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

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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Beneficial effects of adenotonsillectomy in children with sickle cell disease

ERJ Open Research ◽

10.1183/23120541.00071-2020 ◽

2020 ◽

Vol 6 (4) ◽

pp. 00071-2020

Author(s):

Ilaria Liguoro ◽

Michele Arigliani ◽

Bethany Singh ◽

Lisa Van Geyzel ◽

Subarna Chakravorty ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Arterial Oxygen Saturation ◽

Arterial Oxygen ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Beneficial Effects ◽

Admission Rates ◽

The Mean ◽

The Impact

Tonsillectomy and adenoidectomy (T&A) is frequently performed in children with sickle cell disease (SCD). Our aim was to evaluate the impact of this surgery on overnight oxygenation and rates of complications in these patients.Children with SCD who underwent T&A between 2008 and 2014 in two tertiary hospitals were retrospectively evaluated. Overnight oximetry and admission rates due to vaso-occlusive pain episodes (VOEs) and acute chest syndrome (ACS) in the year preceding and following the surgery were compared.19 patients (10 males, 53%) with a median age of 6 years (range 3.5–8) were included. A significant increase of mean overnight arterial oxygen saturation measured by pulse oximetry (SpO2) (from 93±3.6% to 95.3±2.8%, p=0.001), nadir SpO2 (from 83.0±7.1% to 88±4.1%, p=0.004) and a reduction of 3% oxygen desaturation index (from a median value of 5.7 to 1.8, p=0.003) were shown. The mean annual rate of ACS decreased from 0.6±1.22 to 0.1±0.2 events per patient-year (p=0.003), while the mean cumulative rate of hospitalisations for all causes and the incidence of VOEs were not affected.T&A improved nocturnal oxygenation and was also associated with a reduction in the incidence of ACS at 1-year follow-up after surgery.

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Inpatient Utilization Among Children and Adolescents with Sickle Cell Disease: Analysis of a Large Populated-Based Retrospective Cohort

Blood ◽

10.1182/blood.v126.23.3262.3262 ◽

2015 ◽

Vol 126 (23) ◽

pp. 3262-3262 ◽

Cited By ~ 1

Author(s):

Rodney Theodore ◽

Vaughn Barry ◽

Maa-Ohui Quarmyne ◽

Carlton Dampier ◽

Peter A. Lane

Keyword(s):

Sickle Cell Disease ◽

Children And Adolescents ◽

Hospital Discharge ◽

Sickle Cell ◽

Hospitalization Rate ◽

Hospital Utilization ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Readmission Rates ◽

