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

Blood ◽  
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.

Sevuparin for the treatment of acute pain crisis in patients with sickle cell disease: a multicentre, randomised, double-blind, placebo-controlled, phase 2 trial

2021 ◽  
Vol 8 (5) ◽  
pp. e334-e343
Author(s):  
Bart J Biemond ◽  
Anil Tombak ◽  
Yurdanur Kilinc ◽  
Murtadha Al-Khabori ◽  
Miguel Abboud ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Phase 2 ◽  
Cell Disease ◽  
Double Blind ◽  
Double Blind Placebo ◽  
Phase 2 Trial ◽  
Pain Crisis

scholarly journals 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 ◽  
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.

scholarly journals Hematological Changes from Baseline in Children with Sickle Cell Disease Admitted for Acute Chest Syndrome Compared to Acute Pain Crisis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4909-4909
Author(s):  
Timothy Klouda ◽  
Nataly Apollonsky ◽  
Deepti Raybagkar ◽  
Bruce Bernstein
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Neutrophil Count ◽  
Laboratory Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
History Of ◽  
Pain Crisis ◽  
Hematological Changes

Abstract Title: Hematological Changes from Baseline in Children with Sickle Cell Disease Admitted for Acute Chest Syndrome Compared to Acute Pain Crisis Authors: Timothy Klouda1, Deepti. Raybagkar2, Bruce Bernstein1, Nataly Apollonsky2, Institutes:1Pediatrics, St Christopher's Hospital for Children, Philadelphia, PA, United States, 2Hematology, St Christopher's Hospital for Children, Philadelphia, PA, United States, Introduction: Children with Sickle Cell Disease suffer from multiple complications including acute pain crisis (VOC) and acute chest syndrome (ACS). Nearly 30% of children with SCD have had one episode of ACS, with the incidence higher in early childhood. The proposed pathophysiology of ACS is thought to be multi-factorial, with pulmonary fat embolism or infectious etiology being identified in a large number of patients. Increased sickling due to hypoxemia or pain has been shown to place patients at risk for ACS development., Studies have shown an increase in inflammatory markers including leukocytes and neutrophils, along with a decreased hemoglobin in SCD children who developed ACS, but no studies to date have compared laboratory changes during the acute illness to their baseline values. We hypothesized that children with SCD who are admitted for ACS will have a larger decrease in hemoglobin from baseline and a higher increase in white blood cell count from baseline when compared to those admitted for an acute pain crisis. Methods: Through retrospective chart review of patients with SCD admitted to St.Christopher's Hospital for Children we identified 45 patients with ACS. Laboratory data collected on admission from chart review included SCD genotype, age, BMI, hemoglobin, white blood cell count, absolute neutrophil count, absolute eosinophil count, platelets, reticulocyte count, hemoglobin F, vital signs and medication history. All 45 children had laboratory data collected from an acute pain crisis that occurred during a different admission for comparison. Collected data was compared to baseline laboratory data, collected during routine visit at sickle cell clinic within 1 year of admission. Changes in laboratory data from baseline during admission for ACS were compared to changes during admission for uncomplicated VOC. Results: Children with SCD who were admitted or developed ACS during admission had a larger increase in leukocyte count (6.99 vs 4.18, p=0.027) and neutrophil count (6.3 vs 3.74, p=0.04) from baseline compared to those admitted for VOC alone. Patients with ACS development also had a larger decrease in platelets (-124.74 vs -56.21, p=.047) from baseline when compared to VOC admissions. There was no statistically significant change from baseline labs when comparing hemoglobin (p=0.10), eosinophil count (p=.382), reticulocyte count (p=0.754), AST (p=0.061) and ALT (p=0.082) in the ACS and VOC groups. Children with a history of 2 or more lifetime ACS were more likely to have OSA (p=0.021), 3 or more VOCs in the past year (p=0.002), and a history of splenectomy, but it was not found to be statistically significant (p=0.155) Conclusion: Children with SCD who developed or were admitted with ACS had a significant increase in leukocyte and neutrophil count from baseline, and a decrease in platelets when compared to VOC admissions. There was no significant change from baseline in hemoglobin, reticulocyte and eosinophils detected. Future larger and multi-center prospective studies need to be performed to confirm the various changes identified in hematological markers seen in ACS vs VOC. Disclosures No relevant conflicts of interest to declare.

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

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4934-4934 ◽  
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.

