Temperature and Humidity Effects on Acute Vaso-Occlusive Pain Episodes in Sickle Cell Disease

Abstract Background: Sickle cell patients suffer from painful vaso-occlusive episodes (VOE) that interrupt patients' lives and productivity and lead to emergency department visits and hospitalizations and, on occasion, death. The VOE in sickle cell disease (SCD) accounts for 90% of hospital admissions for patients with SCD and constitute a financial burden on both patients and hospitals. Efforts to prevent pain crises have failed to establish a causal relationship in about 40% of reported cases. The remaining 60% of known causes of VOE are related to dehydration, febrile illness, and infections. The Emory University Sickle Cell Center at Grady Memorial hospital has been providing specialized services for SCD patients for 30 years. The center includes a 24/7 acute care unit (ACU) that is staffed by SCD providers who specialize in the management of VOE. The patients are started on intravenous narcotics and fluids within 30 minutes of presenting to the ACC. After eight hours of management, the patient is then either discharged home if the VOE is controlled or admitted to the hospital for continued management. Annually, around 3000 ACU visits are recorded with a 17% hospital admission rate. When patients are questioned on the reason for their presentation, “weather change” is frequently reported as the trigger of the VOE. A review of the literature reveals scant data to support the hypothesis that weather-related changes trigger sickle cell pain crises. Methods: In a retrospective evaluation of patient visits over the last 4 years, we identified temperature and humidity measurements for that period of time and attempted to correlate them with the frequency of ACU visits. We used the “weatherspark” website that records weather changes for the city of Atlanta because > 90% of our patients reside in the city. Results: The four-year review of weather data and ACU visits did not show a direct correlation between graphs of temperature and humidity, and the number of ACU visits. The annual number of visits to the ACU was 2930, 2467, 3195 and 3370 for the years 2011, 2012, 2013 and 2014 respectively. The average admission rate was 16.6% overall, and the admission rate in the years 2011, 2012, 2013 and 2014 was 12.4%, 14.9%, 19.6% and 19.6% respectively. The attached figure has an example of the year 2013 charts for temperature, humidity, sickle cell acute care visits and hospital admission graphs. Conclusion: The retrospective analysis does not reflect a direct correlation between the temperature and humidity graphs and the number of ACU visits and hospital admissions. Given the volume of data analyzed, it is unlikely that there is any correlation between temperature and humidity variations and either acute care visits or admissions. Atlanta, GA- ACU visits, Hospital admissions, Temperature and Humidity Charts for 2013: DATA from weatherspark.com Figure 1. Figure 1. Figure 2. Figure 2. Figure 3. Figure 3. Disclosures No relevant conflicts of interest to declare.

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Characteristics of Sickle Cell Clinic Visits and Hospital Admissions in a Large Urban Academic Hospital

Blood ◽

10.1182/blood-2019-131133 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 5857-5857

Author(s):

Levani Odikadze ◽

Morgan L. McLemore ◽

Neli Stoyanova ◽

Fuad A El Rassi

Keyword(s):

