR, 1.24; 95% CI, 1.22-1.27]). In White individuals with SCD, higher SVI was associated with 0.12 excess deaths per 1 000 000 individuals (RR not available due to unreliable data in Q1). When stratifying by census region, the highest level of SCD-related mortality was in the Northeast, with higher SVI associated with 3.16 excess deaths per 1 000 000 individuals (RR, 8.02; 95% CI, 7.66-8.40).ConclusionsIn this cross-sectional study of the association of SVI with SCD mortality rates, higher SVI was associated with higher SCD mortality across US counties. These findings underscore the importance of addressing social determinants of health to improve mortality outcomes among patients with SCD.

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CRISPR/Cas9 in the treatment of sickle cell disease (SCD) and its comparison with traditional treatment approaches: a review

Tariq,

Khurshid,

Khan

et al. 2024

Annals of Medicine &Amp; Surgery

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Sickle cell disease (SCD) is a common hereditary blood disorder that profoundly impacts individuals’ health, causing chronic pain, anemia, organ damage, increased susceptibility to infections, and social and psychological effects. Over the years, advances in treatment have improved the long-term outcomes of SCD patients. However, problems such as limited access to hematopoietic stem cell transplantation (HSCT) and potential complications associated with the available therapies underscore the importance of continued research and development. The recent FDA approval of Casgevy (Exagamglogene autotemcel), a genetic therapy based on CRISPR/Cas9 technology, demonstrates a comprehensive effort to address the complexity of SCD using new technologies. This review explores the potential of CRISPR/Cas9 for treating SCD and evaluates its efficacy, safety, and long-term outcomes compared to traditional treatment approaches. Long-term research is needed to comprehensively assess the safety, effectiveness, and inclusion of CRISPR/Cas9, ensuring its overall efficacy.

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Changes in Hydroxyurea Use Among Youths Enrolled in Medicaid With Sickle Cell Anemia After 2014 Revision of Clinical Guidelines

Cited by 5 publications

References 47 publications

Association of hydroxyurea adherence with transcranial Doppler screenings in children with sickle cell disease

Association of hydroxyurea adherence with transcranial Doppler screenings in children with sickle cell disease

Social Vulnerability and Sickle Cell Disease Mortality in the US

CRISPR/Cas9 in the treatment of sickle cell disease (SCD) and its comparison with traditional treatment approaches: a review

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