Patients with Sickle Cell Disease and Venous Thromboembolism Experience Increased Frequency of Vasoocclusive Events

Introduction Patients with sickle cell disease (SCD) are at increased risk for venous thromboembolism (VTE). By age 40, 11-12% of SCD patients have experienced a VTE. VTE confers nearly a three-fold increase in mortality risk for individuals with SCD. We hypothesized that VTE increases subsequent SCD severity which may increase acute care utilization. We investigated the association between VTE and rates of vaso-occlusive events (VOE) and acute care utilization for individuals with SCD. Methods We performed a retrospective longitudinal chart review of 239 adults with SCD who received care at our institution between 2003 and 2018. VTE was defined as deep venous thrombosis (DVT) diagnosed by Duplex ultrasound or pulmonary embolism (PE) diagnosed by either ventilation-perfusion scanning or computed tomography angiography. Medical histories, laboratories and medication use for all subjects were obtained. For VTE patients, clinical data for 1- and 5- years post-VTE were obtained and compared to 1 year prior to the VTE. For non-VTE patients, data were obtained at baseline and compared to five years later. We evaluated all acute care visits for the presence of a SCD-related problem, specifically assessing if a VOE or acute chest syndrome (ACS) occurred. We calculated rates of VOE, ACS, Emergency Department (ED) visits and hospitalizations prior to and subsequent to a VTE and compared these to occurrence rates among those without VTE. Data were analyzed using Stata 14.2. Results In our cohort of 239 individuals with SCD, 153 (64%) were HbSS/HbSβ0 and 127(53%) were female. Fifty-six individuals (23%) had a history of VTE; 20 had a DVT (36%), 33 had a PE (59%), and 3 had both (5%). Patients with VTE had a higher frequency of prior history of ACS (p<0.001), stroke (p=0.013), splenectomy (p=0.033), and avascular necrosis (p<0.001) than those without a VTE. Prior to their VTE, these patients had higher white blood cell (11.8 x103 [9-15 x 103] vs 9.7 x103 [7-12 x 103], p=0.047) and platelet counts (378 x 103 [272-485 x 103] vs 322 x 103 [244-400 x 103], p=0.007) than those without a VTE. During five years of follow-up after a VTE, these patients had 6.32 (SD 14.97) ED visits per year compared to 2.84 (SD 5.93, p<0.03) ED visits per year in those without a VTE. Ninety two percent of these ED visits were SCD-related; 73% were for VOE and 4% for ACS. Additionally, SCD patients with a VTE had an increase in all-cause hospital admissions (2.84 [SD 3.26] vs 1.43 [SD 2.86], p=0.003) and SCD-related hospital admissions (2.61 [SD 3.13] vs 1.23 [SD 2.74], p=0.001) per year compared to those without VTE. Conclusion VTE is a frequent complication in patients with SCD. Our study suggests that patients who experience a VTE have greater SCD severity as evidenced by increased VOE, ED and hospital utilization. These data suggest that VTE is not merely an isolated event in SCD patients and that it may either serve as an indicator of disease severity or contribute to overall disease pathophysiology. Disclosures Sloan: Abbvie: Other: Endpoint Review Committee; Stemline: Consultancy; Merck: Other: endpoint review commitee.

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Impact of the COVID-19 Pandemic on Acute Care Visits for Patients with Sickle Cell Disease

Blood ◽

10.1182/blood-2021-150869 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 3097-3097

Author(s):

Dennis Orkoulas-Razis ◽

Nicholas Bishop ◽

Ellen Dupont ◽

Maria R. Baer ◽

Richard Gentry Wilkerson ◽

...

