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Acute Evaluation and Management of Children with Sickle Cell Disease – Pediatric – Emergency Department

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1



Acute Evaluation and Management of
Children with Sickle Cell Disease –
Pediatric – Emergency Dept.
Clinical Practice Guideline

Note: Active Table of Contents – Click to follow link

EXECUTIVE SUMMARY ........................................................................................................... 3
SCOPE ................................................................................................................................... 4
METHODOLOGY .................................................................................................................... 4
INTRODUCTION ..................................................................................................................... 4
RECOMMENDATIONS ............................................................................................................ 5
Management of a Vaso-Occlusive Crisis (VOC) ....................................................................... 5
Management of SCD Patients with a Fever (> 101.3°F or > 38.5°C) ....................................... 7
UW HEALTH IMPLEMENTATION ............................................................................................. 8
REFERENCES .......................................................................................................................... 9




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2


Contact for Content:
Name: Michael Kim, MD- Pediatric Emergency Medicine
Phone Number: (608) 263-1325
Email Address: mkkim@medicine.wisc.edu

Contact for Changes:
Name: Lindsey Spencer, MS- Center for Clinical Knowledge Management
Phone Number: (608) 890-6403
Email Address: lspencer2@uwhealth.org

Coordinating Team Members:
Joshua Ross, MD- Pediatric Emergency Medicine
Catherine O’Krafka, MD- Pediatric Emergency Medicine
Carol Diamond, MD- Pediatric Hematology
Michael Wilhelm, MD- Pediatric ICU
Ben Walker, MD- Anesthesiology (Director of Pediatric Pain Management)
Mary Jean Erschen-Cooke, RN- Pediatric Emergency Medicine
Josh Vanderloo, PharmD- Drug Policy Program

Review Individuals/Bodies:
David Yang, MD- Clinical Laboratories
Joseph McBride, MD- Pediatrics- Pediatric Infectious Disease Fellow
Sheryl Henderson, MD- Pediatrics- Pediatric Infectious Disease
Peggy Riley, RN- Nursing Admin- Children’s Hospital (Pediatric Pain CNS)

Committee Approvals/Dates:
Clinical Knowledge Management (CKM) Council (Last Periodic Review: 04/27/2017)

Release Date: April 2017 | Next Review Date: April 2020
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3


Executive Summary
Guideline Overview
Implementation of clinical algorithms and guidelines for pediatric Sickle Cell Disease have been
shown to promote more rapid stabilization and reduce the use of inappropriate therapies.1-3 The
following guideline outlines care which should be provided in the emergency department.

Key Practice Recommendations

Vaso-occlusive Crisis (VOC)
1. Perform a pain assessment (e.g., determine severity, location, characteristics, associated
symptoms) based on patient self-report and observation.4 (HHS Consensus- Adapted)
2. Rapidly assess recent analgesic use (opioid or non-opioid) (HHS Consensus- Adapted) and
initiate analgesic therapy.4,5 Use of intranasal fentanyl (1.5 mcg/kg; max dose 100
mcg/dose) is recommended until IV access is established.6 (UW Health High quality evidence,
strong recommendation)
3. Base analgesic selection on pain assessment, associated symptoms, outpatient analgesic
use, patient knowledge of effective agents and doses, and past experiences with side
effects.4 (HHS Consensus-Adapted) Providers should reference record of historical therapy
that the patient has received and/or the individualized pain plan documented in the medical
record.5
4. Perform regular assessment for side effects and/or excessive sedation.4 (UW Health Moderate
quality evidence, strong recommendation)
5. All patients who do not achieve adequate pain relief should be admitted to the hospital for
additional therapy.7

