Sickle Cell Pathway V2.1: Suspected Acute Chest Syndrome Table of Contents

Sickle Cell Pathway v2.1: Suspected Acute Chest Syndrome Table of Contents

Inclusion Criteria · New, non-atelactatic, pulmonary infiltrate in patient with a Stop and sickle cell hemoglobinopathy Review Exclusion Criteria · O2 need related to opiate use with no infiltrate on CXR

Sickle Cell Care

Suspected Acute Chest Syndrome

Appendix

Summary of Version Changes Approval & Citation Evidence Ratings

For questions concerning this pathway, contact: Last Updated: October 2020 [email protected] Next Expected Review: October 2025 If you are a patient with questions contact your medical provider, Medical Disclaimer © 2020 Seattle Children’s Hospital, all rights reserved Sickle Cell Pathway v2.1: Suspected Acute Chest Syndrome

Evaluations for Acute Chest · Physical Exam · CXR · O2 sats · Consider viral studies · Blood & Urine Cx · CBC, diff, retic and type and cross

Admit if not already admitted

Standard Conservative Therapy · Aggressive pain management · Incentive spirometry q 1 hrs while awake, 10 breaths every hour from 0800 to 2200 and with vital signs while awake from 2200 to 0800, as well as with every "as needed" IV bolus of pain medication, and prior to chest x-rays · Oxygen to maintain O2 saturation >93% · Ceftriaxone q 24 hours IV (Ciprofloxacin and Clindamycin if allergic to Ceftriaxone) ! · Azithromycin or other macrolide antibiotic to cover atypical organisms · Bronchodilator trial Have a low · Patients with a consolidation on CXR should be evaluated by respiratory therapy for CPT threshold to · CPAP or BiPAP for patients with poor respiratory effort, reduced ventilation or continued evaluate for positive decline despite other interventions pressure ventilation

Transfuse Immediately Targeting HCT of 30-33% Assess for Immediate Transfusion Discuss transfusion plan with Heme Onc · Critically ill fellow or sickle cell team · Multi-lobe Involvement IF Hct <27% · PaO2 <70 or 10mm below baseline · Consider direct transfusion · O2 by facemask requirement with HCT <20% Yes IF Hct ≥27% · Consider exchange transfusion Attributes No No · HbS neg, leukoreduced, antigen matched

Assess for Progressive Pulmonary Decline See Transfusion Guidelines for details on · Persistent increase in O2 need how much blood to transfuse (for SCH Only) (not a transient need) of >1L/shift or 2L/ 24 hours · Steady worsening of physical exam not attributable Discharge Criteria to fluid overload · Improved respiratory symptoms, off O2 and O2 sats at · Increasingly toxic appearance baseline. · Substantially worsened CXR, acknowledging CXR · Afebrile or defervescing per fever guidelines. changes often lag behind clinical status · Negative cultures for 24 hrs if applicable. · Adequate oral intake, and able to take oral antibiotics. · Adequate pain relief (if needed) with oral analgesics and No written taper of opiates given to patient and reviewed. · Discharge teaching on home use of incentive spirometry (if also taking opiates) completed. Anemia Evaluation Yes · Stable Hct and reticulocyte count. · Is HCT <18 or Hgb 1-2g/dl below baseline or more · Follow-up plans coordinated with sickle cell service · See Transfusion Guidelines for detailed transfusion · CXR 8-12 weeks post therapy criteria for anemia and other sickle cell transfusion · Consider follow up pulmonary function tests indications (for SCH Only)

· Version 1.1 (4/15/2013): Go live. · Version 1.2 (3/20/2015): Algorithm wording correction to align with powerplan. · Version 1.3 (2/8/2019): Contact e-mail updated. · Version 2.0 (10/3/2020): Updated the algorithm to a new template and stakeholders reviewed the content for accuracy. · Version 2.1 (10/30/2020): Fixed some links. Corrected citation.

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Approved by the CSW Sickle Cell Pathway team for October 3, 2020 go-live

CSW Sickle Cell Pathway Team:

HemOnc, OBCC, Owner M.A. Bender, MD-PhD ED, Clinical Nurse Specialist Brian Burns, DNP, ARNP-CS, CPEN Acute Care, Clinical Quality Leader Caitlin Siegfried, BSN, RN, CPHON HemOnc, stakeholder Jennifer Wilkes, MD, MSCE HemOnc, inpatient stakeholder Alayne Chapple, MSN, FNP-BC ED, Clinical Director Russ Migita, MD OBCC, stakeholder Alix Dassler, ARNP, FNP-BC Infection Disease, stakeholder Surabhi Vora, MD, MPH HemOnc, Medical Director – CBDC Amy Tellinghuisen, MD

Clinical Effectiveness Team:

Consultant Coral Ringer, MN,RN,CPN Project Manager Asa Herrman Data Analyst James Johnson EHR Informatician Alayne Chapple, MSN, FNP-BC EHR Analyst Alyssa Dunham Librarian Peggy Cruse, MLIS Program Coordinator Kristyn Simmons

Clinical Effectiveness Leadership:

Medical Director Darren Migita, MD Operations Director Jaleh Shafii, MS, RN, CPHQ

Retrieval Website: https://www.seattlechildrens.org/pdf/sickle-cell-acute-chest-algorithm.pdf

Please cite as: Seattle Children’s Hospital, Bender MA, Burns B, Chapple A, Dassler A, Herrman A, Migita R, Ringer C, Siegfried C, Tellinghuisen A, Vora S, Wilkes J, Migita D, 2020 October. Sickle Cell Pathway. Available from: https://www.seattlechildrens.org/pdf/sickle-cell-acute-chest-algorithm.pdf

To Table of Contents Evidence Ratings

This pathway was developed through local consensus based on published evidence and expert opinion as part of Clinical Standard Work at Seattle Children’s. Pathway teams include representatives from Medical, Subspecialty, and/or Surgical Services, Nursing, Pharmacy, Clinical Effectiveness, and other services as appropriate.