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Care Utilization ◽

Cell Disease ◽

Presenting Symptoms ◽

Significant Difference ◽

Acute Care Utilization ◽

Ed Visits ◽

Disease Specific

Abstract Introduction: The COVID-19 pandemic significantly impacted emergency department (ED) and overall hospital utilization, with a substantial decline in non-COVID-19-related medical presentations. In the weeks following the declaration of a national health emergency, ED visits declined by 42%. Patients with sickle cell disease (SCD) are at risk for needing ED-based care and hospitalization due to disease-specific complications. We examined the impact of the COVID-19 pandemic on acute care utilization by patients with SCD at our institution. Methods: We performed a retrospective cohort study at our institution comparing the period of the first "stay at home" order in Baltimore, MD (3/30/2020-6/8/2020) to the same date range in 2019. We included all adult patients with SCD who either presented to the ED or were directly admitted to the hospital. All SCD genotypes were included (HbSS, HbSC, HbSβ +/0 thalassemia). Data collected included presenting symptoms, disposition for ED visits, admission length of stay (LOS), re-admission within 7 days, as well as frequency of sickle cell-specific complications during hospitalization. We collected data regarding the acuity of patients' initial presentation using the emergency severity index (ESI), a five-tiered grading tool utilized by triage nurses to indicate the acuity and resource-intensiveness of a patient's presenting symptoms (1= highest urgency, 5= least urgency). We performed statistical analyses using Pearson's chi square test, Fisher's exact test and the Mann-Whitney U test. Results: During the initial stay at home order in 2020, 77 patients presented to acute care services at our institution, compared to 163 patients during the same dates in 2019, a decrease of > 50%. Statistically significant demographic differences between 2020 and 2019 included gender (53% vs 34% male gender, p = 0.004) and hemoglobinopathy type (2020: SS (66%), SC (27%), Sβ-thal (6.5%) vs 2019: SS (48%), SC (42%), Sβ-thal (10%), p = 0.03), whereas there was no difference in severity on presentation measured by ESI (median score of 3: 88% vs 90%, p = 0.13) or age (30 vs 30 years old, p = 0.925). More patients in 2020 presented with dyspnea (22% vs 11%, p = 0.02), and/or nausea or vomiting (22% vs 11%, p = 0.02), but more patients in 2019 presented with cough (7% vs 17%, p = 0.025). None of the patients tested positive for SARS-CoV-2. There was no statistically significant difference between the study periods in hospitalization rate (44% vs 37%, p = 0.32), LOS (60 vs 64 hrs, p = 0.73), admission to the ICU (3% vs 2.5%, p = 1.0) or step-down unit (0% vs 1%, p = 1.0), or death (0% vs 1%, p = 1.0). There was a difference in ED re-presentation within 7 days of the index visit (14% vs 47%, p < 0.001), but no difference in rate of readmission within 7 days (9% vs 15%, p = 0.225). Discussion: Although fewer patients with SCD presented for acute care in 2020, there was no significant difference in objective metrics, including admission rates, LOS, readmissions, and disease-specific complications. The decrease in ED return visits in 2020 may reflect patients' concerns regarding exposure to SARS-CoV-2 while in the ED. Our data demonstrate that although fewer patients with SCD presented for acute care utilization, they did not appear to be sicker. The data support more frequent management of uncomplicated pain crises outside of the ED, through optimization of outpatient services including infusion centers and telehealth. The advent of new care delivery models as a result of the Covid-19 pandemic may have a positive impact on frequency of ED utilization for patients with SCD. Disclosures No relevant conflicts of interest to declare.

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Characteristics of Acute Care Utilization of a Delaware Adult Sickle Cell Disease Patient Population

Population Health Management ◽

10.1089/pop.2012.0119 ◽

2014 ◽

Vol 17 (1) ◽

pp. 60-65 ◽

Cited By ~ 4

Author(s):

Nina Anderson ◽

Jennifer Bellot ◽

Oluseyi Senu-Oke ◽

Samir K. Ballas

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Patient Population ◽

Disease Patient ◽

Sickle Cell Disease Patient ◽

Care Utilization ◽

Cell Disease ◽

Acute Care Utilization

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Relationships between outpatient pain scores and acute care utilization in sickle cell disease

Journal of Pain ◽

10.1016/j.jpain.2012.01.081 ◽

2012 ◽

Vol 13 (4) ◽

pp. S18

Author(s):

D. Wilkie ◽

M. Ezenwa ◽

M. Suarez ◽

Y. Kim ◽

H. Wittert ◽

...