Fever
1. Immediately evaluate the patient with a history and physical, complete blood count with
differential, reticulocyte count, and blood culture.4,8 A urine culture may be obtained if a
urinary tract infection is suspected.4 (HHS Consensus- Panel Expertise)
2. Promptly administer ongoing empiric parenteral antibiotics that provider coverage against
Streptococcus pneumoniae and gram-negative enteric organisms.4 (HHS Consensus- Panel
Expertise)
3. Treat patients with SCD who have ACS with an intravenous cephalosporin, an oral
macrolide antibiotic, supplemental oxygen (to maintain oxygen saturation > 95%) and close
monitoring for bronchospasm, acute anemia, and hypoxemia.4,8 (HHS Low quality evidence,
strong recommendation)
4. Patients who appear ill should be hospitalized for close observation and intravenous
antibiotic therapy.4 (HHS Consensus- Panel Expertise) However, patients who lack certain high
risk factors (i.e., white blood cell count < 5,000/mm3 or > 30,000/mm3, fever > 40°C, or “ill-
appearing”) may be managed as an outpatient following administration of intravenous
empiric, antipneumococcal antibiotic (e.g., ceftriaxone).8 (UW Health Very low quality evidence,
weak/conditional recommendation)

Companion Documents
1. Evaluation and Initial Management of Children with Sickle Cell Disease and Pain Suspected
to be a Vaso-Occlusive Event Algorithm
2. Evaluation and Initial Management of Children with Sickle Cell Disease and Fever Algorithm
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4


Scope
Disease/Condition(s): Sickle Cell Disease (SCD)

Clinical Specialty: Pediatric Emergency Medicine, Emergency Medicine, Pediatric
Hematology/Oncology, Pediatric Intensive Care

Intended Users: Physicians, Advanced Practice Providers, Registered Nurses, Pharmacists

Objective(s): To outline evidence-based recommendations for the evaluation and management
of patients presenting to the emergency department with an acute Sickle Cell Disease
complication.

Target Population: Pediatric patients with Sickle Cell Disease.

Methodology
Methods Used to Collect/Select the Evidence:
Electronic database searches (e.g., PUBMED) were conducted by the guideline author(s) and
workgroup members to collect evidence for review. Expert opinion and clinical experience were
also considered during discussions of the evidence.

Methods Used to Formulate the Recommendations:
The workgroup members agreed to adopt recommendations developed by external
organizations and/or arrived at a consensus through discussion of the literature and expert
experience. All recommendations endorsed or developed by the guideline workgroup were
reviewed and approved by other stakeholders or committees (as appropriate).

Methods Used to Assess the Quality of the Evidence/Strength of the Recommendations:
Recommendations developed by external organizations maintained the evidence grade
assigned within the original source document and were adopted for use at UW Health.

Internally developed recommendations, or those adopted from external sources without an
assigned evidence grade, were evaluated by the guideline workgroup using an algorithm
adapted from the Grading of Recommendations Assessment, Development and Evaluation
(GRADE) methodology (see Figure 1 in Appendix A).

Rating Scheme for the Strength of the Evidence/Recommendations:
See Appendix A for the rating scheme(s) used within this document.

Recognition of Potential Health Care Disparities: Patients presenting to the emergency
department with Sickle Cell Disease experienced 25-50% longer wait times to see a physician
after arrival than other patients with long bone fracture or other conditions.9 Providers should
triage and treat this patient population quickly, especially as many often present with higher
level of pain than the general population.
Introduction
Vaso-occlusive crisis (VOC) and infection are the two most common reasons for hospitalization
in patients with Sickle Cell Disease. Implementation of clinical algorithms and guidelines for
pediatric Sickle Cell Disease have been shown to promote more rapid stabilization and reduce
Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
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5


the use of inappropriate therapies.1-3 The following guideline outlines the care which should be
provided in the emergency department for pediatric patients with Sickle Cell Disease.
Recommendations

