Sickle Cell Disease with Fever Clinical Pathway — Emergency Department

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Goals and Metrics

Provider Resources

All children with sickle cell disease and a central venous catheter/apheresis port should be treated according to the Non-oncology with CVC and Fever Pathway.

Sickle Cell Disease Patient with Fever

Triage

Review criteria for:
ED RN Standing Order for SCD, fever

As Clinically Indicated
- Sepsis Huddle
- Sepsis Pathway, ED, Inpatient and PICU

FLOC/RN team assessment
- RN: IV access, CBC, blood culture
Complete History, Physical Exam
Diagnostic Studies
- Consider COVID and/or influenza testing

Review:
- Low-risk Criteria,
- Antibiotics, IVF, and Disposition

As Clinically Indicated:
- Sepsis Huddle,
- Sepsis Pathway, ED, Inpatient and PICU

Clinically Suspected
Acute Chest Syndrome

2-11 mos or
≥ 12 mos and All Low-risk Criteria Not Met

12 mos-16 yrs
Meets All Low-risk Criteria

≥ 17 yrs
Meets All Low-risk Criteria

Ampicillin
Azithromycin
Send Type and Cross

Ampicillin

Ceftriaxone
Observe 2 hrs

Levofloxacin

Admit

Discharge

Review with Hematology Consultant
If all Low-risk Criteria are not met, admission is required

If all Low-risk Criteria are met, eligible for discharge after Hematology consultation

Low-risk Criteria
Clinical	Labs, X-ray Findings	PMH, Social History
≥ 12 mos Well-appearing Good VS Tolerating PO well No concern for complications Sequestration Acute chest syndrome VOC requiring IV analgesia No new hypoxia O2 sat ≥ 92% if baseline not known or RA sat < 3% below baseline No central venous catheter	Hgb > 5g/dL Reticulocyte count > 1% (Unless Hgb > 10g/dL) No significant drop Hgb (> 2g/dL) WBC > 5 K and < 30 K Chest X-ray (if indicated) without infiltrate UA (if indicated) normal	No history of: Bacteremia Sepsis History of splenic sequestration in past 4 wks Recent antibiotic treatment Multiple visits for same febrile illness No history of: Non-compliance with penicillin prophylaxis Missing, delayed immunizations Low likelihood of follow-up: No phone No transportation Currently in shelter Missed appointments

Posted: January 2010
Revised: July 2022
Authors: A. Ellison, MD; C. Witmer, MD; J. Lavelle, MD; C. Jacobstein, MD; C. Norris, MD; R. Cecil, RN, MSN; T. McKnight, CRNP; K. Smith Whitley, MD

Evidence

Evidence-Based Management of Sickle Cell Disease, Expert Panel Report, 2014

Educational Media

PEM Podcast Episode 9: Sickle Cell Disease

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

Accordingly, these clinical pathways are not intended to constitute medical advice or treatment, or to create a doctor-patient relationship between/among The Children’s Hospital of Philadelphia (“CHOP”), its physicians and the individual patients in question. CHOP does not represent or warrant that the clinical pathways are in every respect accurate or complete, or that one or more of them apply to a particular patient or medical condition. CHOP is not responsible for any errors or omissions in the clinical pathways, or for any outcomes a patient might experience where a clinician consulted one or more such pathways in connection with providing care for that patient.

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Emergency Department Clinical Pathway for Evaluation/Treatment
of Children with Sickle Cell Disease with Fever

Evidence

Educational Media

Emergency Department Clinical Pathway for Evaluation/Treatmentof Children with Sickle Cell Disease with Fever

Evidence

Educational Media

Emergency Department Clinical Pathway for Evaluation/Treatment
of Children with Sickle Cell Disease with Fever