Sickle Cell Disease: Pain & Fever

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Sickle Cell Disease Pain Fever

• John Cheng, MD
• PEM Fellows Conference
• July 19, 2006

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Sickle Cell Disease

• Hemoglobin S
• Glu ? Val at 6 position of ß hemoglobin
• Various types
• SS
• SC
• Sß-thalessemia
• Others

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Sickle Cell Issues

• Vaso-Occlusive Crisis
• Sickling and subsequent ischemia
• Immunocompromise
• Splenic infarction
• Encapsulated organisms H. influenzae, S.
  pneumonia
• Salmonella

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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

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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

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VOC--Diagnostics

• CXR if respiratory symptoms
• Ultrasound--abdominal
• CT scan--head

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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

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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

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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.

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Fever

• Defined as temp 38.3C
• Immunocompromise
• Splenic infarction
• Usually on Penicillin until 5 y/o
• Usually have PCV7 and Pneumovax
• Remember to treat concurrent pain

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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

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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

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Fever--Disposition

• Consider admission for observation if
• Age lt 1 y/o
• Previous bacteremia/sepsis
• T gt 40C
• WBC gt 30 or lt 5, plts lt 100
• Received Meropenem or Vancomycin
• Infiltrate on CXR
• Unable to comply with follow up
• Other problems pain, aplastic crisis, splenic
  sequestration, ACS, stroke, priapism

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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.

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CAVEAT

• Read notes from previous visits.
• There are some frequent flyers who are supposed
  to have pain plans in place with hematology.