Hospitalization Rates

Abstract Background: Sickle cell disease (SCD) is characterized by marked heterogeneity in clinical outcomes, severity and utilization of health care services. This heterogeneity is particularly evident with regards to utilization of inpatient hospital services. Some children with SCD are frequently admitted to the hospital while others are rarely or never admitted. In addition, rates of readmission within 30 days of hospital discharge are high in SCD. The causes of this variable utilization and high rates of readmission are not well understood. Objectives: We sought to determine rates and primary causes of SCD-related hospital utilization among children and adolescents with SCD and to determine whether rates of hospitalization and of 7-, 14- and 30-day readmissions varied by age, SCD genotype, gender and cause of hospitalization. Methods: Children who lived in the 28-county greater metro Atlanta area with a confirmed SCD diagnosis and who received care at the ChildrenÕs Healthcare of Atlanta (CHOA) SCD program between January 1, 2010 through December 31, 2014 were included. The earliest and latest encounter during the five-year period was used to determine total length of observation for each patient. To ensure a substantial period of observation, individuals with two consecutive encounters >18 months apart or with < 2 yr of observation were excluded. SCD genotype (SS, SC, and Sβ0 and Sβ+ thalassemia) was confirmed for each patient by review of hematologic and clinical data, including results of diagnostic hemoglobin electrophoresis; those with rare SCD genotypes (e.g. SD, SE) were excluded. The primary cause for each admission was determined through medical chart review and classified into 4 mutually exclusive hierarchical categories in the following descending order: acute chest syndrome (ACS), pain crisis, fever/infection, and other complications of SCD (i.e. an admission for both pain and ACS was categorized as ACS). Scheduled hospitalizations for elective procedures (e.g. splenectomy, cholecystectomy, venous catheter placement) were excluded. A readmission was defined as a hospitalization occurring within 7, 14, or 30 days of a previous hospital discharge. Results: The analysis included 1,331 individuals with 5,362 person-years of observation; 68% had SS/Sβ0 thal genotypes, 49% were male. Age at the time of the earliest encounter ranged from 2 months to 19 years; average length of observation was 4.02 years (min, max= 2, 5). Total n of hospital admissions was 5,317; 19.4% were never hospitalized, and 44.8% were hospitalized less than once per year. The most common primary cause for hospital admission was pain (53.1% of admissions). Overall and cause-specific hospitalization rates varied by age and genotype (Figure) with overall hospitalization rates lowest among children 4-9 years old compared to other ages (0.68 vs. 1.00 admissions per person-year) and highest among those with SS or Sβ0 thal compared to those with SC or Sβ+ thal (0.97 vs. 0.68 admissions per person-year). Hospitalization rates associated with pain increased with age while rates for fever/infection decreased with age. Of the 1,073 patients who were admitted, 356 were readmitted within 30 days of a previous admission at least once. All-cause 7-, 14- and 30-day readmission rates were 5.6%, 10.0% and 18.2%, respectively. 30-day readmission rates were lower among younger age groups (15.7% for age 1-3 yr, 15.3% for 4-6 yr, 15.8% for 7-9 yr) but higher in older patients (18.3% for 10-12yr, 19.9% for 13-15yr, and 23.3% for 16-18yr). Readmission rates were highest following index admissions for pain (20.4%) and lowest for ACS (11.3%). Gender was not associated with readmission or cause of readmission. Conclusion: SCD-related hospitalization rates were highest in early life and in later adolescence with admissions for fever/infection most common in younger children and admissions for pain crises in older children. Rates of readmission strongly correlated with age and were highest following admissions for pain. These data highlight to need to develop and implement improved strategies prevention and treatment of pain in children and adolescents with SCD. Further studies should also explore individual and/or family factors that may contribute to the high rates of hospital utilization and 30-day readmissions that occur among a relatively small percentage of patients. Figure 1. Hospitalization rate per person-year by type of admission and age Figure 1. Hospitalization rate per person-year by type of admission and age Disclosures No relevant conflicts of interest to declare.

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Children with Sickle Cell Disease on Chronic Red Cell Transfusion Experience Fewer Hospitalizations for Acute Vaso-Occlusive Episodes Irrespective of the Indication for Transfusion

Blood ◽

10.1182/blood.v124.21.4282.4282 ◽

2014 ◽

Vol 124 (21) ◽

pp. 4282-4282

Author(s):

Carmen C Wallace ◽

Hilda Mata ◽

Nancy J Wandersee ◽

J. Paul Scott ◽

Amanda M Brandow ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Admission Rate ◽

