Sickle Cell Pain
Crisis and Fever
Management
PEM 102
Sickle Cell
 All varieties at risk for pain crisis including:
• Homozygous Hgb SS
• Heterozygous Hgb SC
• Sickle Beta thalasemia, variable complications
 60% will have vaso-occlusive crisis by 2yo
• Dactylitis and acute pain are most common presenting
symptom
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Acute Painful Crisis
 Precipitated by illness, dehydration, stress, menses
 Most common locations include back, chest,
abdomen, and extremities
• Duration typically 2-7 days
 Mechanism under debate
• Direct occlusion of capillaries and recruitment of
inflammatory mediators IL-6, IL-8, substance P
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ECH Pain Management
 Rapid triage on arrival – red triage
 History
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Location, duration, and severity scale
Consider other etiologies: appy, cholecystitis, trauma
Analgesic use at home, timing of last dose
What works in the past
Physical Exam & Labs
 See fever pathway if > 38.3
 Oxygen for hypoxia less than baseline
 Lungs, spleen, bones/joints, GU, neuro exam
 Labs:
• CBCD, Retic (compare to baseline), +/- T&S
• UA if abd/flank pain and symptoms
• CXR if fever, chest pain, tachypnea
• Consider RUQ U/S, LFT’s, pelvic exam if needed
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Treatment
 Based on severity of pain, recent home analgesics,
and prior experience of patient
 Often receive Lortab at triage
 Consider NSAIDs and opiates
• Toradol 0.5mg/kg (max 30mg) IV q6 OR Motrin
• Morphine 0.1-0.15mg/kg q30min
• Dilaudid 0.015-0.02mg/kg
• Nalbuphine (Nubain) 0.2-0.3mg/kg q3h
• Avoid Demerol (inc risk sz with repeated doses)
 IVF: 10cc/kg NS over 1hr then D5 ¼ NS at MIVF
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Management and Admission?
 Look at previous visits to see what works
 If frequent visits or concerns about drug-seeking
noted on previous visits call consultant early to
determine plan
 After treatment, discuss options with family
• If resolution with opioid x 1 consider oral
analgesic and observation in the ER for rebound
pain
• If more than one or two doses required consider
admission
• Discuss case and plan with on-call consultant
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Sickle Cell and Fever
 Splenic dysfunction from Hgb S leads to impaired
splenic filtration → auto-infarction by 2-4yo
 Impaired IgG and IgM responses and complement
dysfunction also play a role
 Increased susceptibility to encapsulated
microorganisms
• especially Streptococcus pneumo and H.
influenzae
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 PCN at 125mg BID to 3yo then 250mg Bid at 5yo
• Pneumococcal sepsis can occur on PCN, usually
associated with sub-optimal compliance
• Safe to stop PCN ppx at 5yo if 2 doses of 23valent vaccine and no significant history of sepsis
Sickle Cell and Fever (>38.3)
 Rapid triage on arrival
 Compliance with PCN, history of SBI, ACS
 Examine for evidence of systemic of localized
infection
 Labs based on protocol:
• CBCD, Retic, Blood cx, consider CRP
• Consider UA and Ucx without other source
• CSF based on clinical examination
• Type & Cross for pallor, AMS, enlarged spleen –
leukocyte depleted sickle negative pRBC (match
for minor Ag if possible)
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Management
 Prompt Ceftriaxone 50-75mg/kg (2g max) IV
 Give IM if access delayed
 If known allergy consider meropenum
 Severe illness with AMS, hypotension add
vancomycin
 IV Abx immediately after blood cx and before
procedures
 Presence of foci (ASOM, Strep throat) does not
alter urgency for Abx
 Add atypical coverage if CXR + and > 2yo
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Management
 NS bolus 10-20cc/kg for dehydration, hypotension,
MIVF for well hydrated pts
 CXR if:
• cough, resp symptoms, tachypnea, chest or abd
pain, abnl exam, Pox less than baseline
 Sickle cell consult:
• Likely admit if < 1yo, previous SBI, Temp >40,
WBC >30 or < 5, plt < 100, CXR +, co-existing
acute pain, or social concerns
• Outpt management: minimum of phone f/u to
repeat exam and Ceftriaxone at 24hrs (with or
without repeat CBC)
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