Sickle Cell Disease Hospitalizations Research Articles

EE117 Insights Into Sickle Cell Disease Hospitalizations and Outcomes: A Tale of 2 HCUP Databases

EE117 Insights Into Sickle Cell Disease Hospitalizations and Outcomes: A Tale of 2 HCUP Databases

Short-term air pollution levels and sickle cell disease hospital encounters in South Carolina: A case-crossover analysis

BackgroundSickle cell disease (SCD) is a genetic disorder and symptoms may be sensitive to environmental stressors. Although it has been hypothesized that exposure to outdoor air pollution could trigger acute SCD events, evidence is limited. MethodsWe obtained SCD administrative data on hospital encounters in South Carolina from 2002 to 2019. We estimated outdoor air pollutant (particulate matter<2.5 μm (PM2.5), ozone (O3), and PM2.5 elemental carbon (EC) concentrations at residential zip codes using spatio-temporal models. Using a random bi-directional, fixed-interval case-crossover study design, we investigated the relationship between air pollution exposure over 1-, 3-, 5-, 9-, and14-day periods with SCD hospital encounters. ResultsWe studied 8410 patients with 144,129 hospital encounters. We did not observe associations among all patients with SCD and adults for PM2.5, O3, and EC. We observed positive associations among children for 9- and 14-day EC (OR: 1.05 (95% confidence interval (CI): 1.02, 1.08) and OR: 1.05 (95% CI: 1.02, 1.09), respectively) and 9- and 14-day O3 (OR: 1.04 (95%CI: 1.00, 1.08)) for both. ConclusionsOur findings suggest that short-term (within two-weeks) levels of EC and O3 and may be associated with SCD hospital encounters among children. Two-pollutant model results suggest that EC is more likely responsible for effects on SCD than O3. More research is needed to confirm our findings.

Patterns of Blood Transfusion in Sickle Cell Disease Hospitalizations

Background: Transfusional iron overload causes significant morbidity and mortality in sickle cell disease (SCD). Nevertheless, red blood cell transfusions continue to be essential in its management. This study describes the transfusion patterns among SCD hospitalizations. Methods: Hospitalizations for SCD in the 2017–2018 Nationwide Readmissions Database were divided into two groups based on whether they received transfusions. Descriptive analysis was performed to compare their demographics and complications. Multivariable logistic regression was performed to determine the factors associated with transfusions. Results: Out of 109,783 hospitalizations, 28,300 were transfused, and 81,483 were not transfused. Females and older individuals were higher in the transfused category than the non-transfused category (59.49% vs. 53.52% and 28.86% vs. 21.27%, respectively; p &lt; 0.001 for both). The wealthiest population was more likely to be in the transfused category (11.27% vs. 8.34%; p &lt; 0.001). Admissions to teaching hospitals, large metropolitan hospitals, and highest-volume hospitals were higher in the non-transfused category vs. transfused category (79.89% vs. 72.17%; p &lt; 0.001, 69.26% vs. 65.35%; p 0.003 and 74.71% vs. 63.51%; p &lt; 0.001, respectively). Most admissions were transfused once, with three or more transfusions being given more in the non-teaching hospitals than the teaching hospitals (1.27% vs. 0.41%; p 0.01). Furthermore, a higher proportion of early transfusions occurred in the non-teaching hospitals (65.6% vs. 57.82% for admission days 1 and 2; p &lt; 0.001). Admission to a teaching hospital was associated with lower blood transfusion odds than a non-teaching hospital. Conclusion: A quarter of admissions for SCD receive a blood transfusion. In addition to performing more frequent and early transfusions, the odds of being transfused are higher in non-teaching hospitals.

Delayed Management of Sickle Cell Disease Vaso-Occlusive Episodes and the Impact on Prolonged Hospitalizations at a Single Academic Center