T Test ◽

Acute Chest Syndrome ◽

Red Cell ◽

Cell Disease ◽

Splenic Sequestration ◽

Red Cell Transfusion ◽

The Impact

Abstract While there is strong evidence that chronic red cell transfusion is effective in preventing primary stroke and reducing the risk of recurrent stroke in sickle cell disease (SCD), it is less clear whether chronic transfusions will prevent admissions for other acute vaso-occlusive complications, including pain, priapism and/or acute chest syndrome. To our knowledge, no study to date has investigated the effect of chronic transfusion on the frequency of admissions for acute vaso-occlusive complications in children with all diagnoses of SCD and treated with chronic transfusion for a variety of indications. In addition, this study included a special focus on the effect of chronic transfusion on children who were transfused specifically for recurrent vaso-occlusive episodes. We performed a single-site retrospective chart review. We selected subjects from all children aged 0 to 19 years who were treated (lived in the Milwaukee area) and followed by the Wisconsin Sickle Cell Center at Children’s Hospital of Wisconsin from 1984 to May 2014 (n=695 subjects). Data was extracted from any individual who was enrolled in a chronic transfusion program for a minimum of six months. Data on admissions for painful vaso-occlusive crises, acute chest syndrome (ACS), other SCD complications as well as sickle diagnosis, age at time of transfusion, CBC, reticulocyte count, and percent sickle hemoglobin (HbS%) were collected for 24 months prior to onset of transfusion and for all months during transfusion until the age of 19 yrs. Unless otherwise indicated, all statistical analyses on extracted data were done by paired Student’s t-Test. We extracted data from 103 unique subjects for 108 chronic transfusion programs (as defined above); 5 subjects were chronically transfused twice, separated by at least 4 years without chronic transfusion. 55% were female; average age was 8.6 ± 5.6 (mean ± SD) years and the sickle diagnosis included 94% SS, 3% SC, 2% Sβ°-Thalassemia and 1% SD. The indication for transfusion included pain (n=31), priapism (n=6), ACS (n=5), central nervous system complications (n=37, including stroke, TIA, and abnormal TCD), splenic sequestration (n=25), pulmonary hypertension (n=2), retinopathy (n=1) and osteomyelitis (n=1). The hemoglobin level increased from a baseline of 7.6 ± 2.2 gm/dL to 9.6 ± 0.8 gm/dL during transfusion (p<0.0001, paired t-Test). HbS% was also reduced from a baseline of 84.2 ± 10.8% to 35.8 ± 0.3% during transfusion (p<0.0001). We found that rate of admissions for acute painful episodes, including priapism, dropped from 2.2 ± 2.9 admits/yr during the 24 months pre-transfusion to 1.0 ± 1.9 admits/yr during transfusion (p<0.0001). Similarly, the rate of admission for ACS decreased from 0.3 ± 0.5 admits/yr for 24 months pre-transfusion to 0.1 ± 0.3 admits/yr during transfusion (p=0.0001). Subanalyses were performed on specific indications for transfusion. For children transfused due to frequent acute vaso-occlusive complications (pain, priapism and ACS were arbitrarily included in this group), the average age at initiation of transfusion was 11.9 ± 4.4 yr, and admissions for acute painful episodes dropped from 4.0 ± 3.2 admits/yr during the 24 months pre-transfusion to 2.1 ± 2.6 admits/yr during transfusion (p=0.003). When the indication for transfusion was splenic sequestration (age 2.3±2.7 yr), the admission rate for acute painful episodes did not change (0.8±1.7 vs 0.3±0.5 admits/yr, p= 0.14). For children transfused for CNS complications (age 8.5±4.6 yr), the admission rate for pain improved from 0.9±1.3 to 0.2±0.5 admissions/yr (p=0.007). In agreement with previous studies, our data also showed an increase in the rate of admissions for pain (nontransfused) as subjects aged (r2=0.19, p<0.0001). Thus, the significant improvement in admission rate for pain during transfusion, while the child continues to age, further accentuates the impact of transfusion on the natural history of pain in SCD. In summary, our data suggest that chronic transfusion reduces hospital admissions for pain and acute chest syndrome in children with SCD. Our data also support the notion that chronic transfusion is an effective treatment to prevent not only stroke, but also other painful, life-threatening and life-limiting complications of sickle cell disease. Disclosures No relevant conflicts of interest to declare.

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

Blood ◽

10.1182/blood.v126.23.992.992 ◽

2015 ◽

Vol 126 (23) ◽

pp. 992-992

Author(s):

Fuad El Rassi ◽

Eldrida Randall ◽

Sidney F. Stein ◽

Hanna Jean Khoury ◽

James R. Eckman ◽

...

