Higher hydroxyurea adherence among young adults with sickle cell disease compared to children and adolescents

Reddy, Paavani S; Cai, Stephanie; Barrera, Leonardo; King, Kathryn M.; Badawy, Sherif M.

doi:10.1080/07853890.2022.2044509

Cited by 8 publications

(9 citation statements)

References 59 publications

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“…Additionally, poor treatment adherence is one of the most important and changeable factors to ensure treatment effectiveness ( 19 ). Several studies emphasized the lack of medication adherence among patients with SCD, as Zhou et al claims study showing that the median adherence to HU varied between age classes, and was the lowest among young adults, with around 30% ( 20 , 21 ). Due to the nature of the database, it was not possible to distinct cases when HU is not prescribed – reflecting prescription patterns – from cases when HU is prescribed but not dispensed – reflecting adherence itself.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Prevalence and cost of sickle cell disease in France: real-world analysis using data from the Echantillon Généraliste des Bénéficiaires

Beillat,

Durand-Zaleski,

Pirenne

et al. 2023

Front. Public Health

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Sickle cell disease (SCD) is a genetic disorder of the hemoglobin resulting in chronic anemia, hemolysis, and vaso-occlusions. Its treatment mostly relies on hydroxycarbamide, transfusions, and stem cell transplantation. This study aimed at describing the epidemiology and management of SCD in adolescent and adult patients in France. This was a retrospective study performed among SCD patients aged ≥12 years between 2016 and 2018 and controls. SCD patients were matched on a 1:3 ratio with a group of individuals with no diagnosis of SCD, referred as control group. The matching of SCD patients and controls was a direct matching based on age, sex, CMU-c status (which corresponds to free-of-charge complementary coverage for people with low resources) and geographical region of residence. SCD patients and their matched controls were followed-up for the same amount of time by adjusting controls’ follow-up period to that of the associated patients. This study used claims data from the French representative 1/97th sample of health data system. The main outcomes were the patients’ characteristics and treatments received, healthcare consumptions and related costs among SCD cases and controls. Between 2016 and 2018, 151 patients with ≥6 months of follow-up were identified out of the total population of 732,164 individuals. SCD prevalence extrapolated to the entire population [95% CI] was 19,502 [19,230, 19,778] in 2018. The median (Q1–Q3) age at inclusion date was 37.0 (25.0–48.0) years, with 69.5% of patients being female. The mean (SD) reimbursed cost over follow-up was €24,310 (89,167), mostly represented by hospitalization costs accounting for €21,156 (86,402). A switch in SCD management was observed with age, as younger patients presented more frequent hospitalizations and acute procedures, while older ones had more frequent medical visits and paramedical care. Mean (SD) annual costs were €25,680 (91,843) and vs. €3,227 (23,372) for patients and controls, respectively (p < 0.001), representing an extra cost of almost €150 million over the entire SCD population. This study highlighted the important costs related to SCD and the related medical need with treatment alternatives, which could be filled by the emergence of new therapies.

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Section: Discussionmentioning

confidence: 99%

“…CMUc corresponds to free-of-charge complementary coverage for people with low resources. (20,21). Due to the nature of the database, it was not possible to distinct cases when HU is not prescribedreflecting prescription patterns -from cases when HU is prescribed but not dispensed -reflecting adherence itself.…”

Section: Patient Characteristicsmentioning

confidence: 99%

Prevalence and cost of sickle cell disease in France: real-world analysis using data from the Echantillon Généraliste des Bénéficiaires

Beillat,

Durand-Zaleski,

Pirenne

et al. 2023

Front. Public Health

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“…12 Hydroxyurea (HU) is approved by the US Food and Drug Administration (FDA) for SCD given the strong evidence for safety and efficacy, [13][14][15][16] with the National Heart, Lung, and Blood Institute recommending it starting at 9 months of age. 17 Treatment with HU reduces hospitalization rates, 18,19 decreases number of VOCs, 15 and improves HRQoL for people with SCD. 20 There has been significant advancement in the field with the recent FDA approval of L-glutamine in 2017 as well as crizanlizumab, and voxelotor in 2019 for SCD.…”

Section: Introductionmentioning

confidence: 99%

Barriers to medication adherence in sickle cell disease: A comprehensive theory‐based evaluation using the COM‐B model