Introduction: Sickle Cell Disease (SCD) affects nearly 100,000 people in the United States (Centers for Disease Control and Prevention, 2023). Vaso-occlusive episode (VOE) is the most common reason for hospitalization in SCD. Many providers are unfamiliar with the pain management timing and dosages required to adequately treat these episodes (Jang et al., J Transl Med 2021). Nationally, areas with comprehensive sickle cell centers have shown decreased hospital utilization, fewer VOEs, and improved quality of life (Oyedeji et al. JAMA 2020). Even in areas without a comprehensive center, implementing individualized pain care plans for SCD has reduced admission rates of high-utilizing patients while not impacting their pain rating scores (Welch-Coltrane et al. Pain Medicine 2021). Our academic hospital, Oregon Health and Science University (OHSU), lacks a comprehensive SCD center and VOE treatment plans are not easily accessible for these patients. We conducted a retrospective analysis on a cohort of patients with SCD to investigate the factors influencing the length of emergency department (ED) and hospital stays for VOE. We aim to identify if implementing individualized pain plans via a standardized template within the electronic health record (EHR) for these patients will decrease hospital admission, length of stay and time to ED discharge. We also aim to integrate individualized pain plans into the EHR that would allow for direct exposure to the care team by creating plans that are easily accessible, reviewed, and routinely utilized for clinical decision-making. Methods: We performed a preliminary retrospective analysis of encounters (n=107) from 2008-2023 at OHSU, where the primary diagnosis was either a VOE or an equivalent diagnosis. Based on data demonstrating earlier maximum opioid doses leading to shortened hospital stays in frequent pain patients, we recorded the timing of the first opioid and morphine milliequivalents per hour in the first 24 hours (Payne et al. Pediatr Blood Cancer 2018). Additionally, we recorded the length of stay, admission rates, time to first IV fluid, and the type of IV fluid used. We performed a linear regression analysis on the collected data with the dependent variable as the length of stay across the independent variables of time to first opioid, time to first intravenous fluid, and morphine milliequivalents per hour during the first 24 hours after presentation. Results: Eighteen patients accounted for the 107 analyzed encounters. Thirteen patients (72%) had Hgb-SS disease, 2 (11%) had Hgb-SC disease, and 3 (17%) had Hgb-S-beta thalassemia. Sixty-two admissions (58%) were ED discharges, and 45 (42%) were ED to hospital admissions. The length of stay and time to the first opioid positively correlated with p=0.005 (Figure 1a). The length of stay and time to first intravenous fluid administration was also positively correlated with p=0.01. The length of stay and morphine milliequivalents per hour in the first 24 hours were negatively correlated with p&amp;lt;0.0001 (Figure 1b). Conclusions: Longer time to administration of opioids and intravenous fluids correlated with a longer length of stay in both the ED and the hospital. Additionally, lower morphine milliequivalents in the first 24 hours of presentation were associated with a prolonged hospital stay at our institution. We hypothesize that these three factors are secondary to delays in care and inappropriate initial treatment of VOE. To address these factors, we aim to implement standardized pain plans in the EHR of our institution. Implementation of individualized pain plans, particularly in the ED, has led to the earlier initiation of opioids (Della-Moretta et al. Ann Emerg Med 2020). We anticipate similar effectiveness for timely and appropriate fluid administration and adequate opioid dosing. We plan to continue retrospective analysis and compare this data to VOE encounters post-implementation. By implementing individualized plans that are integrated within the EHR and efficiently utilized by the care team, we hope to improve VOE management at our institution with decreased length of stay, less ED to hospital admissions, and improved patient satisfaction.

Knowing the Dose to Control the Dose: Optimizing Patient-Controlled Analgesia in Adults with Sickle Cell Disease (SCD)

Introduction: Acute painful episodes are the most common cause of hospitalization in SCD. Intravenous (IV) opioids are the analgesic of choice to treat pain requiring hospitalization in adults. On-demand IV opioid dosing can be either via a patient-controlled analgesia (PCA) pump or via intermittent nurse bolus. Advantages of PCA include better analgesia accessibility and relative independence from staff. However, significant variability exists in PCA management. A multicenter, randomized-controlled trial of PCA strategies in SCD was terminated early due to slow accrual, stemming from barriers such as suboptimal communication between inpatient teams. Additional limitations with PCA include technological limitations and incomplete charting through a manual process. We initiated a quality improvement project to understand patterns of PCA use in patients hospitalized for SCD pain crises at our institution. Methods: We performed a retrospective manual chart review on all adult patients diagnosed with SCD pain crises admitted to the University of Cincinnati Medical Center (UCMC) from October 1 through December 31, 2022. Inpatient data collected included reason for admission, length of stay, and documentation of inpatient opioid usage in daily clinical notes. Hospitalizations requiring intensive care admission for severe complications were excluded. Outpatient data collected included demographics and outpatient opioid prescriptions via the state prescription drug monitoring database. The study was approved by the UCMC IRB. Results: Fifteen patients were admitted to UCMC a total of 24 times for SCD pain crises during the 3-month period. The patients ranged in age from 21 years to 73 years (median age 33 years, interquartile range (IQR) 19.5 years) and the majority (11/15) were women. Disease-modifying therapies prescribed included hydroxyurea (8/15, 53%), chronic transfusions (2/15, 13%), chronic transfusions plus hydroxyurea (1/15), voxelotor (1/15), no disease-modifying therapy (2/15). Nine patients were prescribed daily outpatient opioids that included an oral long-acting opioid plus an oral short-acting opioid. Out of the 24 hospitalizations, 11 were for an uncomplicated pain crisis, 10 were complicated by mild acute chest syndrome (mild hypoxia and/or abnormal chest imaging), and 3 patients experienced other treatable complications (2 with Staphylococcus epidermidis bacteremia, 1 with deep venous thrombosis). Five patients were admitted two or more times. Two patients, admitted a total of 5 times, were maintained on nurse-administered IV bolus opioids during their admissions, in addition to oral opioids. The remainder of the patients were treated with a hydromorphone PCA pump with on-demand bolus dosing only, in addition to home long-acting oral opioids when applicable. Two patients were ultimately transitioned from PCA onto nurse-administered bolus IV opioids, due to failure to achieve adequate analgesia with PCA. In 9 out of the 19 admissions using a PCA, daily MME or the equivalent was charted in at least one progress note (47%). The median LOS in the subgroup in which PCA use was charted at least once was 7 (IQR 3.5) days. In the 10 admissions on a PCA in which daily MME was never charted (53%), the median LOS was 11 (IQR 6.25) days. Among patients not receiving outpatient opioids, daily MMEs from the PCA in the first few days of hospitalization (when recorded) ranged from 24 to 520 (median 178, IQR 241) MME. In patients who had daily chronic pain on outpatient opioid therapy, daily inpatient MME from PCA ranged from 360 to 1020 (median 545, IQR 418) MME. In patients with multiple admissions during the study period, the recorded daily opioid use was about two times that in the single admission group (median 673, IQR 500 MME in the multiple admissions group; vs median 329, IQR 351 MME in the single admission group). Conclusions: Our data suggest that length of stay is shortened by ~4 days when clinicians quantify daily opioid use for patients on PCA. In addition, patients with frequent admissions tend to have higher inpatient daily opioid use. This hints that readmission may be partly driven by mild withdrawal when on outpatient opioids at lower MME. A major barrier to data collection was inconsistent documentation of daily opioid use. The next step in our quality improvement project is an educational curriculum on inpatient opioid management for residents and hospitalists.