Sickle Cell Disease ◽

Emergency Room ◽

Acute Care ◽

Sickle Cell ◽

Hospital Admissions ◽

Disease Patient ◽

Admission Rate ◽

Cell Disease ◽

The Mean ◽

Clinic Visits

Introduction: Although high rates of emergency department (ED) visits by sickle cell disease patient population is a significant health-care system burden, there are limited studies available that explore outpatient/ED visit patterns. The purpose of this study was to evaluate the pattern of medical care utilization including clinic visits and hospital admission in adults with sickle cell disease in a large urban academic center with a dedicated 24 hour/7 day a week sickle cell emergency room. Methods: Patients seen in the Acute Care at Grady Memorial Hospital Comprehensive Sickle Cell Center (GMHSCC) over a three years from January 2015 to December 2017 where analyzed. The Acute Care center at GRMHSCC is a unique 24 hour 7 day a week dedicated emergency room for sickle cell patients. Patient demographics, specialized sickle cell clinic visits and hospital admissions were analyzed descriptively. Independent samples t-test was used for comparison of hospitalization and acute care visits between males/females and phenotype groups. Results: The total of 1,277 patients were included in analysis representing 12,129 acute care visits. 683 (53.5%) females and 593 males (46.5%). Mean age was 32.9 years. Genotypes where similar to known expected distributions, 878 patients had Hb SS (68.8%), 301 had SC (23.6%), 23 had SB0TH (1.8%) and 74 had SB+TH (5.8%). The mean number of acute care visits over 3 years for SS phenotype was 9.71, SC phenotype 9.74, SB+Th phenotype 5.73 and SB0TH phenotype 10.48. Overall admission rate was 16%. There was a significant statistical difference between the mean number of admissions among SS and SC phenotype patients (p=0.009). Rate of hospitalization per clinic visit for SS phenotype was 18.19% , for SC phenotype 10.78% , SB+TH phenotype was 15.58% and SB0TH phenotype 9.55%; Conclusions and future objectives: We have demonstrated that a dedicated Sickle Cell emergency room is able to achieve a significantly lower admission rate than standard Emergency rooms. Preliminary analysis shows that the distribution of acute care visits is distributed across the genotypes at the known distribution. In addition there is no difference in mean number of sickle cell acute visits between patients with SS and SC phenotype. This is interesting as Hgb SC is considered a less severe form of the disease. There was a statistically significant higher admission rate for SS patients compared to SC patients. Future analyses will look at additional parameters associated with acute care visits and admission. Disclosures No relevant conflicts of interest to declare.

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Emergency Department Management of Children with Sickle Cell Disease.

Blood ◽

10.1182/blood.v104.11.1660.1660 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1660-1660

Author(s):

Melissa J. Frei-Jones ◽

Amy L. Baxter ◽

Charles T. Quinn ◽

George R. Buchanan

Keyword(s):

Emergency Department ◽

Steady State ◽

Sickle Cell Disease ◽

Hospital Admission ◽

Sickle Cell ◽

Admission Rate ◽

Categorical Variables ◽

Continuous Variables ◽

Cell Disease ◽

Ed Visits

Abstract Vaso-occlusive crises (VOC) are a common cause of emergency department (ED) visits for children with sickle cell disease (SCD). To better understand our patient population and compare with reports from other centers, we sought to describe the presentation, management, and disposition of children with VOC at our center’s ED. We also aimed to identify predictors of hospital admission. We retrospectively reviewed hospital records of all patients with SCD, age 8–19 years, who presented to our urban pediatric ED in 2003 with a chief complaint of pain. We identified all subjects diagnosed with VOC and not another cause of pain. We obtained the following data for each: SCD genotype; duration of VOC and treatment prior to ED presentation; the nature of analgesia and use of intravenous fluids (IVF) in the ED; hemoglobin (Hgb) concentration; and disposition (admitted, discharged, discharged with subsequent ED visit for same crisis). Categorical variables were evaluated by the χ2 and Fisher exact tests and continuous variables by the t-test. Odds ratios (OR) and 95% confidence intervals (CI) were calculated where appropriate. In 2003, there were 320 ED visits for patients with SCD and pain. Among these, there were 279 diagnoses of VOC in 105 individual patients: 45 had one visit, 25 two visits, and 16 ≥5 visits. Mean number of visits per patient was 2.7; 23 (22%) patients accounted for 145 (55%) visits. Homozygous sickle cell anemia (Hgb SS) was present in 73/105 patients, accounting for 222 (79%) ED visits. Overall admission rate was 179/279 (64%), with 167/179 admitted on their first visit and 12 on their return visit. Subjects with Hgb SS accounted for 147/179 (82%) admissions. Among those discharged who later returned to the ED during the same VOC, the admission rate was 86% (5 returned in 24 hours, 5 in 48 hours, 3 in 72 hours, and one 4 days later). Pre-ED home opioid use was reported in 75% of visits and was associated with increased likelihood of discharge (OR 1.63, CI 0.94–2.84, p=0.082). Duration of VOC before presentation did not significantly affect admission rate and averaged 53.2 hours for admitted patients and 49.7 hours for those discharged (p=0.689). Patients who received IVF in the ED (219/279; 79%) were less likely to be admitted (31% vs 56%, p<0.001). Hgb concentration was increased in 61%, decreased in 36% and unchanged in 3% of patients from steady-state values and was not associated with admission. After receiving 2 doses of morphine, 31 patients were discharged from the ED, while only 5 patients were discharged after receiving 3 or 4 doses of morphine. A departmental VOC protocol was followed for 25.4% of patients, with no impact on admission rate (p=0.290). In this retrospective analysis of a large series of pediatric sickle cell ED visits, patients presented later in their VOC, and admission rate was higher than previously reported. A small proportion of older patients with SCD accounted for most ED visits and hospitalizations. Hgb variation from steady-state was neither clinically significant nor predictive of admission or discharge. In contrast to previous studies, receiving IVF in the ED was associated with a greater likelihood of discharge. Home opioids prior to ED presentation seemed to decrease hospital admission. Adherence to our center’s VOC protocol did not appear to influence disposition from the ED.

