Acute Care Utilization at End of Life in Sickle Cell Disease: Highlighting the Need for a Palliative Approach

Johnston, Emily E.; Adesina, Oyebimpe O.; Alvarez, Elysia; Amato, Heather K.; Paulukonis, Susan; Nichols, Ashley; Chamberlain, Lisa; Bhatia, Smita

doi:10.1089/jpm.2018.0649

Cited by 16 publications

(17 citation statements)

References 41 publications

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“…Included cohorts were validated and followed over time using data from the California Sickle Cell Data Collection Program (CA SCDC) as previously described. 7 , 8 In brief, those included in CA SCDC must either have been identified through newborn screening, confirmed by laboratory analysis to have SCD at one of six SCD clinical centers in California, or found in administrative data with three or more SCD‐specific International Classification of Diseases‐Clinical Modification (ICD‐CM) codes over a 5‐year period. People meeting this definition were then linked, using Social Security numbers (SSN) and date of birth to (1) the Patient Hospital Discharge and (2) Emergency Department Utilization databases from the California Office of Statewide Health Planning and Development, (3) vital records death files, and (4) Medicaid (Medi‐Cal) claims.…”

Section: Methodsmentioning

confidence: 99%

“…Included cohorts were validated and followed over time using data from the California Sickle Cell Data Collection Program (CA SCDC) as previously described. 7,8 In brief, those included in CA SCDC must either have been identified through newborn screening, confirmed by laboratory analysis to have SCD at one of six SCD clinical centers in California, or found in administrative data with three or more SCD- We tracked utilization and discharge codes starting on the date of the first pharmacy fill claim of HU (HU Only cohort), DFX (DFX Only cohort), or whichever drug was started second in the Both Drugs cohort. To be included in the HU Only cohort or DFX Only cohort, the person had to be on the drug for at least 90 days, Table 1.…”

Section: Cohortsmentioning

confidence: 99%

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Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Wong

Valle

Paulukonis

2021

Health Science Reports

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Background and aims When patients with sickle cell disease have appropriate indications, they can be prescribed hydroxyurea (HU) and deferasirox (DFX) concurrently despite little knowledge about how the two medications interact. We wished to analyze whether there was evidence of adverse interaction between HU and DFX when taken simultaneously and hypothesized that those who took both drugs together had similar clinical complications when compared to those who took only one or neither drug. Methods We conducted this retrospective cohort investigation between 2009 and 2016 of persons with SCD in the California Sickle Cell Data Collection Program, a validated database of Californians with SCD a statewide. People in the database who took HU and DFX simultaneously for at least 3 months as compared to those who took either HU or DFX alone or to matched persons who took neither drug were eligible. Results We identified 104 people who were prescribed both HU and DFX concurrently, 877 who were prescribed HU only, and 314 who were prescribed DFX only during the study period. We identified 416 matched controls who took neither HU nor DFX. People who took both HU and DFX concurrently had similar rates of ED and inpatient encounters and had similar rates and distribution of adverse effects compared to those who took either HU or DFX alone or took neither drug. Conclusion Three months of concurrent use of DFX and HU appears safe, but further studies are required to better understand the safety and effectiveness of this medication combination. (Funded by CDC, CDC Foundation, and others).

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Section: Methodsmentioning

confidence: 99%

“…Included cohorts were validated and followed over time using data from the California Sickle Cell Data Collection Program (CA SCDC) as previously described. 7,8 In brief, those included in CA SCDC must either have been identified through newborn screening, confirmed by laboratory analysis to have SCD at one of six SCD clinical centers in California, or found in administrative data with three or more SCD- We tracked utilization and discharge codes starting on the date of the first pharmacy fill claim of HU (HU Only cohort), DFX (DFX Only cohort), or whichever drug was started second in the Both Drugs cohort. To be included in the HU Only cohort or DFX Only cohort, the person had to be on the drug for at least 90 days, Table 1.…”

Section: Cohortsmentioning

confidence: 99%

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Wong

Valle

Paulukonis

2021

Health Science Reports

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“…87 Understanding the patterns of morbidity, mortality, HRQoL, and health care utilization in individuals with SCD living in low-resource settings will be relevant in designing clinical trials with clinical trial end points that are impactful in these settings. [164][165][166] Table 9 summarizes suggested clinic trial end points that focus on reducing mortality in low-resource settings.…”

Section: Reducing Scd Mortality In Low-resource Settingsmentioning

confidence: 99%

End points for sickle cell disease clinical trials: renal and cardiopulmonary, cure, and low-resource settings

et al. 2019

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To address the global burden of sickle cell disease and the need for novel therapies, the American Society of Hematology partnered with the US Food and Drug Administration to engage the work of 7 panels of clinicians, investigators, and patients to develop consensus recommendations for clinical trial end points. The panels conducted their work through literature reviews, assessment of available evidence, and expert judgment focusing on end points related to patient-reported outcome, pain (non–patient-reported outcomes), the brain, end-organ considerations, biomarkers, measurement of cure, and low-resource settings. This article presents the findings and recommendations of the end-organ considerations, measurement of cure, and low-resource settings panels as well as relevant findings and recommendations from the biomarkers panel.

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“…Sickle cell disease (SCD), the most common inherited hematological disorder with a global birth prevalence of approximately 1-5 per 10,000 predominantly affects subjects of African origin [1] . The condition is associated with early mortality although improved healthcare has raised the life expectancy of patients with SCD to around 50 years [ 2 , 3 ]. The number of pregnant women with SCD is increasing along with this improvement in life expectancy.…”

Section: Introductionmentioning

confidence: 99%

Sickle cell disease and COVID-19 in pregnant women

Kolańska¹,

Vasileva²,

Lionnet³

et al. 2022

Journal of Gynecology Obstetrics and Human Reproduction

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Introduction The effect of coronavirus disease (COVID-19) on pregnancy outcome in women with sickle cell disease (SCD) is unknown. Objectives To analyze the severity of the SARS-CoV-2 infection in pregnant women with SCD and its impact on pregnancy. Methods This retrospective cohort study included SCD pregnant women tested positive for COVID-19 between March 2020 – February 2021. The primary endpoint was the severity of the COVID-19 infection. Secondary endpoints were pregnancy complications and fetal outcomes. Results During the study period among 82 pregnant women with SCD, 8 have presented symptoms suggestive of COVID-19 and were tested positive. A common mild clinical presentation was observed in 6 women (75%), one woman was asymptomatic and one required oxygen. The latter was admitted to the Intensive Care Unit and a cesarean section was performed in the context of an ongoing vaso-occlusive crisis and acute chest syndrome together with incidental preeclampsia. Labor was induced in another patient who developed a vaso-occlusive crisis after COVID-19 remission. Fetal outcomes were good with an average Apgar score of 10 and normal umbilical blood pH at birth. Two newborns were small-for-gestational-age as expected on the ultrasound follow-up before occurrence of COVID-19. Conclusion COVID-19 infection in our population of pregnant women with SCD had typical presentation and rarely triggered a sickle cell crisis or other complications. Fetal outcomes were good and did not seem to be directly influenced by the SARS-CoV-2 virus. Further studies are required to confirm these observations as compared to the population of women with SCD without COVID-19 infection.

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Acute Care Utilization at End of Life in Sickle Cell Disease: Highlighting the Need for a Palliative Approach

Cited by 16 publications

References 41 publications

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

Concurrent use of hydroxyurea and deferasirox in Californians with sickle cell disease

End points for sickle cell disease clinical trials: renal and cardiopulmonary, cure, and low-resource settings

Sickle cell disease and COVID-19 in pregnant women

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