Management of a Vaso-Occlusive Crisis (VOC)
1. The initial medical assessment should focus on detection of the following medical
complications requiring specific therapy: infection, dehydration, acute chest syndrome
(fever, tachypnea, chest pain, hypoxia, and/or rales), or severe anemia.5 In patients with an
oxygen saturation < 95% on room air, administer oxygen.4 (HHS Consensus- Panel Expertise)
2. Perform a pain screen and assessment (e.g., determine severity, location, quality, and
frequency ) based on patient self-report and observation.4 (HHS Consensus- Adapted)
a. If a pain rating scale is used, it should be chosen based upon patient age and
cognitive ability. (UW Health Moderate quality evidence, strong recommendation) The
Numeric Pain Scale10 is recommended in patients age 8 years or older, the Pain
Face Scale-Revised (FPS-R)11 in patients age 4-8 years, and the Neonatal/Infant
Pain Scale (NIPS)12 should be used in patients age less than 1 year. The Face Legs
Activity Cry and Consolability Scale- Revised (FLACC-R)13 may be used in patients
ages 1 year and above who are unable to verbally report pain due to cognitive or
developmental delays.
b. Patients are often able to recognize whether the pain is typical of their Sickle Cell
Disease.5 If the VOC pain is atypical per patient report, providers should investigate
other possible etiologies of pain.4,5 (HHS Consensus- Adapted)
3. Rapidly assess recent analgesic use (opioid or non-opioid) (HHS Consensus- Adapted) and
initiate analgesic therapy.4,5 Use of intranasal fentanyl (1.5 mcg/kg; max dose 100
mcg/dose) is recommended until IV access is established.6 (UW Health High quality evidence,
strong recommendation)
4. Once IV access is established, it is important to obtain a complete blood count with
differential, reticulocyte count and Type and Screen. (UW Health Very low quality evidence,
strong recommendation). A blood culture is suggested if a fever is present. (UW Health Very low
quality evidence, weak/conditional recommendation)
5. In euvolemic patients who are unable to drink fluids, provide intravenous hydration at no
more than the maintenance rate to avoid over-hydration.4,7 (HHS Consensus- Adapted)
6. There are no large controlled studies of analgesic regimens in Sickle Cell Disease and a
number of smaller studies have failed to define any optimal regimens.4,5 Base analgesic
selection on pain assessment, associated symptoms, outpatient analgesic use, patient
knowledge of effective agents and doses, and past experiences with side effects.4 (HHS
Consensus-Adapted) Providers should reference record of historical therapy that the patient
has received and/or the individualized pain plan documented in the medical record.5
7. In patients with mild to moderate pain who report relief with NSAIDs in the absence of
contraindications, continue treatment with NSAIDS.4 (HHS Low quality evidence, moderate
recommendation) A low dose opioid should also be administered in conjunction with the
NSAID.5,8 (UW Health Low quality evidence, weak/conditional recommendation)
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6


8. In patients with severe pain, rapidly initiate treatment with parenteral opioids.4 (HHS High
quality evidence, strong recommendation) NSAIDs should also be administered, if not taken in
the last 6 hours.5,8 (UW Health Low quality evidence, weak/conditional recommendation)
a. Reassess pain and re-administer opioids if necessary for continued severe pain
every 15-30 minutes until pain is under control.4 (HHS Consensus-Adapted)
b. Consider maintenance or dose escalation by 50% based upon response to analgesic
therapy until pain is controlled.4 (UW Health Very low quality evidence, weak/conditional
recommendation)
9. Perform regular assessment for side effects and/or excessive sedation.4 (UW Health Moderate
quality evidence, strong recommendation)
10. Use of non-pharmacotherapeutic interventions (e.g., local heat application, distraction) is
recommended as adjunctive therapy.4 (HHS Consensus- Adapted)
11. All patients who do not achieve adequate pain relief should be admitted to the hospital for
additional therapy.7 Opioid administration by patient-controlled analgesia (PCA) is preferred
over as requested (PRN) administration.4 (HHS Low quality evidence, moderate
recommendation) Providers may consider initiating a PCA in the emergency department while
awaiting inpatient admission. For dosing recommendations and consideration of side
effects, refer to the following resources:
a. Pain Care Fast Fact: IV Patient Controlled Analgesia in the Pediatric Patient
b. UW Health Continuous Opioid Therapy – Adult/Pediatric – Inpatient Clinical
Practice Guideline.
12. Prior to discharge from the emergency department, patients may be educated on techniques
for VOC prevention including avoiding triggers (e.g., infection, emotional stress, exposure to
cold, wind, and high altitude), use of hydroxyurea, and maintenance of intravascular volume
via oral fluid intake.8 (UW Health Very low quality evidence, weak/conditional recommendation)
13. Red blood cell transfusion is not recommended to resolve VOC.4,8 (UW Health Low quality
evidence, strong recommendation)