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Trends in Hospital Utilization for Acute Illness in a Large Population-Based Cohort of Children and Adolescents with Sickle Cell Disease (SCD): 2010–2017

Blood ◽

10.1182/blood-2018-99-120067 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 3528-3528

Author(s):

Kristina Lai ◽

Sonia Anand ◽

Maa-Ohui Quarmyne ◽

Carlton Dampier ◽

Peter A. Lane ◽

...

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Large Population ◽

Admission Rate ◽

Acute Illness ◽

Hospital Utilization ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Admission Rates

Abstract Disease severity and healthcare utilization varies widely among persons with sickle cell disease (SCD). Hydroxyurea (HU) has been demonstrated to reduce rates of pain and acute chest syndrome, the leading causes of inpatient utilization in patients with Hb SS and S β°-thalassemia in clinical trials. We recently reported that HU was clinically effective in reducing rates of pain and acute chest syndrome in patients who initiate treatment. Use of hydroxyurea in SS/S β°-thalassemia has increased markedly since 2010. Thus we sought to detemine trends of hospital utilization for acute illness during an 8 yr in which HU utilization increased markedly (2010-2017). Data from years 2010-201 were obtained from the SCD database and patient records at Children's Healthcare of Atlanta (CHOA). Utilization data were restricted to acute care admissions. Admissions for elective procedures, non-SCD related discharge diagnoses, rare SCD genotypes, and patients who had undergone bone marrow transplant were excluded. Patients were compared based on number of hospitalizations, age, sex, SCD genotype (SS/S β°-thalassemia vs Hb SC/S β+thalassemia), and discharge diagnosis. A total of 3,116 patients had at least one encounter between 2010 and 2017; 2,947 patients met inclusion criteria. From 2010-2017 the total number of active patients per year increased from 1,546 patients to 1,789 patients (+16%), while the total number acute care admissions increased from 1,295 admissions to 1,609 admissions (+24%). There were no significant differences in the proportion of patients with genotypes SS/S β° thalassemia genotypes (67.0% vs 63.9%, p=0.06). Overall patients with SS/S β° thalassemia had higher admission rates compared to SC/S β+ thalassemia patients (0.94 vs 0.57 admissions per patient per year). During the study period. overall admission rates in SCD (acute illness hospitalizations/patient/yr) increased from a low of 0.74 in 2011 to a high of 0.90 in 2017. The proportion of admissions attributed to SS/S β°-thalassemia patients decreased (79.2% in 2010 vs 72.3% in 2017, p<0.0001). However, admission rate in SC/S β+ thalassemia increased (0.53 to 0.69 admissions per patient per year). Overall, over 60% of patients were not admitted in any given year, and the proportion of patients with 0-1 admissions in a given year remained unchanged. However, the proportion of super high hospital utilizers (SHHU), patients with 8 or more admissions in a given year, increased by 185%. In 2001 this group made up 0.6% of all patients and accounted for 7.3% of admissions; in 2017 SHHU had increased 1.8% of patients and 24.3% of admissions. There was no difference in genotype or sex between SHHU and non-SHHU patients. SHHU were older (>90% of patients over age 8 years), and had greater percentages of admissions for pain and acute chest syndrome then non-SHHU. In conclusion, during a period in which HU utilization in SS/S β°-thalassemia increased significantly, hospital utilization for acute illness in SS/S β° thalassemia decreased as expected. However, during the same period there was an unexpected increase in overall hospital utilization for acute illness in SCD. This increase in hospital utilization was the result of 1) a marked increase in SHHU and 2) an overall increased utilization in SC/S β+ thalassemia. Disclosures Dampier: Pfizer: Research Funding.