Impact of Psychiatric Comorbidity on Readmissions of Sickle Cell Disease -a Nationwide Readmissions Database Study

Introduction: While advances in the management of sickle cell disease (SCD) treatment have improved outcomes, readmission rates among patients remain a significant concern. A previous study with the National Readmission Database (NRD) 2016 found that alcohol use, tobacco use, opoid use ,substance use and depression were associated with SCD readmission even though the general rate of readmission has decreased over the years. This study aims to update the literature, and further determine the predictors associated with increased readmission among individuals with SCD. Methods: Using the Nationwide Readmissions Database (NRD), we evaluated readmissions for SCD for the year 2016-2020. On the basis of International Classification of Diseases, TenthRevision, Clinical Modification (ICD-10-CM) codes, we identified SCD hospitalizations (D57.0, D57.1, D57.2, D57.4, D57.8), and comorbid conditions. Usingamultivariateregressionanalysis,weestimatedtheindependent predictors of 30-day readmissions of SCD. We used STATA® Version 18.0 Software (STATACORP, TEXAS, USA) for analysis. The p-value was set at p &amp;lt; 0.05 for statistical significance. Results: A total of 262,571 index admissions for adult patients with a primary diagnosis of SCD were admitted within the study period; 61,687 patients (23.6%) were readmitted within 30 days. A total of 886 and 105 deaths were reported respectively among index admissions and readmissions. Compared to index admissions, readmissions had decreased THC of 2147 U.S. dollars (95%CI: -2678 - -1615), while we found no significant change in the LOS between index admissions and readmissions. Common causes of increased rate of readmissions were SCD with mood disorder (HR=1.15, 95%CI: 1.11-1.20) , anxiety disorder (HR= 1.16, 95%CI: 1.11-1.20) and opioid use (HR=1.42, 95%CI: 1.35-1.50). Conditions associated with lower rates of readmission are Alcohol use disorder (HR 0.76, 95%CI: 0.62-0.94) and cannabis use ( HR=0.94, 95%CI: 0.88-0.99). Discussion: Our findings suggest that among individuals admitted with a primary diagnosis of SCD, having concurrent mood and anxiety disorders, and opioid use disorder increases rates of readmission. Interestingly, we observed that alcohol use disorder and cannabis use was associated with lower rates of readmission in SCD patients. Optimizing the comorbid conditions in patients with SCD may reduce readmission rates. However, further studies are required to establish the relationship between psychiatric comorbidity and healthcare resources utilization among SCD patients.

Characterizing the Immature Immunophenotype of Sickle Cell Disease Monocytes during Vaso-Occlusive Crisis

Sickle cell disease (SCD) is marked by episodic vaso-occlusive crisis (VOC). Painful VOC is the leading cause of emergency room visits and hospitalization in SCD, contributing heavily to the morbidity and mortality of this disease. Recurrent VOC creates a pro-inflammatory state that induces phenotypic alterations in the innate immune system. This inflammation-induced immune profile remodeling results in chronically active monocytes and neutrophils that antagonizethe vascular endothelium and further predispose SCD patients to VOC. While the mechanism of neutrophil involvement in VOC has been elucidated, less is known about the pathologic role of monocytes. Monocytes are of particular interest to VOC pathophysiology because they are especially malleable to inflammatory signaling. Indeed, inflammatory disease states such as COPD, obesity and atherosclerosis are known to influence monocyte development and alter monocyte subpopulations. Recently, nonclassical monocytes (CD14 dimCD16 +) with high heme oxygenase-1 expression have been shown to have a critical role in preventing VOC, generating renewed interest in monocytes as a therapeutic focus in SCD. In this study, we describe the monocyte subsets found in SCD patients during VOC by performing immunophenotypic flow cytometric, enzymatic, and morphologic analysis on peripheral blood obtained during hospitalization for VOC. Herein, we add to the growing body of evidence suggesting aberrant monocyte populations underpin VOC pathophysiology. We found that SCD monocytes during VOC possess an immature phenotype as demonstrated by 1) decreased CD4 positivity (p &amp;lt; .01), 2) low α-naphthyl butyrate esterase (ANBE) expression, and 3) naïve morphologic features (high nuclear/cytoplasmic ratio, indented or less lobular nuclei, and decreased cytoplasmic vacuolization). We additionally found an increase in CD14 +CD16 -CD4 - monocytes (p &amp;lt; .01), a subset associated with the impaired immune response of post-trauma patients. Interestingly, we also found a large proportion of CD14 +CD4 -HLA-DR - monocytes which, under normal circumstances, are exclusively found in neonates (p &amp;lt; .01). Finally, we report an increase in nonclassical monocytes (CD14 dimCD16 +), providing further evidence for the connection between this subset and the molecular mechanisms driving VOC. Taken together, these findings suggest that SCD patients in VOC deploy relatively immature monocytes that possess a lower capacity for antigen presentation, likely contributing to the immune dysfunction observed in SCD.