Keyword(s):

Sickle Cell Disease ◽

Hospital Admission ◽

Acute Care ◽

Sickle Cell ◽

Hospital Admissions ◽

Admission Rate ◽

Emergency Department Visits ◽

Cell Disease ◽

Temperature And Humidity ◽

The City

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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Acute Chest Syndrome Rate at the Emory University Georgia Comprehensive Sickle Cell Center at Grady Health System

Blood ◽

10.1182/blood.v126.23.3420.3420 ◽

2015 ◽

Vol 126 (23) ◽

pp. 3420-3420

Author(s):

Fuad El Rassi ◽

Eldrida Randall ◽

Sidney F. Stein ◽

H. Jean Khoury ◽

James R. Eckman ◽

...

Keyword(s):

Platelet Count ◽

Sickle Cell Disease ◽

Acute Care ◽

Sickle Cell ◽

Acute Chest Syndrome ◽

Management Approach ◽

Cell Disease ◽

Comprehensive Management ◽

Emory University ◽

D Dimer

Abstract Background: Acute chest syndrome (ACS) is one of the most feared complications of sickle cell disease. This syndrome is defined by a clinical constellation of symptoms including fever, infiltrate on lung imaging and hypoxia. ACS is a well-known complication to occur early in the hospitalization of patients for sickle cell vaso-occlusive pain crises (VOP). It is hard to differentiate from pneumonia and its rate of occurrence has not been well characterized. Management of ACS includes exchange or simple transfusion, depending on the severity of its presentation, along with critical care support and pain control. Early recognition of this clinical syndrome can lead to better management and outcomes for patients with sickle cell disease. Methods: The Emory University Georgia Comprehensive Sickle Cell Center at Grady Health System has 1073 sickle cell patients and provides a 24/7 acute care unit (ACU) for the evaluation of patients presenting with pain crises. We conducted a retrospective review of all the ACS cases reported at the Center for the last 5 years. Results: The number of sickle cell patient-visits in the ACC was 4409. 30 cases of ACS were confirmed on retrospective review. The rate of ACS was 6.8 per 1000 patient visits. The median age was 39, and the patients' phenotypes 89% SS and 11% SC. Of the patients evaluated, 18% were smokers, 43% were taking hydroxyurea and 89% were taking folic acid. Seventy five percent of the patients were successfully managed with supportive care only, while the remaining 25% required red cell exchanges. On admission, the median white cell count (WBC), platelet count, LDH and reticulocyte count were increased above their upper limits of normal. Eighteen percent of the patients had a d-dimer checked and the median was well above the range of normal (6097). Table 1 shows the patient characteristics and data. Discussion: The rate of ACS over the last 5 years at our sickle cell center is well below historic reports of ACS. We believe that several factors explain our observation. Because our 24/7 ACU is staffed with providers who know their patients' histories and treatment plans and are experienced in the management of VOP, we postulate that our comprehensive management approach involving the rapid and appropriate initiation of intravenous fluids, the administration of pain medications along with antibiotics, and the use of anti-inflammatory medications contribute to the truncation of the sickle cell-related inflammatory state and the prevention of serious complications such as ACS. The fact that inflammatory markers (WBC, Platelet count, LDH) were initially elevated is to be expected in the setting of VOP. What is remarkable is the high percentage of good patient outcomes associated with our comprehensive management approach. This retrospective report should be validated in a prospective study that compares the results of the management of all acute care VOP visits delivered in a 24/7 ACU with the management of acute care VOP delivered in a general emergency room. Such a study should include the collection of laboratory and clinical data that is typically abnormal in patients with VOP. If a significantly decreased rate of ACS rate is observed when 24/7 ACU care is provided, then the institution of 24/7 acute care units would be warranted on a national level to improve the survival of patients with sickle cell disease. Table 1. Characteristics of patients studied Age median (range) 39 (19-58) Gender Females 14/ males 14 Genotype n (%) SC 3 (11%)/ SS 25 (89%) Smoking (%) 18 % Hydrea use (%) 43% Folic acid use (%) 89% Management n (%) Exchanged 7 (25%)/ supportive 21 (75%) Hemoglobin median (range) 10.4 (4.9-11.1) White count median (range) 18.6 (6.1-47) Platelet median (range) 492 (79-646) Reticuolcyte count median (range) 300.6 (151-597) LDH median (range) 677 (238-2287) D-dimer % patients/ median (range) 18%/ 6097 (609-33370) Ferritin % patients/ median (range) 11%/ 2844 (335.2-6060) Total/direct bilirubin median (range) 3.7/1.3 (1-19/0.2-4.9) Creatinine median (range) 1.3 (0.5-3.5) AST/ALT median (range) 59/49 (21-110/12-118) Disclosures No relevant conflicts of interest to declare.