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The Use of Chronic Transfusions in Sickle Cell Disease for Non-Stroke Related Indications

Blood ◽

10.1182/blood.v124.21.4934.4934 ◽

2014 ◽

Vol 124 (21) ◽

pp. 4934-4934 ◽

Cited By ~ 1

Author(s):

Lindsay Mize ◽

Shelly Burgett ◽

Julia Xu ◽

Jennifer Rothman ◽

Nirmish Shah

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Emergency Department Visits ◽

Acute Chest Syndrome ◽

Blood Transfusions ◽

Recurrent Pain ◽

Cell Disease ◽

Pain Crisis

Abstract Introduction: Sickle cell disease (SCD) is a chronic disease that can cause significant complications including acute chest syndrome, recurrent pain and stroke. Current guidelines for the use of chronic transfusions include primary and secondary prevention of stroke. Although there is currently limited support for the routine use of transfusions for acute vaso-occlusive crisis (VOC), there has been increasing use of chronic transfusions as an alternative treatment for recurrent VOC. Moreover, there is evidence that patients on chronic transfusions have less VOC. We sought to review the outcomes of patients at our institution placed on chronic transfusions for non-stroke related indications. Methods: We performed a retrospective cohort study to summarize clinical and nonclinical features of sickle cell patients on transfusions for non-stroke related complications. Demographic, clinical, and laboratory information were summarized. Acute care events per month were calculated for both the year prior and up to one year following initiation of chronic transfusions. Acute care events were defined as emergency department visits or hospitalization. Results: Of the 378 patients with SCD treated in the pediatric specialty clinic, there were 21 patients being either chronically transfused or exchange transfused. Six (20%) of these patients were initiated on chronic blood transfusions (CBT) for recurrent pain crisis (median age = 12, range 8 to 17). One of these patients also had suspected hepatic sequestration. All patients were type SS and had been treated with hydroxyurea (HU) for an average length of 6.5 years (range 1 to 12 years) at a mean dose of 25 mg/kg (SD 4) prior to initiation of CBT. All patients continued on HU during chronic blood transfusions. Patients were on chronic transfusions for a median of 11 months (range 3 to 58 months) with mean %S while on transfusions of 39.6% (SD 10). Patients were transfused on average every 5 weeks (range 4 to 6 weeks). Following initiation of transfusions, 50% were started on chelation based on criteria of having a ferritin >1000 ng/mL. Mean peak ferritin was not significantly increased in the year following the start of CBT (513 ng/mL ± 343 vs. 1260 ± 934, p=0.13). There was one new alloantibodies (anti-Jk) reported following initiation of CBT, which developed within 3 months. Acute care visits per month were significantly higher in the year prior as compared to after initiation of chronic blood transfusions (1.04 ± 0.45 vs. 0.28 ± 0.22, respectively; p=0.006) (Figure 1). Discussion: We found that patients started on chronic transfusions for pain crisis had a non-significant increase in peak ferritin and a significant reduction in acute care visits. Prior to CBT, all patients had been initiated on hydroxurea (mean dose of 25mg/kg) in an attempt to treat recurrent VOC. However, following therapy for an average of 6.5 years, patients were placed on CBT to prevent further acute care visits and reduce morbidity. All patients were continued on HU while on CBT with no dose adjustment or effort to titrate to maximum tolerated dose. While on CBT, patients had a mean %S of 40%, which is higher than the recommended goal of 30% for stroke related indications. Importantly, despite the higher mean %S, there was a drastic and significant decrease in acute care visits. It should be noted that although only three patients (50%) of patients were placed on chelation, the remaining three had been on transfusions for less than or equal to 6 months and likely to require chelation with continued therapy. The expected elevated ferritin highlights the difficulty in long-term treatment with chronic transfusion and risk for eventual iron overload. The balance between the clinical benefit and potential long-term complications leads to individual assessment of the risks and benefits prior to initiation of chronic transfusions for VOC. These results advocate for the use of prospective studies to evaluate the role for chronic transfusions for non-stroke related indications in SCD. Figure 1 Figure 1. Disclosures Shah: Novartis: Speakers Bureau.

