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Sickle Cell Disease: Pain & Fever. John Cheng, MD PEM Fellows’ Conference July 19, 2006. Sickle Cell Disease. Hemoglobin S Glu  Val at 6 position of β hemoglobin Various types: SS SC S β -thalessemia Others. Sickle Cell Issues. Vaso-Occlusive Crisis

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sickle cell disease pain fever

Sickle Cell Disease: Pain & Fever

John Cheng, MD

PEM Fellows’ Conference

July 19, 2006

sickle cell disease
Sickle Cell Disease
  • Hemoglobin S
    • Glu  Val at 6 position of β hemoglobin
  • Various types:
    • SS
    • SC
    • Sβ-thalessemia
    • Others
sickle cell issues
Sickle Cell Issues
  • Vaso-Occlusive Crisis
    • Sickling and subsequent ischemia
  • Immunocompromise
    • Splenic infarction
    • Encapsulated organisms: H. influenzae, S. pneumonia
    • Salmonella
vaso occlusive crisis voc
Vaso-Occlusive Crisis (VOC)
  • Usual type of pain?
  • Concerns:
    • Abdominal pain: splenic sequestration, gallstones
    • Hip pain: avascular necrosis
    • Headache: stroke
    • Chest pain: acute chest syndrome
    • Eye pain: optic artery ischemia
    • Groin pain (male): priapism
    • Extremity pain: dactylitis, osteomyelitis
    • Other pain: possible abscess
voc labs
VOC--Labs
  • CBC with diff
  • Reticulocyte count
  • Blood cultures if h/o fever
  • Consider electrolytes
    • BMP if dehydrated
    • LFTs if RUQ or epigastric abd pain
  • Consider U/A and Ucx if abd/flank pain
  • Consider Type and Screen
voc diagnostics
VOC--Diagnostics
  • CXR if respiratory symptoms
  • Ultrasound--abdominal
  • CT scan--head
voc treatment
VOC--Treatment
  • Oxygen
    • Keep SaO2 ≥ 92%
    • May be hypoxic at baseline
  • Hypotonic fluids (D5 1/4NS)
    • Reverse sickling
    • Dehydration: 10 cc/kg NS bolus vs 1.5 maintenance
    • BEWARE fluid overload
  • Blood transfusion
    • If neeed, try to get leukocyte-depleted and, if available, C, E, Kell-compatible and sickle neg RBCs
voc meds
VOC--Meds
  • Pain meds
    • NSAIDs: Ketorolac 0.5 mg/kg, max 30 mg
    • Opiates:
      • Morphine 0.1-0.2 mg/kg q 15-30 min PRN
      • Dilaudid 0.015-0.02 mg/kg
    • Mixed Opiate Agonist/Antagonist:
      • Nubain 0.2-0.3 mg/kg q3h PRN
  • Other meds:
    • Benadryl 1.25 mg/kg PO (NOT IV) q6 PRN
voc disposition
VOC--Disposition
  • Admission if not able to control pain OR significant drop in Hgb and/or retic
  • Ask if they think they can manage at home.
  • Home meds:
    • Ibuprofen 10 mg/kg q6-8h x 2d, then PRN
    • Tylenol #3 1 mg/kg q4-6h PRN breakthrough pain
    • Consider Lortab, Oxycodone, Morphine IR
  • Follow up with Sickle Cell clinic in 1-2 days by phone or in clinic
  • Call sickle cell consult.
fever
Fever
  • Defined as temp ≥ 38.3°C
  • Immunocompromise
  • Splenic infarction
  • Usually on Penicillin until 5 y/o
  • Usually have PCV7 and Pneumovax
  • Remember to treat concurrent pain
fever labs diagnostics
Fever--Labs & Diagnostics
  • CBC with diff
  • Reticulocyte count
  • Blood cultures
  • Consider CRP and Type & Screen
  • Consider urine or CSF as warranted
  • Chest XRay if respiratory symptoms
fever meds
Fever--Meds
  • No source:
    • GOAL: 30 minutes from door to antibiotics
    • Rocephin 50-75 mg/kg, max 2 gm IV/IM
    • If cephalosporin allergy: Meropenem 20 mg/kg IV, max 1 gm
  • If source found: treat as usual after IV Abx
  • If Acute Chest Syndrome:
    • Oxygen, pain meds
    • Consider adding Zithromax, nebulizers, and steroids
fever disposition
Fever--Disposition
  • Consider admission for observation if:
    • Age < 1 y/o
    • Previous bacteremia/sepsis
    • T > 40°C
    • WBC > 30 or < 5, plts < 100
    • Received Meropenem or Vancomycin
    • Infiltrate on CXR
    • Unable to comply with follow up
    • Other problems: pain, aplastic crisis, splenic sequestration, ACS, stroke, priapism
fever disposition14
Fever--Disposition
  • If labs unremarkable and well appearing, d/c home and f/u in 24 hours in sickle cell clinic for re-check and 2nd dose of Rocephin.
  • Call sickle cell consult.
caveat
CAVEAT
  • Read notes from previous visits.
    • There are some frequent flyers who are supposed to have pain plans in place with hematology.