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Care Utilization ◽

Cell Disease ◽

Pain Scores ◽

Acute Care Utilization

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Acute care utilization in pediatric sickle cell disease and sickle cell trait in the USA: prevalence, temporal trends, and cost

European Journal of Pediatrics ◽

10.1007/s00431-020-03656-x ◽

2020 ◽

Vol 179 (11) ◽

pp. 1701-1710

Author(s):

Elisha E. Peterson ◽

Jason L. Salemi ◽

Deepa Dongarwar ◽

Hamisu M. Salihu

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Sickle Cell Trait ◽

Temporal Trends ◽

Care Utilization ◽

Cell Disease ◽

The Usa ◽

Acute Care Utilization ◽

Pediatric Sickle Cell Disease

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Acute Care Utilization and Rehospitalizations for Sickle Cell Disease

JAMA ◽

10.1001/jama.2010.378 ◽

2010 ◽

Vol 303 (13) ◽

pp. 1288 ◽

Cited By ~ 317

Author(s):

David C. Brousseau

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Care Utilization ◽

Cell Disease ◽

Acute Care Utilization

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Health Care Utilization By Age and Hydroxyurea Response in Pediatric and Adolescent Patients with Sickle Cell Disease

Blood ◽

10.1182/blood.v128.22.2500.2500 ◽

2016 ◽

Vol 128 (22) ◽

pp. 2500-2500

Author(s):

Emily Stewart ◽

Amy Sobota

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Conflicts Of Interest ◽

Care Utilization ◽

Patient Navigator ◽

Inverse Association ◽

Cell Disease ◽

Acute Care Utilization ◽

Clinic Visits

Abstract Background: Children and adolescents with sickle cell disease (SCD) have high rates of healthcare utilization which peak around the age of transition from pediatric to adult care. Adherence to evidence based care, including regular clinic visits, annual transcranial doppler (TCD) studies, and hydroxyurea (HU) use, is often suboptimal in this population. Over the past 4 years we have used a registry and patient navigator to improve access to routine care for our patients with SCD. We hypothesized that increased access to clinic and higher rates of HU use would positively impact acute care utilization. The purpose of this study was to 1) assess adherence to clinic visits and TCD rates, 2) describe patterns of acute care utilization, and 3) analyze the association between response to HU and acute care utilization in our patient cohort. Methods: We analyzed data on 208 unique patients with SCD between the ages of birth to 22 years of age who received care through the pediatric hematology program at BMC from April 2013 through March 2016. Demographics (age and genotype) and data on clinic visits, TCD screening, ED visits, inpatient stays, and HU use was obtained from the data ware house based on patients in our SCD registry. We calculated emergency department reliance (EDR), a ratio of clinic adherence relative to acute care utilization, for each patient. TCD rates and HU use were analyzed for patients with HbSS genotype only. Patient were stratified into three HU categories; patients not prescribed HU, those prescribed HU with a HbF response of less than 15%, and those prescribed HU with a HbF response of at least 15%. A Kruskal-Wallis test was used to analyze differences in rates of acute care utilization between the HU groups, Wilcoxon rank-sum test was used to assess whether EDR differed by HbF category, and Spearman's rank correlation was used to test for an association between HbF and EDR. Results: On average over the three year period 78% of patients had two clinic visits per year, 85% of eligible patients were prescribed HU and 96% had annual TCD screening. Acute care utilization was higher among older patients, consistent with the existing literature. Patients in the oldest age bracket had the highest mean EDR at 0.33 (Table 1). Among patients prescribed HU, increased HbF% was associated with lower rates of acute care utilization among all measures analyzed (Table 2). There was also a statistically significant, inverse association between HbF percentage and EDR of -0.2183 (p=0.0275). Conclusion: Through use of a SCD registry and patient navigator we have maintained adherence to biannual clinic visits, TCD screening, and HU uptake much higher than the rates reported in the literature. Our patients also had low rates of EDR at 0.23 which may reflect our clinic's accessibility. Increased levels of HbF, representing better adherence to HU, were associated with less acute care utilization, highlighting the importance of finding ways to increase adherence in our patient population. Disclosures No relevant conflicts of interest to declare.

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