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Managing Sickle Cell Disease Patients with Acute Pain Crisis in the Most Appropriate Setting

Blood ◽

10.1182/blood-2019-131102 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 2169-2169

Author(s):

Michel Gowhari ◽

Tiesa Hughes-Dillard ◽

Catherine Ryan ◽

Alexia Johnson

Keyword(s):

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Acute Pain ◽

Admission Rate ◽

Cell Disease ◽

Observation Unit ◽

Improvement Project ◽

Hospital Admission Rate ◽

Pain Crisis

Introduction: Pain is the top concern of individuals with sickle cell disease (SCD). Acute painful vaso-occlusive episodes in SCD are the leading cause of emergency department (ED) encounters and frequent hospital admissions. Well-documented disparities include significant delay and under treatment of SCD patients with acute pain crisis in the ED. An acute care observation unit (ACOU) staffed with SCD specialists can help to address these disparities. SCD patients treated in a dedicated ACOU have a 40% lower admission rate than patients treated in the ED. An expedited transfer and treatment program at our dedicated sickle ACOU at the University of Illinois Hospital (UIH) was implemented with the goal of improving overall care and decreasing the hospital admission rate for SCD patients. Method: This is an outcome study of individuals with SCD >16 years of age who presented with an acute painful episode to UIH. A quality improvement project used the Plan-Do-Study-Act translation method. The following key areas were identified for intervention: 1) established criteria for direct ACOU admission, 2) expedited transfer to the ACOU from the ED, 3) addition of a third provider to expand hours, and 4) establishing a consistent but individualized pain treatment plan across the ED and ACOU. The number of admissions to hospital of patients with SCD was examined from September 2018 through August 2019. Applying the Donabedian triad of Structure, Process, and Outcomes, we demonstrated improved outcome and decrease hospitalization. Results: There were 877 admissions to the ACOU from January to July of 2019, which is an increase of 37% compared to the same period in 2018. Of the 877 admissions, 793 were discharged home (90.4%) as compared to 88.6 % in 2018. The average time to first dose of opioids in the ACOU in 2019 was 55 minutes with and average decrease in the pain score of 2.62 during an average length of stay of 4:18 hours. Conclusions: Expedited care and treatment with a focus on improving quality and improving access resulted in increased volume of patients treated and decreased rate of admission to the hospital. Allocating resources to a dedicated sickle acute care observation unit can significantly decrease inpatient hospitalizations. Table. Disclosures No relevant conflicts of interest to declare.