A Retrospective Study Evaluating the Association of Qualitative and Quantitative Aspects of Vaso-Occlusive Crises (VOC) with Length of Hospital Stay in Children with Sickle Cell Disease Hospitalized for a VOC

Background: Children with sickle cell disease (SCD) are at risk for developing a vaso-occlusive pain crisis (VOC) due to sickled red blood cells causing turbulent blood flow and obstruction within the vasculature, leading to local tissue infarcts and subsequently pain. A VOC can often be managed with analgesics and supportive care either at home or in the emergency room. However, sometimes the pain is not adequately controlled in those settings, so children require hospitalization for further management of their VOC. There exists a high degree of variability in length of hospital stay (LOS) among children who are admitted for VOCs. The purpose of this study is to determine which factors are associated with decreased or increased length of hospital stay (LOS). By identifying these factors, it will allow for a more targeted approach to VOC management with the goal of improving overall pain and reducing LOS. Methods: This 3 year (2019 - 2022) retrospective cohort study includes 88 patients (5 years to 27 years of age) with sickle cell disease (HbSS, HbSB0, HbSB+, HbSC) who were hospitalized for VOC management. Several variables were analyzed to determine if they correlated with LOS. These variables included SCD genotype, age, sex, pain scores, average hemoglobin and reticulocyte levels during hospitalization, pain medication dosage and frequency, blood transfusions and complications during hospitalization. The statistical analysis of these variables was conducted across all VOCs of all patients. Results: There were 289 hospital admissions for VOCs with a mean of 3.55 hospital admissions per patient among the 88 patients with SCD during study duration. LOS for these VOC hospital admissions ranged from 1 day to 10 days with an overall mean of 3.81 days per hospital admission. LOS was independently highest in females (mean LOS = 4.123 days, F (1, 287) = 6.415, p = .012) and patients who developed acute chest syndrome (mean LOS = 6.333 days, F (9, 277) = 4.771, p &amp;lt; .001). LOS positively correlated with pain scores (r 2 = .272, p = .002), number of blood transfusions (r 2 = .229, p &amp;lt; .001) and total units of blood transfused during admission (r 2 = .230, p &amp;lt; .001) and negatively correlated with morphine frequency (r 2 = -.125, p = .042) and average hemoglobin during admission (r 2 = -.142, p = .016). SCD genotype, age, reticulocyte levels and medication dosage were not found to have a statistically significant association with LOS. Conclusion: Our study showed that factors such as higher average pain scores, number of blood transfusions, total units of blood transfused, females and acute chest syndrome were associated with longer LOS. Other factors such as more frequent morphine administration and higher average hemoglobin levels were associated with shorter LOS. While this study provides initial insight into some of the factors that are associated with LOS in children with SCD admitted for a VOC, further studies are warranted to determine if these factors can be used to risk stratify patients in terms of severity of VOC and thereby better optimize and individualize their pain management.

Acute chest syndrome in children with sickle cell disease: Data from a national AIEOP cohort identify priority areas of intervention in a hub-and-spoke system.

Acute chest syndrome (ACS) is a frequent cause of hospitalization in sickle cell disease (SCD). Despite advances in acute care, many settings still lack knowledge about ACS best practices. After the AIEOP Guidelines were published in 2012, suggesting standardized management in Italy, a retrospective study was performed to assess the diagnostic and therapeutic pathways of ACS in children. From 2013 to 2018, 208 ACS episodes were presented by 122/583 kids in 11 centres. 73 were male, mean age 10.9 years, 85% African, 92% HbSS or Sβ°. In our hub-and-spoke system, a good adherence to Guidelines was documented, but discrepancies between reference centres and general hospitals were noted. Improvement is needed for timely transfer to reference centres, use of incentive spirometry, oxygen therapy and pain management.

Pharmacogenetics of pain management in Zimbabwean patients with sickle cell disease.

Background: Pain is a common cause of hospitalization in sickle cell disease (SCD) patients. Failure to effectively control pain remains a challenge in patient care. Materials & methods: The authors conducted a cross-sectional study to determine the effect of CYP2D6 and UGT2B7 polymorphisms on pain management in 106 Zimbabwean SCD patients. Participant information was collected on a questionnaire. Genotyping was conducted using the GenoPharm® pharmacogenomics open array panel containing CYP2D6 and UGT genetic variants implicated in opioid response. Results: The reduced function alleles CYP2D6*17 and *29 had high frequencies of 15.9% and 12.9%, respectively. UGT2B7 rs73823859 showed a statistically significant correlation with pain levels (p=0.0454). Conclusion: This study demonstrated the role of UGT2B7 polymorphism in SCD patient pain management.