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7


Management of SCD Patients with a Fever (> 101.3°F or > 38.5°C)
1. Febrile illnesses in patients with Sickle Cell Disease are considered an emergency due to
the possibility of penicillin-resistant organisms and incomplete vaccination status, despite
the declining incidence of invasive pneumococcal infection as a result of prophylactic
penicillin and pneumococcal vaccination.4 Immediately evaluate the patient with a history
and physical, complete blood count with differential, reticulocyte count, and blood culture.4,8
A urine culture and urinalysis may be obtained if a urinary tract infection is suspected.4,7
(HHS Consensus- Panel Expertise)
a. Providers may confirm the patient’s immunization status, as this information can aid
in the development of differential diagnoses.7 (UW Health Low quality evidence,
weak/conditional recommendation)
b. Additional laboratory tests to consider for patients in pain with a fever include a Type
and screen, potassium, sodium, bicarbonate, and chloride. (UW Health Low quality
evidence, weak/conditional recommendation)
2. Patients who appear ill should be hospitalized for close observation and intravenous
antibiotic therapy.4 (HHS Consensus- Panel Expertise)
3. Promptly administer ongoing empiric parenteral antibiotics that provider coverage against
Streptococcus pneumoniae and gram-negative enteric organisms.4 (HHS Consensus- Panel
Expertise)
4. Acute chest syndrome (ACS) is a common and potentially lethal complication of Sickle Cell
Disease.8 The incidence is highest (25 episodes/100 patient years) in children between the
ages of 2 and 5 years.8 In patients whose febrile illness is accompanied by shortness of
breath, tachypnea, cough, and/or rales, it is recommend to obtain an immediate chest x-ray
to investigate for acute chest syndrome (ACS).4 (HHS Consensus- Panel Expertise) ACS is
defined by a new pulmonary infiltrate on chest x-ray in addition to one or more of the
following: fever, tachypnea, dyspnea, hypoxia, and chest pain.7,8
a. Treat patients with SCD who have ACS with an intravenous cephalosporin, an oral
macrolide antibiotic, supplemental oxygen (to maintain oxygen saturation > 95%)
and close monitoring for bronchospasm, acute anemia, and hypoxemia.4,8 (HHS Low
quality evidence, strong recommendation)
b. Hospitalize patients with ACS.4 (HHS Consensus- Panel Expertise) Patients may be
admitted to the PICU or general care floor under the Pediatric Hematology/Oncology
service based upon their supplemental oxygen needs.
5. Patients who lack certain high risk factors (i.e., white blood cell count < 5,000/mm3 or >
30,000/mm3, fever > 40°C, or “ill-appearing”) may be managed as an outpatient following
administration of intravenous empiric, antipneumococcal antibiotic (e.g., ceftriaxone).8 (UW
Health Very low quality evidence, weak/conditional recommendation) When deciding whether to
discharge a patient from the emergency department, it is also important to consider the
patient’s age, ability of the family to return promptly for recurrent fever or clinical
deterioration, and the availability of close follow-up.8
6. In febrile patients who have localized or multifocal bone tenderness, especially when
accompanied by erythema and swelling, include bacterial osteomyelitis in the differential
diagnosis and manage accordingly.4 (HHS Consensus- Panel Expertise)



Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
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8


UW Health Implementation
Potential Benefits:
ξ Pain relief
ξ Treatment of infection

Potential Harms:
ξ Oversedation

UW Health Policies & Procedures
1. UW Health Clinical Policy 3.5.5- Pain Management
2. UW Health Clinical Policy 6.1.2- Intravenous Patient Controlled Analgesia (PCA) for Adult
and Pediatric Patients

