1. Sickle Cell Pain Crisis and Fever Management
PEM 102

2. 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

3. 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

4. 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

5. 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

6. 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 mg/kg q30min
Dilaudid mg/kg
Nalbuphine (Nubain) mg/kg q3h
Avoid Demerol (inc risk sz with repeated doses)
IVF: 10cc/kg NS over 1hr then D5 ¼ NS at MIVF

7. 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

8. 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

9. 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)

10. 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

11. 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)