King

Cai

Barrera

et al. 2023

Pediatric Blood & Cancer

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Background: Sickle cell disease (SCD) affects more than 100,000 Americans, with complications such as pain episodes and acute chest syndrome. Despite the efficacy of hydroxyurea in reducing these complications, adherence remains low. Study objectives were to examine barriers to hydroxyurea adherence, and to evaluate the relationship between barriers and their impact on adherence. Methods:In this cross-sectional study, patients with SCD and their caregivers were enrolled if they were taking hydroxyurea. Study measures included demographics, self-report of adherence using visual analog scale (VAS), and the Disease Management and Barriers Interview (DMI)-SCD. The DMI-SCD was mapped to the Capability, Opportunity, Motivation, and Behavior (COM-B) model. Results:Forty-eight caregivers (females 83%, median age 38 [34][35][36][37][38][39][40][41][42][43]) and 19 patients (male 53%, median age 15 [13.5-18]) participated. Using VAS, many patients (63%) reported low hydroxyurea adherence, while most caregivers (75%) reported high adherence. Caregivers endorsed barriers across multiple COM-B components, with physical opportunity (e.g., cost) and reflective motivation (e.g., SCD perceptions) being the most identified categories (48% and 42%), respectively. Patients' most identified barriers included psychological capability (e.g., forgetfulness) and reflective motivation (84% and 68%), respectively. Patients' and caregivers' VAS scores negatively correlated with the number of barriers (r s = -.53, p = .01; r s = -.28, p = .05) and COM-B categories

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“…3 Factors such as disease severity and non-adherence to disease-modifying therapies, such as hydroxyurea, contribute to the frequency of VOEs. [4][5][6][7] VOEs are the leading cause of emergency department (ED) visits and hospitalizations, and greatly impact the quality of life for individuals with SCD. 8,9 The main strategies for managing a VOE are supportive care with intravenous fluid (IVF) therapy and analgesics coupled with non-pharmacological approaches.…”

Section: Introductionmentioning

confidence: 99%

“…VOEs can last for several hours to days and, over time, can lead to chronic pain and disability, 2 leading to a significant economic burden across a patient's lifespan 3 . Factors such as disease severity and non‐adherence to disease‐modifying therapies, such as hydroxyurea, contribute to the frequency of VOEs 4–7 . VOEs are the leading cause of emergency department (ED) visits and hospitalizations, and greatly impact the quality of life for individuals with SCD 8,9 .…”

Section: Introductionmentioning

confidence: 99%

Intravenous fluid therapy and hospital outcomes for vaso‐occlusive episodes in children, adolescents, and young adults with sickle cell disease

Haimed,

Weiss,

Kwon

et al. 2023

Pediatric Blood & Cancer

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BackgroundWhile intravenous fluid (IVF) therapy in patients with sickle cell disease (SCD) admitted for a vaso‐occlusive episode (VOE) can help reduce red blood cell sickling, clinical practice varies across institutions. We examined the relationship between IVF therapy and hospital length of stay (HLOS), as well as adverse events, such as acute chest syndrome (ACS), pediatric intensive care unit (PICU) transfer, and 28‐day re‐admission.MethodsThis is a single‐center retrospective analysis of SCD VOE hospitalizations between January 2015 and April 2020. Patients with SCD, age 0–30, with consecutive hospitalizations for VOE were included. For the first 3 days of each admission, an “IVF ratio” was calculated by dividing actual IVF rate administered by weight‐based maintenance IVF (mIVF) rate.ResultsA total of 617 hospitalizations for 161 patients were included. Mean HLOS was 5.7 days, (SD 3.9), and mean IVF volume over the first 3 days of admission was 139.6 mL/kg/day (SD 57.8). Multivariate analysis showed that for each additional 0.5 times the mIVF rate, HLOS increased by 0.53 day (p < .001; 95% confidence interval [CI]: 0.609–0.989), but there was no significant association between IVF therapy and adverse events. History of chronic pain was associated with increased odds of re‐admission (OR 6.4; 95% CI: 3.93–10.52).ConclusionsDespite the theoretical potential for IVF therapy to slow down the sickling process, our findings suggest that increased IVF therapy was associated with prolonged HLOS, which places a burden on patients, families, and the health system.

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Higher hydroxyurea adherence among young adults with sickle cell disease compared to children and adolescents

Cited by 8 publications

References 59 publications

Prevalence and cost of sickle cell disease in France: real-world analysis using data from the Echantillon Généraliste des Bénéficiaires

Prevalence and cost of sickle cell disease in France: real-world analysis using data from the Echantillon Généraliste des Bénéficiaires

Barriers to medication adherence in sickle cell disease: A comprehensive theory‐based evaluation using the COM‐B model

Intravenous fluid therapy and hospital outcomes for vaso‐occlusive episodes in children, adolescents, and young adults with sickle cell disease

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