Patient Resources
1. HFFY #7267- A Guide for Families of Children with Sickle Cell Anemia at AFCH
2. HFFY #4273- Intravenous Patient Controlled Analgesia (IV PCA)
3. HFFY #7233- Intranasal Fentanyl Frequently Asked Questions
4. HFFY #6835- Non-Drug Pain Control for Kids
5. HFFY #7711- Using Pediatric Pain Scales: Neonatal Infant Pain Scale (NIPS)
6. HFFY #7712- Using Pediatric Pain Scales: Faces Legs Activity Cry Consolability Revised
Scale (FLACC-R)
7. HFFY #7713- Using Pediatric Pain Scales: Faces Pain Scale
8. HFFY #7714- Using Pediatric Pain Scales: Numeric Rating Scale
9. Kids Health- Sickle Cell Disease
10. Kids Health- Sickle Cell Crisis (Pain Crisis)

Guideline Metrics
1. Time to IN fentanyl administration in VOC patients
2. Time to administration of antibiotics in patients presenting with a fever
3. Adherence to recommended antibiotic regimens

Implementation Plan/Clinical Tools
1. Guideline will be posted on uConnect in a dedicated location for Clinical Practice Guidelines.
2. Release of the guideline will be advertised in the Physician/APP Briefing newsletter.
3. Content and hyperlinks within clinical tools, documents, or Health Link related to the
guideline recommendations (such as the following) will be reviewed for consistency and
modified as appropriate.

UW Health Clinical Practice Guidelines
Influenza and Pneumococcal Vaccination – Adult/Pediatric – Inpatient/Ambulatory
Continuous Opioid Therapy – Adult/Pediatric – Inpatient

Delegation Protocols
Immunization – Adult/Pediatric – Inpatient [60]
Emergency Department Immediate Orders – Adult/Pediatric [61]

Order Sets & Smart Sets
ED – Sickle Cell Disease with Pain/Fever – Pediatric [5752]
ED – Immediate Orders Delegation Protocol – RN/ED Tech – Pediatric [4274]

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9


Disclaimer
Clinical practice guidelines and clinical reference tools assist clinicians by providing a framework
for the evaluation and treatment of patients. This tool outlines the preferred approach for most
patients. It is not intended to replace a clinician’s judgment or to establish a protocol for all
patients. It is understood that some patients will not fit the clinical condition contemplated and
that a clinical reference tool or clinical practice guideline will rarely establish the only appropriate
approach to a problem.

References
1. Ender KL, Krajewski JA, Babineau J, et al. Use of a clinical pathway to improve the
acute management of vaso-occlusive crisis pain in pediatric sickle cell disease. Pediatr
Blood Cancer. 2014;61(4):693-696.
2. Kavanagh PL, Sprinz PG, Wolfgang TL, et al. Improving the Management of Vaso-
Occlusive Episodes in the Pediatric Emergency Department. Pediatrics.
2015;136(4):e1016-1025.
3. Ellison AM, Thurm C, Alessandrini E, et al. Variation in pediatric emergency department
care of sickle cell disease and fever. Acad Emerg Med. 2015;22(4):423-430.
4. National Heart L, and Blood Institute. Evidence-based Mangagement of Sickle Cell
Disease. In. Expert Panel Report: U.S. Department of Health and Human Services
National Institutes of Health; 2014:32-38.
5. Rees DC, Olujohungbe AD, Parker NE, et al. Guidelines for the management of the
acute painful crisis in sickle cell disease. Br J Haematol. 2003;120(5):744-752.
6. Fein DM, Avner JR, Scharbach K, Manwani D, Khine H. Intranasal fentanyl for initial
treatment of vaso-occlusive crisis in sickle cell disease. Pediatr Blood Cancer. 2016.
7. Simon E, Long B, Koyfman A. Emergency Medicine Management of Sickle Cell Disease
Complications: An Evidence-Based Update. J Emerg Med. 2016;51(4):370-381.
8. McCavit TL. Sickle cell disease. Pediatr Rev. 2012;33(5):195-204; quiz 205-196.
9. Haywood C, Tanabe P, Naik R, Beach MC, Lanzkron S. The impact of race and disease
on sickle cell patient wait times in the emergency department. Am J Emerg Med.
2013;31(4):651-656.
10. von Baeyer CL, Spagrud LJ, McCormick JC, Choo E, Neville K, Connelly MA. Three new
datasets supporting use of the Numerical Rating Scale (NRS-11) for children's self-
reports of pain intensity. Pain. 2009;143(3):223-227.
11. Hicks CL, von Baeyer CL, Spafford PA, van Korlaar I, Goodenough B. The Faces Pain
Scale-Revised: toward a common metric in pediatric pain measurement. Pain.
2001;93(2):173-183.
12. Suraseranivongse S, Kaosaard R, Intakong P, et al. A comparison of postoperative pain
scales in neonates. Br J Anaesth. 2006;97(4):540-544.
13. Voepel-Lewis T, Zanotti J, Dammeyer JA, Merkel S. Reliability and validity of the face,
legs, activity, cry, consolability behavioral tool in assessing acute pain in critically ill
patients. Am J Crit Care. 2010;19(1):55-61; quiz 62.




Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
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10


Appendix A. Evidence Grading Scheme(s)

Figure 1. GRADE Methodology adapted by UW Health


GRADE Ranking of Evidence
High We are confident that the effect in the study reflects the actual effect.
Moderate We are quite confident that the effect in the study is close to the true effect, but it
is also possible it is substantially different.
Low The true effect may differ significantly from the estimate.
Very Low The true effect is likely to be substantially different from the estimated effect.

GRADE Ratings for Recommendations For or Against Practice
Strong The net benefit of the treatment is clear, patient values and circumstances
are unlikely to affect the decision.
Weak/conditional
Recommendation may be conditional upon patient values and
preferences, the resources available, or the setting in which the
intervention will be implemented.



Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
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04/2017CCKM@uwhealth.org

Patient Presentation
ξ Perform cardiorespiratory monitoring, pulse oximetry, and pain screen/assessment
ξ Administer O
2
if O
2
saturation < 95%
ξ Administer IN fentanyl 1.5 mcg/kg (max 100 mcg/dose) ASAP (until IV access established)
ξ Obtain initial labs: CBC with differential, reticulocyte count, Type and Screen
Evaluation and Initial Management of Children with Sickle Cell Disease and Pain Suspected
to be a Vaso-Occlusive Event – Pediatric – Emergency Dept. Algorithm
Patient Population:
HbSS, SC, Sβ
0
-thalassemia
ξ Give normal saline bolus 20 mL/kg (max 1 L) as indicated
ξ Consider 2 view chest x-ray if tachypneic, chest pain, shortness of breath and/or rales
ξ If fever, obtain blood culture and refer to Evaluation and Initial Management of Children with Sickle Cell
Disease and Fever – Pediatric – Emergency Dept. Algorithm
Is pain well
controlled?
Administer PO oxycodone + PO ibuprofen
(if no NSAID in last 6 hours)
*REASSESS IN 60 MINUTES*
Yes
Is pain well
controlled?
Contact Peds Hem/Onc to discuss
possible discharge and follow-up
Yes
Meet
discharge
criteria?
DISCHARGE
Provide prescription for 3 days of scheduled
oxycodone, ibuprofen, and miralax.
Yes
No
Administer IV opioid + IV ketorolac
(if no NSAID in the last 6 hours)
*REASSESS IN 20 MINUTES*
No
No
Is pain
well controlled?
Yes
Discharge Considerations
ξ Pain well-controlled
ξ Tolerating PO liquids
ξ Absence of other acute SCD complications
IV opioid: 50% loading dose
*REASSESS IN 20 MINUTES*
No
Is pain
well controlled?
IV opioid: 50% loading dose
No
ADMIT TO PEDS/HEM ONC
ξ Contact Peds Hem/Onc with lab results and patient status
ξ Consider initiation of PCA or scheduled long-acting oral opioid.
Yes
References:
1. National Heart L, and Blood Institute. Evidence-based Management of Sickle Cell Disease. Expert Panel Report: U.S. Department of
Health and Human Services National Institutes of Health; 2014:32-38.
2. Rees DC, Olujohungbe AD, Parker NE, et al. Guidelines for the management of the acute painful crisis in sickle cell disease. Br J
Haematol. 2003;120(5):744-752.
