Sickle Cell Pain Crisis and Fever

ManagementPEM 102

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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

• Location, duration, and severity scale• Consider other etiologies: appy, cholecystitis, trauma• Analgesic use at home, timing of last dose• What works in the past

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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

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 23-

valent vaccine and no significant history of sepsis

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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)