When possible, we used the GRADE method of rating evidence quality. Evidence is first assessed as to whether it is from randomized trial or cohort studies. The rating is then adjusted in the following manner (from: Guyatt G et al. J Clin Epidemiol. 2011;4:383-94, Hultcrantz M et al. J Clin Epidemiol. 2017;87:4-13.):

Quality ratings are downgraded if studies: · Have serious limitations · Have inconsistent results · If evidence does not directly address clinical questions · If estimates are imprecise OR · If it is felt that there is substantial publication bias

Quality ratings are upgraded if it is felt that: · The effect size is large · If studies are designed in a way that confounding would likely underreport the magnitude of the effect OR · If a dose-response gradient is evident

Certainty of Evidence High: The authors have a lot of confidence that the true effect is similar to the estimated effect Moderate: The authors believe that the true effect is probably close to the estimated effect Low: The true effect might be markedly different from the estimated effect Very low: The true effect is probably markedly different from the estimated effect Guideline: Recommendation is from a published guideline that used methodology deemed acceptable by the team Expert Opinion: Based on available evidence that does not meet GRADE criteria (for example, case-control studies)

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Literature Search Methods

Studies were identified by searching electronic databases using search strategies developed and executed by a medical librarian, Susan Klawansky. Searches were performed in June 2011 in the following databases: on the Ovid platform – Medline (1980 to current), Medline in Process, Cochrane Database of Systematic Reviews (2005 to current), Cochrane Central Register of Controlled Trials (all); elsewhere – Clinical Evidence, DynaMed, UpToDate, AHRQ, National Guideline Clearinghouse, American Academy of Pediatrics Policies, TRIP and Cincinnati Children’s Evidence-Based Guidelines. Retrieval was limited to humans of any age and English language. In Medline, appropriate Medical Subject Headings (MeSH) were used, along with text words, and the search strategy was adapted for other databases using their controlled vocabularies, where available, along with text words. Concepts searched were sickle cell anemia, thalassemia AND acute chest syndrome AND blood transfusion. All retrieval was further limited to certain evidence categories, such as relevant publication types, Clinical Queries, index terms for study types and other similar limits.

Susan Klawansky, MLS, AHIP June 29, 2012

Identification Records identified through Additional records identified database searching (n=108) through other sources (n=4)

Screening Records after duplicates removed (n=112)

Records screened (n=112) Records excluded (n=61)

Eligibility Records assessed for eligibility (n=51) Articles excluded (n=37) Quality threshold (n=16) Did not answer clinical question (n=21)

Included Studies included in pathway (n=14)

Flow diagram adapted from Moher D et al. BMJ 2009;339:bmj.b2535

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Included Studies Alhashimi D; Fedorowicz Z; Alhashimi F; Dastgiri S; Blood Transfusions for Treating Acute Chest Syndrome in People with Sickle Cell Disease. Cochrane Database Syst Rev. 2010 Jan 20;(1):CD007843 Emre U; Miller ST; Gutierez M; Steiner P; Rao SP; Rao M et al. Effect of Transfusion in Acute Chest Syndrome of Sickle Cell Disease. Journal of Pediatrics 1995; 127(6): 901-904. Haynes J; Allison RC; Pulmonary Edema: Complication in the Management of Sickle Cell Pain Crisis. Am J Med 1986; 80:833. Lottenberg R; Hassell KL An Evidence-Based Approach to the Treatment of Adults with Sickle Cell Disease. Hematology Am Soc Hematol Educ Program. 2005:58-65. Thame JR; Hambleton IR; Serjeant GR; RBC transfusion in sickle cell anemia (HbSS): Experience from the Jamaican Cohort Study. Transfusion. 2001; 41(5): 596-601. Mallouh AA; Asha M; Beneficial Effect of Blood Transfusion in Children With Sickle Cell Chest Syndrome. Arch Pediatr Adolesc Med. 1988; 142(2): 178-182. Meremikwu MM; Okomo U; Sickle Cell Disease. Clin Evid (Online). 2011; 2402. Miller ST. How I treat acute chest syndrome in children with sickle cell disease. Blood. 2011 May 19;117(20):5297-305. Epub 2011 Mar 15. Review. PubMed PMID: 21406723. Sprinkle RH; Cole T; Smith S; Buchanan GR; Acute Chest Syndrome in Children with Sickle Cell Disease. Am J Pediatr Hematol Oncol. 1986; 8:105. Saborio P; Scheinman JI; Sickle Cell Nephropathy. J Am Soc Nephrol 1999;10:187-192. Talano JM; Hillery CA; Gottschall JL; et al. Delayed Hemolytic Transfusion Reaction/ Hyperhemolysis Syndrome in Children with Sickle Cell Disease. Pediatrics 2003; 111: 661-665. U.S. Department of Health and Human Services, National Institute of Health (2002). The management of sickle cell disease. NIH publication no. 02-0117. www.nhlbi.nih.gov/ health/prof/ blood/sickle/sc_mngt.pdf Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000; 342:1855-1865. Wun T; Hassell K; Best Practices for Transfusion for Patients with Sickle Cell Disease. Hematology Reports. 2009; 1(2):

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Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required.

The authors have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication.

However, in view of the possibility of human error or changes in medical sciences, neither the authors nor Seattle Children’s Healthcare System nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they are not responsible for any errors or omissions or for the results obtained from the use of such information.

Readers should confirm the information contained herein with other sources and are encouraged to consult with their health care provider before making any health care decision.

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