3. Fein DM, Avner JR, Scharbach K, Manwani D, Khine H. Intranasal fentanyl for initial treatment of vaso-occlusive crisis in sickle cell
disease. Pediatr Blood Cancer. 2016.
ADMIT TO
PEDS HEM/ONC
Reference historical therapy received and/or pain plan
documented in the patient’s medical record (Problem List)
Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
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Patient Presentation
ξ Perform cardiorespiratory monitoring, pulse oximetry, and pain screen/assessment
ξ Administer oxygen if O
2
saturation < 95%
ξ Initial labs: CBC with differential, reticulocyte count, blood culture
ξ Consider immediate chest x-ray if tachypneic, chest pain, shortness of breath and/or rales
ξ Consider additional labs such as: total bilirubin, creatinine, urinalysis and urine culture.
If in pain, consider Type and Screen, potassium, sodium, bicarbonate, and chloride and
refer to Evaluation and Initial Management of Children with Sickle Cell Disease and Pain Suspected
to be a Vaso-Occlusive Event – Pediatric – Emergency Dept. Algorithm
ξ Check immunization status
ξ Start maintenance fluids (bolus if clinically dehydrated)
Evaluation and Initial Management of Children with Sickle Cell Disease and Fever – Pediatric –
Emergency Dept. Algorithm
Patient Population:
Age > 2 months and < 18 years
HbSS, SC, Sβ
0
-thalassemia
Fever > 38.5°C in the past 48 hours
Is the pt
ill appearing or
any historical red
flags*?
Administer IV ceftriaxone
**Acute Chest Syndrome:
New infiltrate on chest x-ray AND one or more
of the following NEW symptoms :
ξ Fever
ξ Cough
ξ Sputum production
ξ Dyspnea
ξ Hypoxia
Fluids in Acute Chest Syndrome:
Avoid excessive fluid administration,
resuscitate as necessary
Contact CCKM for revisions.
Reference the UW Health Acute Evaluation and Management of
Children with Sickle Cell Disease – Pediatric – ED Guideline
Acute chest
syndrome?**
Yes
ADMIT TO PICU or
PEDS HEM/ONC
Administer IV ceftriaxone
+ oral azithromycin
ADMIT TO PICU or PEDS HEM/ONC
(based on supplemental O
2
need)
***Low Risk Features :
ξ Normal vital signs
ξ Tolerating PO
ξ Hgb > 6 mg/dL
ξ Reticulocyte count > 5% (unless Hgb > 10 mg/dL)
ξ No significant drop in Hgb from baseline
ξ WBC 5,000-30,000/mm
3
ξ No history of bacteremia or sepsis
ξ No splenic sequestration within the past 3 months
ξ Not multiple visits for same febrile illness
ξ Compliant with penicillin
ξ Fully immunized
ξ Able to follow-up next day with Peds Hem/Onc via appointment or telephone contact
Age > 18
months?
ADMIT TO PEDS HEM/ONC
No
Low risk?***
Yes
Observe for 1 hour
post antibiotics
Yes
Contact PEDS HEM/ONC
to discuss discharge, follow-up, and
additional antibiotic therapy
No
*Historical Red Flags
ξ History of Acute Chest
Syndrome
ξ History of stroke
ξ History of splenic
sequestration
ξ History of sepsis or
bacteremia
ξ Reactive airway disease
ξ Requires chronic transfusions
ξ History of PICU admission for
SCD related complications
Administer IV
ceftriaxone
Consult with
PEDS HEM/ONC or PICU
for admission
Perform further
evaluation and
management as clinically
appropriate
(e.g., fluid resuscitation,
vancomycin, etc.)
Yes
INTERVENE IMMEDIATELY-
PATIENT MAY BE CRITICALLY ILL
No
No
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