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Inpatient pain management in sickle cell disease

American Journal of Health-System Pharmacy ◽

10.1093/ajhp/zxz228 ◽

2019 ◽

Vol 76 (23) ◽

pp. 1965-1971 ◽

Cited By ~ 2

Author(s):

Stefanie M Zassman ◽

Francis J Zamora ◽

John D Roberts

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Acute Pain ◽

Pain Control ◽

Hospital Admissions ◽

Healthcare Providers ◽

Emergency Department Visits ◽

Cell Disease ◽

Usual Practice ◽

Blood Disorder

Abstract Purpose A novel strategy for management of acute pain associated with sickle cell disease (SCD), referred to as the oral tier approach, is described. Summary SCD is an inherited blood disorder characterized by episodic acute pain known as vaso-occlusive crisis (VOC), which is the most common reason for emergency department visits and hospital admissions in patients with SCD; these patients are often treated with parenteral opioids on admission and then transitioned to oral opioids prior to discharge. In this report, experience with use of the oral tier approach in 3 patients with SCD hospitalized for management of VOC is reported. As per usual practice, acute pain was initially managed with parenteral opioids via patient-controlled analgesia (PCA). Once pain control was established, an oral tier was added. The oral tier consisted of 3 orders. The first order was for an oral opioid, to be administered every 3 hours on a scheduled basis; however, the patient could refuse 1 or more of these scheduled doses. Two additional orders specified that the patients could receive additional oral opioids in incremental doses for moderate (grade 4–7) or severe (grade 8–10) pain if appropriate. To facilitate transition to an oral regimen with which the patients might be discharged, they were encouraged to use oral opioids in preference to parenteral opioids. Opioid usage and average daily pain scores for the 3 patients are reported. Conclusion Healthcare providers can use the oral tier approach to facilitate rapid inpatient conversion from i.v. PCA to oral opioids while providing adequate pain control in patients with SCD who develop VOC.

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Opioid management strategy decreases admissions in high-utilizing adults with sickle cell disease

Journal of Opioid Management ◽

10.5055/jom.2017.0382 ◽

2017 ◽

Vol 13 (3) ◽

pp. 143 ◽

Cited By ~ 6

Author(s):

Amy Mager, PA-C, MPAS ◽

Kristin Pelot, MSSW ◽

Kathryn Koch, APNP ◽

Lawrence Miller, PsyD ◽

Collin Hubler, BS ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Management Strategy ◽

Hospital Admissions ◽

Admission Rate ◽

Psychological Disorder ◽

Successful Implementation ◽

Cell Disease ◽

Opioid Use ◽

Percent Decrease

Background: A subset of adults with sickle cell disease (SCD) heavily utilizes the emergency department (ED) and hospital. The objective of our study was to determine the efficacy of a multidisciplinary strategy to address unmet needs in highly utilizing adults with SCD.Methods: In a prospective study, adults with SCD with ≥10 admissions per year were assessed by a multidisciplinary team for gaps in medical, social, and psychological care. Thereafter, the team decided upon the subject's predominant domain that drove admissions and instituted an interventional plan. All plans included an opioid management strategy. Preintervention and postintervention admission rate, as well as opioid use, was compared.Results: Twelve subjects were enrolled. Median rate of ED and hospital admissions preintervention was 25 per year. The predominant domains identified were social needs (n = 6), psychological disorder (n = 1), and substance use disorder (n = 5). Multifaceted interventional plans were developed to address a wide range of gaps in care, but an opioid management strategy was the only intervention successfully completed. Even so, when the preintervention versus postintervention admission rate was compared, regardless of the domain, there was a 40 percent decline in hospital admissions (p = 0.03). Consistent with the successful implementation of an opioid management plan, the decrease in admissions was accompanied by a 37 percent decrease in intravenous opioid use (p = 0.02) and 10 percent decrease in oral opioid use (p = 0.04).Conclusion: An opioid management strategy, as part of a larger effort to improve care for high-utilizing adults with SCD, decreased rate of admissions and opioid use.

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

Blood ◽

10.1182/blood-2019-122199 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 999-999

Author(s):

Romy Carmen Lawrence ◽

Sarah L Khan ◽

Vishal Gupta ◽

Brittany Scarpato ◽

Rachel Strykowski ◽

...