National Burden of Encapsulated Bacterial Infections in Sickle Cell Disease

Introduction: Patients with sickle cell disease (SCD) are at much higher risk for infections from encapsulated organisms, particularly Streptococcus pneumoniae (pneumococcus), Neisseria meningitidis (meningococcus), and Hemophilus influenzae. Vaccinations and prophylactic penicillin (for pneumococcus) have declined but not eliminated their incidence. We aim to study the SCD admissions with infections from above mentioned encapsulated organisms. Methods: We performed a retrospective analysis using the National Inpatient Sample (NIS) database from 2016 to 2019 to identify SCD hospitalizations with either S. pneumoniae, N. meningitidis, or H. influenzae infections using appropriate International Classification of Diseases, Tenth Revision, Clinical Modification (ICD 10-CM) codes. SCD included HbSS (D57.0x), HbSC (D57.2x) and others (D57.1, D57.4x and D57.8x). Infections from S. pneumoniae and H. influenzae included sepsis (A40.3, A41.3), pneumonia (J13, J14), meningitis (G00.1, G00.0), and others (M00.1x, B95.3, A49.2, B96.3, and J20.1). N. meningitidis infections were identified by ICD-10-CM code A39.x. Admissions were divided into pediatric (<18 years) and adult (≥18 years) age groups and descriptive analysis was performed to compare demographics, hospital characteristics, and comorbid conditions/complications. We also calculated the annual hospitalization rate for encapsulated organisms per 10,000 SCD admissions. The methodology provided by the HCUP (Healthcare Cost and Utilization Project) was utilized, and weights were applied to produce national estimates. Results: Out of 533,175 SCD admissions from 2016-2019, 830 (0.16%) had an infection from S. pneumoniae or H. influenzae. There were no admissions with N. meningitidis during our study period. The annual hospitalization rate for encapsulated bacterial infection per 10,000 SCD admissions ranged from 15.6/year to 40.6/year for the pediatric population and 11/year to 15.2/year among adults (Figure 1). Overall rate was 30 per 10,000 SCD admissions in pediatric population and 12 per 10,000 in adults (p <0.001). Among S. pneumoniae infections, hospitalizations were highest for children ≤5 years at 64 per 10,000 SCD admissions, followed by 14 per 10,000 in children aged >5 & <18 years and lowest in adults (≥18 years) at 12 per 10,000 (p <0.001). Among 695 SCD admissions with S. pneumoniae, 325 (46.8%) were HbSS, and 65 (9.4%) had HbSC disease. Furthermore, 260 (37.4%) were <18 years and 435 (62.6%) were ≥18 years. The median age for children was 5 (2-10) years and 34 (27-47) for adults (p <0.001). Most admissions were males (56.1%), without significant comorbidities (Charlson comorbidity index 0 in 59.7%) and belonged to lowest income communities (47.8%). More than a quarter of adults (26.4%) had tobacco use disorder, and 8.1% had HIV/AIDS. Incidence of acute respiratory failure and acute kidney failure was higher in adults than in children (25.3% vs. 1.9%; p <0.001 and 29.9% vs. 3.9%; p <0.001 respectively). The median length of stay was 6 (4-11) days for adults and 4 (3-7) days for <18-year-olds (p <0.001). There were 26.4% adults compared to 9.6% children with a length of stay >10 days (p 0.02). Median hospital charges and costs were also higher for adults compared to children. Whereas there were no pediatric discharges requiring home health care, 17.4% of adults had home health care arranged (p 0.01). All-cause-in-hospital mortality was 1.4%. Out of 145 H. influenzae admissions, 40 (27.6%) were <18 years, and 105 (72.4%) were ≥18 years. Like S. pneumoniae, the majority of SCD admissions with H. influenzae admissions were males (65.5%), belonged to the lowest income communities (51.7%), admitted to large (48.3%), teaching hospitals (89.7%), and had no significant comorbidities (Charlson comorbidity index 0 in 55.2%). Around 24.1% stayed in the hospital for >10 days, and 20.7% required invasive mechanical ventilation. The median length of stay was 7 (5-10) days, and all-cause-in-hospital mortality was 3.5% (Table 1). Conclusion: Despite vaccinations and prophylactic penicillin, the burden of encapsulated bacterial infections in pediatric SCD hospitalizations remains disproportionately higher than in adults. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Economic Disparities in the Outcomes of Sickle Cell Disease-Related Admissions over a Decade