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ECMO Therapy in Acute Chest Syndrome for Patients with Sickle Cell Disease: a Case Report and Literature Review

SN Comprehensive Clinical Medicine ◽

10.1007/s42399-021-00987-0 ◽

2021 ◽

Author(s):

Soi Avgeridou ◽

Ilija Djordjevic ◽

Anton Sabashnikov ◽

Kaveh Eghbalzadeh ◽

Laura Suhr ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Scientific Data ◽

Acute Chest Syndrome ◽

Data Sets ◽

Limited Data ◽

Cell Disease ◽

Life Saving ◽

Institutional Experience ◽

Ecmo Therapy

AbstractExtracorporeal membrane oxygenation (ECMO) plays an important role as a life-saving tool for patients with therapy-refractory cardio-respiratory failure. Especially, for rare and infrequent indications, scientific data is scarce. The conducted paper focuses primarily on our institutional experience with a 19-year-old patient suffering an acute chest syndrome, a pathognomonic pulmonary condition presented by patients with sickle cell disease. After implementation of awake ECMO therapy, the patient was successfully weaned off support and discharged home 22 days after initiation of the extracorporeal circulation. In addition to limited data and current literature, further and larger data sets are necessary to determine the outcome after ECMO therapy for this rare indication.

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Streptococcus pneumoniae and Its Virulence Factors H2O2 and Pneumolysin Are Potent Mediators of the Acute Chest Syndrome in Sickle Cell Disease

Toxins ◽

10.3390/toxins13020157 ◽

2021 ◽

Vol 13 (2) ◽

pp. 157

Author(s):

Joyce Gonzales ◽

Trinad Chakraborty ◽

Maritza Romero ◽

Mobarak Abu Mraheil ◽

Abdullah Kutlar ◽

...

Keyword(s):

Streptococcus Pneumoniae ◽

Sickle Cell Disease ◽

Virulence Factors ◽

Sickle Cell ◽

Biological Activities ◽

Acute Chest Syndrome ◽

Antibiotics Resistance ◽

Cell Disease ◽

Antibiotic Drug ◽

Tract Infections

Sickle cell disease (SCD) is one of the most common autosomal recessive disorders in the world. Due to functional asplenia, a dysfunctional antibody response, antibiotic drug resistance and poor response to immunization, SCD patients have impaired immunity. A leading cause of hospitalization and death in SCD patients is the acute chest syndrome (ACS). This complication is especially manifested upon infection of SCD patients with Streptococcus pneumoniae (Spn)—a facultative anaerobic Gram-positive bacterium that causes lower respiratory tract infections. Spn has developed increased rates of antibiotics resistance and is particularly virulent in SCD patients. The primary defense against Spn is the generation of reactive oxygen species (ROS) during the oxidative burst of neutrophils and macrophages. Paradoxically, Spn itself produces high levels of the ROS hydrogen peroxide (H2O2) as a virulence strategy. Apart from H2O2, Spn also secretes another virulence factor, i.e., the pore-forming exotoxin pneumolysin (PLY), a potent mediator of lung injury in patients with pneumonia in general and particularly in those with SCD. PLY is released early on in infection either by autolysis or bacterial lysis following the treatment with antibiotics and has a broad range of biological activities. This review will discuss recent findings on the role of pneumococci in ACS pathogenesis and on strategies to counteract the devastating effects of its virulence factors on the lungs in SCD patients.

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