Keyword(s):

Venous Thromboembolism ◽

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Hospital Admissions ◽

Care Utilization ◽

Cell Disease ◽

History Of ◽

Acute Care Utilization ◽

Ed Visits

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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Acute Care Visits in Sickle Cell Disease: a Population-Based Multi- State Study

Blood ◽

10.1182/blood.v112.11.165.165 ◽

2008 ◽

Vol 112 (11) ◽

pp. 165-165 ◽

Cited By ~ 1

Author(s):

David Brousseau ◽

Julie A. Panepinto ◽

Pamela Owens ◽

Claudia Steiner

Keyword(s):

Emergency Department ◽

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Population Based ◽

Emergency Department Visits ◽

Cell Disease ◽

Principal Diagnosis ◽

Inpatient Hospitalization ◽

Entire Cohort

Abstract Background: The number of times a patient will seek acute care in the emergency department or require hospitalization for sickle cell related illness has not been described in a population-based manner. Twenty years ago, rates of acute care visits for 3,578 patients who were part of the Cooperative Study of Sickle Cell Disease were reported, eloquently describing patterns of acute care utilization for people followed at select centers. In that study, only 1% of patients had more than six visits per year and 5% of the population (who made three to 10 visits per year) accounted for almost one-third of all visits. The objective of this study is to describe the emergency department and hospital utilization for patients with sickle cell related conditions over a two- year period. This study will be the first to provide a complete assessment of the utilization patterns of patients with sickle cell disease, one that is multi-state, inclusive of all ages, all insurance types, and includes patients that are followed at community, academic and tertiary care centers. Methods: We conducted a retrospective cohort study using 2005 and 2006 State Emergency Department and State Inpatient Databases that include encrypted personlevel identifiers to allow linkage of record level information. The data are from the Healthcare Cost and Utilization Project (HCUP), a Federal-State-Industry partnership sponsored by the Agency for Healthcare Research and Quality. Data for all sickle cell related emergency department visits and hospitalizations within the following seven states (Arizona, California, Florida, Missouri, South Carolina, Tennessee, and New York) were extracted for each patient. To be eligible, a patient had at least one sickle cell specific visit, defined as a visit with a principal diagnosis of sickle cell crisis or a secondary diagnosis of sickle cell disease with a principal diagnosis that was sickle cell related (e.g. pneumonia, stroke, fever). All sickle cell related visits were linked by unique personal identifiers, thus clustering visits by patient and allowing population-based statewide assessments of utilization. An emergency department visit on the same day as an inpatient hospitalization was counted only as an inpatient hospitalization to avoid over counting care-seeking visits. The distribution of acute care visits for each patient (presented as numbers of emergency department visits and hospitalizations over the two-year period) was determined for the entire cohort, then stratified as child versus adult. Results: A total of 24,668 patients with sickle cell disease made 86,535 acute care visits during the two-year study period, 33,520 (38.7%) were emergency department visits and 53,015 (61.3%) were inpatient visits. Of the 24,668 patients, 8,895 (36.1%) were less than 18 years of age; 15,773 were adults. 52.8% of the entire cohort made one visit in the two year period. 1,320 (5.4%) patients had more than 12 visits over the two-year time period; 3,210 (13.0%) made 6–20 visits over two years, and accounted for 31,752 (36.7%) acute care visits. An additional 579 (2.4%) patients had more than 20 visits over two years, accumulating 18,701 (21.6%) acute care visits. Children were less likely to have more than 12 visits over the two years (1.9%) compared to adults aged 18–45 (8.1%) and were also less likely to be in the high utilization group of 6–20 visits over two years (9.9% of children compared to 15.8% of those 18–45 years old). Conclusions: A significant proportion of patients with sickle cell disease seek acute care multiple times in an emergency department setting or through hospitalization. Our population-based study demonstrates an increased proportion of high utilizers compared to previous work, especially among adult patients. Our findings likely reflect the difference in healthcare utilization in the broader community as compared to that within a cooperative study in academic settings. It suggests that some patients, adults in particular, may have limited access to urgent care in a primary care setting and would benefit from better access and more aggressive preventive care. Further work on patterns of and reasons for utilization, especially emergency department care, in this high-utilizer group, would be helpful in targeting and improving overall care for these patients.

