121 - Acute Chest Syndrome (ACS) in Children and Adolescents Hospitalized for Sickle Cell Disease (SCD) Vaso-occlusive Pain Episodes (VOE)

Publication Number: 2116.121

Dunia Hatabah, Emory University School of Medicine, Atlanta, GA, United States; Fahd A. Ahmad, Washington University in St. Louis School of Medicine, St. Louis, MO, United States; Gladstone Airewele, Baylor College of Medicine, Houston, TX, United States; Bolanle T. Akinsola, Emory University School of Medicine, Atlanta, GA, United States; Nitya Bakshi, Yale School of Medicine, New Haven, CT, United States; David Brousseau, Nemours Children's Hospital, Wilmington, DE, United States; Kathleen M. Brown, Children's National, washington, DC, United States; Andrew D.. Campbell, Childrens National Hospital, Washington, DC, United States; Charlie Casper, University of Utah School of Medicine, Salt Lake City, UT, United States; Todd Chang, Children's Hospital Los Angeles, Los Angeles, CA, United States; Corrie E. Chumpitazi, Duke University School of Medicine, Durham, NC, United States; Daniel Cohen, Nationwide Children's Hospital, Bexley, OH, United States; Keli Coleman, Medical College of Wisconsin, Milwaukee, WI, United States; Andrea Cruz, Baylor College of Medicine, Houston, TX, United States; Angela M. Ellison, Childrens Hospital of Philadelphia, Philadelphia, PA, United States; Melanie Fields, Washington University in St. Louis School of Medicine, Saint Louis, MO, United States; Hailey Jensen, University of Utah School of Medicine, Salt Lake City, UT, United States; Elizabeth S. Klings, Boston Univ Sch of Med, Boston, MA, United States; Rawan Korman, Medical College of Georgia, Augusta, GA, United States; Sara Leibovich, UCSF Benioff Children's Hospital Oakland, Oakland, CA, United States; Derek Meyer, University of Utah School of Medicine, Salt Lake City, UT, United States; Seth Otto, University of Utah School of Medicine, Salt Lake City, UT, United States; Jonathan Race, The University of Utah School of Medicine - Salt Lake City, UT, Salt Lake City, UT, United States; Chris A. Rees, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, United States; Allison Remiker, Medical College of Wisconsin, Milwaukee, WI, United States; Nidhi Singh, Baylor College of Medicine, Houston, TX, United States; Alexis Thompson, Childrens Hospital of Philadelphia, Philadelphia, PA, United States; Elliott Vichinsky, University of California San Francisco, Oakland, CA, United States; Anthony Villella, Nationwide Childrens Hospital, Columbus, OH, United States; Bridget Wynn, Emory University School of Medicine, Atlanta, GA, United States; Noor Alzraikat, Emory University School of Medicine, Atlanta, GA, United States; Carlton Dampier, Emory University School of Medicine, Atlanta, GA, United States; Claudia R.. Morris, Emory University School of Medicine, Atlanta, GA, United States

Background: ACS occurs in up to 20% of hospitalized patients with SCD-VOE, often prolonging and complicating hospital stay. Despite its clinical significance, variations exist in the management and outcomes of ACS across institutions.

Objective: Determine prevalence of ACS in children and young adults hospitalized for SCD-VOE and describe emergency department (ED) presentation, clinical course and practice variation across institutions.

Design/Methods: Cross-sectional analysis of data collected from a multicenter, double-blinded, randomized, placebo-controlled phase-3 trial of intravenous arginine therapy in hospitalized patients with SCD-VOE aged 3-21 (NCT04839354) at 10 pediatric EDs across the US. ACS defined by radiology-interpreted chest radiograph (CXR) positive for new infiltrate and clinical team diagnosis

Results: ACS occurred in 20% (n = 55) of the 271 patients enrolled. ACS patients were significantly younger and predominantly male with HbSS/SBeta0 Thal vs no ACS (Table1). In the ED, ACS patients had more O2 desaturations at < 94%, higher frequency of cough, wheeze, and chest pain vs no ACS (Table1). No significant difference in fever across groups. 83% of patients with ACS presented with a normal respiratory exam in the ED. Clinical outcomes were worse in patients with ACS vs no ACS (Table 1). Of the 55 ACS patients, 19 were diagnosed in the ED with a positive CXR, and 36 were diagnosed during their hospitalization with a more severe ACS clinical course (Table2). The mean time to diagnosis of inpatient ACS was 2.4±1.6 days. LOS was significantly longer in moderate/severe vs mild ACS. Variations across sites are described in Table 3. Sex, age, ED O2 saturation < 94%, hemoglobin, and chest pain were independently associated with ACS in the multivariate model with an AUC=0.79.

Conclusion(s): ACS remains common in patients with SCD-VOE (20%), with 14% of ACS diagnosed during hospitalization. ACS may have been missed in patients who did not get a CXR, and radiographic changes often lag behind symptoms. Lack of ACS-specific clinical signs and symptoms, including a normal ED respiratory exam in most patients, may delay diagnosis. Patients with inpatient-diagnosed ACS had a more severe hospital course vs ED-diagnosed ACS. Considerable practice variation exists in ACS management across institutions, with variable use of antibiotics and transfusion protocols. Further research on standardizing ACS treatment may be warranted. ED O2 desaturation was associated with a >3.9-fold risk of ACS, not included in prior ACS risk models.

Table 1: Patient demographics and clinical characteristics


Table 2: Patient demographics and clinical characteristics by chest radiograph (CXR) result


Table 3: Clinical characteristics by Hospital - Among patients who were ever positive for ACS