Background: According to the CDC, Sickle Cell Disease (SCD) occurs in 1 out of every 365 African American births and 1 out of every 16,300 Hispanic American births. It is an inherited disorder caused secondary to a mutation in the hemoglobin beta subunit and has complex phenotypes. The disease is characterized by recurrent hemolysis, vaso-occlusion leading to pain episodes, and chronic organ damage due to ischemia. A comprehensive care management team is not well established for this disease. There is sparse information regarding the trends in outcomes of SCD admissions in the past decade where rapid advances have been made in treatment including the evolving hematopoietic stem cell transplant. In this study, we sought to analyze the trends and outcomes of SCD admissions in the United States from 2011- 2019 and the influence of socioeconomic status. Methods: The National Inpatient Sample (NIS) database was queried to identify adult patients admitted with a principal diagnosis of Sickle Cell Disease using the International Classification of Disease (ICD-9) and ICD-10 codes. Trends for primary in-hospital outcomes including mortality, length of stay (LOS), and total hospitalization charges (THC) were assessed. The impact of economic status on these outcomes was also studied. Statistics were performed using STATA and multivariate linear and logistic regression analysis was used to adjust for confounders. Results: The rate of hospitalizations for SCD remained stable from 2010 to 2019. Women constituted 53.99% and the majority of patients were young adults (86.62%) and Black (90.36%) as expected. Many patients belonged to the low-income quartile group and were admitted to hospitals with a large bed size and urban teaching hospitals. The overall mortality rate among adult SCD hospitalizations from 2010 to 2019 decreased by an odds ratio of 0.96 (p=0.008) after adjusting for age, sex, and race and is reassuring. However, on subgroup analysis, this trend was replicated for those belonging to a higher income quartile group whereas there was no statistically significant decrease in the mortality rate trend for those belonging to the lower income quartile group. After adjusting for confounders there was an increase in the total charges in all subgroups (p<0.001). The mean LOS decreased for the total population and most subgroups (p<0.001). Conclusion: Despite the advances in the understanding of SCD and its treatment, its benefits have not reached all the people affected. Access to healthcare and the treatment costs associated could be one among the possible reasons why the lower income quartile group have not yet benefited. Meaningful progress in healthcare is not achievable unless these economic disparities are addressed. It is imperative to develop economic policies to address these inequities. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Intravenous Fluid Administration and the Risk of Adverse Outcomes in Sickle Cell Disease Patients Hospitalized for Vaso-Occlusive Crisis.

Vaso-occlusive crisis (VOC) is the leading cause of hospitalization in sickle cell disease (SCD). Intravenous fluid (IVF) administration is the usual practice during VOC episodes to slow the sickling process. In the absence of an evidence-based, clear-cut consensus on the optimal choice, route, and rate of fluid administration, there has been a wide variability in the practice of IVF administration in the treatment of VOC. However, there are growing concerns about the safety of this practice. This systematic review summarized the current evidence on the risk of negative outcomes in SCD patients treated for VOC with IVFs. A database search of Medline/PubMed, EMBASE, Scopus, Web of Science, CINAHL, Wiley Cochrane Library, Clinicaltrials.gov, and conference proceedings of the European Hematology Association (EHA) and American Society of Hematology (ASH) were performed. The results were presented using narrative analysis of quantitative data. Of the 2,821 identified records, a total of three eligible retrospective cohort studies with a total demographic population of 549 SCD patients were included in this review. Normal saline, a frequently used IVF for VOC may be associated with adverse outcomes such as poor pain control and volume overload. Volume overload, new oxygen requirement, acute chest syndrome, and acute kidney injury are potential adverse outcomes of inappropriate IVF use in VOC. There is limited evidence supporting the current practice of IVF use in VOC. Randomized controlled trials are required to fully clarify the place and safety of IVF in the management of VOC.

MSR51 Sickle Cell Disease Hospitalizations and Social Determinants of Care: An Investigation into the Disproportionate Burden of Disease

Sickle cell disease (SCD) interferes with oxygen delivery to tissues caused by a mutation in the b-globin gene alleles with at least one sickle cell mutation. Sickle cell disease affects approximately 100,000 Americans and occurs in about 1 in 365 African American births. With the increasing number of SCD patients, the number of hospitalizations in the US are also increasing with each year.

Hospital Use and Mortality in Transition-Aged Patients With Sickle Cell Disease.

Childhood mortality in sickle cell disease (SCD) has decreased, but the transition period is associated with poor outcomes and higher mortality rates. We analyzed recent US hospitalizations and mortality trends in the transition-aged population and evaluated for differences between patients with and without SCD. Nationwide Inpatient Sample database was used to analyze hospitalizations among individuals aged 16 to 24 years from 2003 to 2017. Diagnoses were coded by using International Classification of Diseases, Ninth Revision, Clinical Modification and International Classification of Diseases, 10th Revision, Clinical Modification. We performed bivariate analyses to assess associations between sociodemographic characteristics and SCD hospitalizations, joinpoint regression analysis to describe mortality rate trends in SCD hospitalizations, and adjusted survey logistic regression to assess associations between patient characteristics and in-hospital mortality among transition-aged SCD and non-SCD-related hospitalizations. There were 37 344 532 hospital encounters of patients aged 16 to 24 years during 2003-2017; both SCD and non-SCD hospitalizations increased with age. Female patients accounted for 78% of non-SCD and 54.9% of SCD hospitalizations. Although there was a +3.2% average annual percent change in SCD hospitalizations, total SCD in-hospital mortality rates did not have a statistically significant increase in average annual percent change over the study period. Patients with SCD aged 19 to 21 and 22 to 24 were more likely to suffer in-hospital mortality than those aged 16 to 18 (odds ratio = 2.09 and 2.71, respectively); the increased odds in mortality by age were not seen in our non-SCD population. Transition-aged hospitalizations increase with age, but SCD hospitalizations have disparate age-related mortality rates. Hospital-based comprehensive care models are vital to address the persistent burden of early adulthood mortality in SCD.