Sickle Cell Pain Crisis

2019 ◽  
pp. 271-280
Author(s):  
Vikram Bansal ◽  
Deva Sharma ◽  
Uma Shastri
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Pain Medicine ◽  
Cell Disease ◽  
Significant Challenge ◽  
Pain Crisis ◽  
Delivery Options ◽  
Underlying Mechanisms

Acute sickle cell pain crises pose a significant challenge to acute pain medicine physicians. Knowledge of underlying mechanisms, whether vaso-occlusive or not, can be useful in planning successful analgesic regimens. This chapter describes the pathophysiology and epidemiology of sickle cell disease. It also addresses how anesthesiologists should evaluate patients with sickle cell disease and acute pain. The chapter then presents numerous opioid delivery options that should be judiciously utilized to optimize patient function. Nonopioid adjuncts, such as acetaminophen or ketamine, should be aggressively pursued, and in some scenarios, perineural interventions may be appropriate. In any scenario, a comprehensive team-based approach is encouraged.

scholarly journals The effect of acute pain crisis on exhaled nitric oxide levels in children with sickle cell disease

2008 ◽  
Vol 50 (1) ◽  
pp. 111-113 ◽  
Author(s):  
Sachin S. Pawar ◽  
Julie A. Panepinto ◽  
David C. Brousseau
Keyword(s):  
Nitric Oxide ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Exhaled Nitric Oxide ◽  
Cell Disease ◽  
Pain Crisis

scholarly journals Acute Care in the Emergency Department Differs before and after Transition for Adolescents and Young Adults with Sickle Cell Disease

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3267-3267
Author(s):  
Nathan R. Stehouwer ◽  
Bohyung Park ◽  
Preston Edge ◽  
Hong Li ◽  
Connie M. Piccone ◽  
...  
Keyword(s):  
Emergency Department ◽  
Young Adults ◽  
Sickle Cell Disease ◽  
Acute Care ◽  
Sickle Cell ◽  
Acute Pain ◽  
Medication Administration ◽  
Wait Times ◽  
Cell Disease ◽  
Transition Age

Abstract Background Painful vaso-occlusive crises (VOC) are the most common cause of emergency department (ED) visits in patients with sickle cell disease (SCD) and are a major point of contact between patients and the health care system. Transition from pediatric to adult care has not been studied in the acute care setting. We examined whether management of VOC in adolescents and young adults differed between the pediatric and the adult ED. Methods A retrospective chart review was performed for all ED encounters in our hospital system for acute pain in patients with SCD, ages 13-23 years, between 2011-2013. Comprehensive medication administration data was collected for the ED visit as well as for the ensuing hospitalization, when applicable. Demographics and baseline medical data were collected. The equianalgesic dose, expressed as mg of intravenous (IV) morphine, and time to first analgesic administration were calculated. Equianalgesic dose and time to first analgesic were compared between the pediatric and adult ED using analysis of variance. In patients who were hospitalized, the total opioid dose received in the first 24 hours and the mean daily total opioid dosage during hospitalization were compared. Results were adjusted for correlation within multiple visits by individual patients, baseline hemoglobin, and presenting pain scores. Results: 193 visits by 45 subjects, half of whom were male (44% M pediatric, 56% M adults), and most of whom had either HbSS or HbSβ0 (59% pediatric, 83% adults), were examined. Time to medication administration in the pediatric ED was 72 minutes vs 131 minutes in the adult ED (p<0.05, Figure 1A). Opioid medications were administered in 96% of all visits. The adjusted equianalgesic dose of the first opioid medication was 5.4 mg and 10.6 mg in the pediatric and the adult ED, respectively (p<0.0001, Figure 1B). The first opioid administered was parenteral hydromorphone in 4% of pediatric visits and 72% of adult visits. Preliminary analysis suggests that transition-age patients also received higher medication dosages during ensuing admissions to the adult hospital when compared with the pediatric hospital. In a secondary analysis of 5 patients seen in both the pediatric and adult EDs during the study period, wait times were 56 and 158 minutes (p<0.05), and the equianalgesic doses of the first opioid administered were 4.7 mg and 7 mg, respectively (p<0.05). Conclusions These data suggest significant differences in acute pain management between pediatric and adult hospitals for transition-age patients with SCD. Possible reasons for observed differences include a larger patient volume in the adult ED, increasing disease severity with age, and the preference for use of higher potency hydromorphone among adult providers. Our data suggest that optimal transition management for adolescents with SCD must include strategies for transition in acute care management, focusing on limiting wait times and consistent dosing and titration of pain medications. Disclosures No relevant conflicts of interest to declare.

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