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Elective Cholecystectomy Decreases the Morbidity of Cholelithiasis and Cholecystectomy in Pediatric Sickle Cell Disease.

Blood ◽

10.1182/blood.v114.22.1544.1544 ◽

2009 ◽

Vol 114 (22) ◽

pp. 1544-1544

Author(s):

Emily F. Goodwin ◽

Paige M. Ivey ◽

Jeffrey D. Lebensburger ◽

Roy P. McDonald ◽

Thomas H. Howard

Keyword(s):

Abdominal Pain ◽

Sickle Cell Disease ◽

Hospital Admission ◽

Sickle Cell ◽

Hospital Admissions ◽

Conflicts Of Interest ◽

Cell Disease ◽

Surgical Preparation ◽

The Impact ◽

Elective Cholecystectomy

Abstract Abstract 1544 Poster Board I-567 PURPOSE Cholelithiasis frequently occur in sickle cell disease, are easily diagnosed by ultrasound, and are associated with hospital admissions for abdominal pain. Elective cholecystectomy is controversial in sickle cell disease despite small series which suggest that elective cholecystectomy decreases the morbidity of the procedure. Therefore we examined the impact of cholecystectomy on morbidities associated with cholelithiasis and cholecystectomy in sickle cell disease. METHODS: Records of 191 consecutive pediatric sickle cell patients with cholelithiasis who underwent cholecystectomy between January 1999 and May 2009 were retrospectively reviewed. The cholecystectomies were classified into 3 groups 1) elective: no pre-operative symptoms, cholelithiasis on screening ultrasound, pre-planned surgical preparation; 2) symptomatic: pre-operative symptoms of cholelithiasis on ultrasound, pre-planned surgical preparation; 3) emergent: hospitalization for acute cholecystitis symptoms, cholelithiasis on ultrasound, no pre-planned surgical preparation. We compared the morbidity of cholelithiasis and cholecystectomy by examining pre-operative hospitalizations, cholecystectomy hospitalization, and post-operative hospitalizations. RESULTS: Patients with sickle cell disease underwent a total of 191 cholecystectomies over a ten year period: 51 elective, 110 symptomatic, and 30 emergent. Patients who required an emergent cholecystectomy had a longer post-operative hospitalization time than elective cholecystectomy (7.3 vs 4.3 P< 0.001). Prior to hospitalization for the cholecystectomy, patients needing emergent and elective cholecystectomy had similar number of total of hospital admission days (5.2 vs 5.6 P=0.73). However, the emergent cholecystectomy population required more hospital admission days prior to surgery for abdominal pain than the elective patients (1 vs 0.37, P=0.01). After the cholecystectomy, emergent patients required more total hospital admission days (7.2 vs 2.9, P=0.002) and more admission days for abdominal pain (0.5 vs 0.2, P=0.049) than patients that underwent elective cholecystectomy. In 18 patients with the most severe abdominal pain (>2 inpatient admission days) prior to hospitalization for cholecystectomy, 11 (61%) were not admitted after cholecystectomy for pain. Patients receiving chronic blood transfusions prior to surgery had a reduced need for emergent cholecystectomy as compared to non-transfused patients (8% vs 25%, P= 0.056 by chi-square). Patients receiving hydroxyurea had a similar rate of need for an emergent cholecystectomy as compared to patients not on hydroxyurea (22% vs 13% P= NS). No differences in degree of anemia or reticulocytosis were identified in patients requiring emergent vs. elective cholecystectomy (Hb: 8.7 vs 8.6 g/dL; Reticulocyte percent: 10.9% vs 10.4%). The morbidity of patients in the symptomatic cohort was intermediate between the elective and emergent cohorts without demonstrating statistical significance. CONCLUSIONS: This represents the largest reported series of pediatric cholelithiasis and cholecystectomy in sickle cell disease to date. This data strongly suggests that elective cholecystectomy decreases morbidity associated with cholelithiasis and cholecystectomy in sickle cell disease. Disclosures No relevant conflicts of interest to declare.

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