Sickle Cell Disease and Cystic Fibrosis Are Associated with Increased Non-Administration of VTE Prophylaxis in Adult Patients

Background:Sickle cell disease (SCD) and cystic fibrosis (CF) are associated with an increased risk of venous thromboembolism (VTE). VTE prophylaxis non-administration is a risk factor for VTE. Since many patients with SCD or CF experience frequent hospitalizations, we hypothesized that missed doses of VTE prophylaxis may be common in these populations. The purpose of this study was to characterize VTE prophylaxis non-administration among patients with SCD or CF compared to medically ill patients without SCD or CF.Methods:We conducted a single-center retrospective cohort study of patient admissions from July 1 st, 2016 to December 31 st, 2019 who were prescribed at least 2 doses of pharmacologic VTE prophylaxis. Three adult inpatient cohorts were defined: patients with SCD, patients with CF, and medically ill patients without SCD or CF. We excluded patients on pediatric and surgical units, and excluded doses received while in intensive care units or prescribed as once doses. The primary outcome was the proportion of missed doses of VTE prophylaxis by cohort. Secondary outcomes included the proportion of patients who missed at least one dose by cohort, the proportion of missed doses by medication and frequency, and the proportion of missed doses in patients with more than one admission during the study period. Multivariable logistic regression was used to identify characteristics associated with non-administration.Results:The 13,316 subjects included 246 with SCD, 89 with CF, and 12,981 medically ill. Subjects with SCD and CF were younger than medically ill patients [median (IQR) age 31 (16) and 30 (11) years, respectively versus 58 (24) years, p<0.01]. A higher number of SCD and CF patients had multiple hospital admissions than medically ill patients (52.5% and 73.0%, respectively vs. 21.8%, p<0.01). VTE prophylaxis dose non-administration overall and by prescribed regimen are shown in Table 1. During the study period, 95,697 doses were prescribed; 32.7% were not administered in the SCD cohort, 61.8% in the CF cohort, and 19.7% in the medically ill cohort (p<0.01). In the multivariable logistic regression, SCD diagnosis [odds ratio (OR) 1.73, 95% confidence interval (CI) 1.29-2.32], CF diagnosis (OR 4.64, 95% CI 2.98-7.21), age < 30 years (OR 2.29, 95% CI 1.98-2.63), and multiple admissions (OR 1.13, 95% CI 1.01-1.26) were associated with non-administration of one or more doses. Black race (OR 0.86, 95% CI 0.78-0.95) and receiving a once daily prophylaxis regimen (OR 0.76, 95% CI 0.69-0.84) were associated with reduced odds of missing a dose. Patients with SCD, patients with CF, and younger patients missed greater proportions of their individually prescribed doses (Table 2).Conclusion:Patients with SCD or CF missed a larger proportion of pharmacologic VTE prophylaxis compared to medically ill patients. Factors independently associated with increased odds of non-administration include age < 30 years and multiple hospital admissions. Black race and once daily administration were associated with decreased odds of non-administration. These findings suggest that targeted education or once-daily dosing may improve VTE prevention in high-risk lifelong disorders such as SCD and CF. [Display omitted] DisclosuresNaik: Rigel: Research Funding. Lanzkron: GBT: Research Funding; Shire: Research Funding; Novo Nordisk: Consultancy; Teva: Current holder of individual stocks in a privately-held company; Pfizer: Current holder of individual stocks in a privately-held company; Imara: Research Funding; CSL Behring: Research Funding; Novartis: Research Funding; Bluebird Bio: Consultancy. Dane: Sanofi Genzyme: Honoraria; Janssen: Honoraria; Alexion: Honoraria.

Profile of Medicaid enrollees with sickle cell disease: A high need, high cost population.

Sickle cell disease is a progressively debilitating genetic condition that affects red blood cells and can result in a variety of serious medical complications, reduced life expectancy, and diminished quality of life. Medicaid nationwide covered 66 percent of sickle cell disease hospitalizations in 2004 and 58 percent of emergency department visits for the disease between 1999 and 2007. Using Medicaid data from four states with large populations that account for more than one-third of Medicaid program enrollment, we examined the characteristics of those with sickle cell disease. We found instances of mortality rates more than nine times the age-adjusted population average (in Texas, a mortality rate for Medicaid enrollees with SCD of 1.11 percent compared to 0.12 percent overall); rates of disability-related eligibility–which is associated with long-term Medicaid enrollment–of up to 69 percent; and half or more of affected enrollees having (all-cause) hospital stays, emergency department visits, and opioid prescription fills. With gene therapies on the horizon that will spur discussions of treatment coverage, costs, and outcomes for people with sickle cell disease, it is important for relevant stakeholders to understand the affected populations.

The Emotional Impact of Sickle Cell Disease Symptoms: A Case Review

Background: Sickle Cell Disease (SCD) is a chronic and debilitating disease that can affect almost any organ system in the body; hence, it is associated with a broad range of complications and comorbidities that may have a profound impact on a patients’ quality of life through medical and mental health consequences. Psychosocial and affective comorbidities are prevalent in SCD and could considerably influence disease outcomes; however, treatment options for SCD psychosocial sequelae are suboptimal, and evidence on their effectiveness is limited. Case Report: We report a 21-year-old African American female with a history of SCD and recurrent hospital admissions due to acute SCD complications, who was referred to the consultation-liaison psychiatry service for evaluation of psychiatric comorbidities and management recommendations. Conclusion: This case demonstrates the complexity of the relationship between somatic complications and neuropsychiatric disorders in SCD and highlights the importance of early identification and management of mental health conditions in this patient population. We emphasize treatment challenges and the need for large scale research on pharmacotherapies and nonpharmacological interventions in treating psychosocial comorbidities associated with SCD.

Mortality and Hospitalizations among Sickle Cell Disease Patients with End-Stage Kidney Disease Initiating Dialysis

Background: Sickle cell disease (SCD) is the most common inherited hematological disorder and a well-described risk factor for end-stage kidney disease (ESKD). Mortality and hospitalizations among patients with SCD who develop ESKD remain understudied. Furthermore, prior studies focused only on SCD patients where ESKD was caused by SCD. We aimed to describe mortality and hospitalization risk in all SCD patients initiating dialysis and explore risk factors for mortality and hospitalization. Methods: We performed a national observational cohort study of African American ESKD patients initiating dialysis (2000–2014) in facilities affiliated with a large dialysis provider. SCD was identified by diagnosis codes and matched to a reference population (non-SCD) by age, sex, dialysis initiation year, and geographic region of care. Sensitivity analyses were conducted by restricting to patients where SCD was recorded as the cause of ESKD. Results: We identified 504 SCD patients (mean age: 47 ± 14 years; 48% females) and 1,425 reference patients (mean age: 46 ± 14 years; 49% females). The median follow-up was 2.4 (IQR 1.0–4.5) years. Compared to the reference, SCD was associated with higher mortality risk (hazard ratio 1.66; 95% confidence interval [CI]: 1.36–2.03) and higher hospitalization rates (incidence rate ratio 2.12; 95% CI: 1.88–2.38) in multivariable analyses. Exploratory multivariable mortality risk models showed the largest mortality risk attenuation with the addition of time-varying hemoglobin and high-dose erythropoietin, but the association of SCD with mortality remained significant. Sensitivity analyses (restricted to ESKD caused by SCD) also showed significant associations between SCD and mortality and hospitalizations, but with larger effect estimates. High-dose erythropoietin was associated with the highest risk for mortality and hospitalization in SCD. Conclusions: Among ESKD patients, SCD is associated with a higher risk for mortality and hospitalization, particularly in patients where SCD is identified as the cause of ESKD.

Impact of Pulmonary Hypertension on in-hospital outcomes amongst Sickle Cell Disease hospitalizations

Introduction: Pulmonary hypertension (PH) is a common and severe complication of Sickle Cell Disease (SCD), and an independent risk factor for mortality. While there is a clear association between SCD and PH, the predictors of PH in SCD and the impact of PH on in-hospital outcomes of SCD hospitalizations remains unknown. In our study, we sought to assess the in-hospital prevalence, predictors, and the impact of PH in SCD hospitalizations. Methods: We used the 2016 and 2017 National Inpatient Sample (NIS) to identify all adult hospitalizations with a primary discharge diagnosis of SCD. The sample was then stratified based on the presence or absence of PH. We used the Pearson chi-square test and weighted Student's t-test to analyze categorical and continuous variables, respectively. Multivariate logistic regression analysis was performed to calculate the adjusted odds ratio for various clinical outcomes. SAS was used for the analysis, and the p-value was defined as &amp;lt;0.05. Results: We identified n=191,080 weighted hospitalizations for SCD, of which, 5.54% (n=10590) had concomitant PH. Female gender and comorbidities including hypertension, obesity, illicit drug use, hepatic cirrhosis, renal failure, prior venous-thromboembolism, valvular, and congenital heart disease were identified as significant predictors of PH in SCD. PH was associated with increased in-hospital mortality (1.04% vs 0.22%, AOR=2.14, 95% CI 1.15-3.98, p=0.0158). PH in SCD hospitalizations also increased the odds of - acute kidney injury (AKI), need for dialysis, acute respiratory failure (ARF), and need for mechanical ventilation for &amp;gt; 96 hours. The adjusted odds ratio for venous thromboembolism, shock-state, and the need for cardiac catheterization (both right and bilateral) were also higher in patients with PH. Overall hospitalization cost and length of stay increased (7.06±0.16 vs 1.82±0.02 days) in patients with SCD and PH (see table 1). Conclusion: In sickle cell disease hospitalizations, PH is independently associated with increased in-hospital morbidity and mortality, with an increased need for in-hospital catheterizations thereby, prolonging the length of stay and overall health care costs. Identifying and treating PH in the SCD population would improve in-hospital outcomes. Disclosures No relevant conflicts of interest to declare.