Vaso-occlusive pain crisis

Background

Precipitating Factors

• For vaso-occlusion:
  • Stress
  • Cold weather
  • Dehydration
  • Hypoxia
  • Infection
  • Acidosis
  • Alcohol intoxication
  • Pregnancy
  • Exertional stress

Clinical Features

• Pain
  • Most common manifestation of SCA (79-91% of ED visits)
  • Lower back, long bones most commonly affected
  • Because anemia can precipitate a crisis, must check for acute hemoglobin drop

Differential Diagnosis

Sickle cell crisis

• Vaso-occlusive pain crisis
• Bony infarction
• Dactylitis
• Avascular necrosis of femoral head
• Acute chest syndrome
• Asthma
• Pulmonary hypertension
• Gallbladder disease
• Acute hepatic sequestration
• Infection
  • Meningitis
  • Pneumonia
  • Septic joint
  • Osteomyelitis
• Parvovirus B19
• Splenic sequestration
• CVA
• Cerebral aneurysm and ICH
• Priapism
• Papillary necrosis

Evaluation

• CBC
  • Assess for significant anemia
• Chemistry / LFT / lipase
  • If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
• Retic count (if aplastic crisis considered - rare in adults)
  • Should be >0.5%
• CXR
  • If cough, shortness of breath, or febrile
• O2
  • If hypoxic; otherwise may inhibit erythopoesis
• ECG
• T&S/T&C
• blood culture2
• VBG
• UA
• Urine pregnancy
• Head CT/MRI
  • If symptoms of stroke

Management

• Analgesia
  • IV opiods prefered (Morphine or hydormorphone)
    • Avoid IM route (use SQ if necessary)
    • Avoid meperidine
    • Use PCA pump if available
    • Redose in 30min if inadequate
    • Normally admit if needs more than three doses
  • Use of concurrent acetaminophen encouraged, unless contraindicated
• Hydration
  • Initial bolus should be 1/2NS with goal of increasing MCV of sickled cells
  • 1.5 times maintenance with D5 1/2NS
  • Normal Saline boluses for hypovolemic shock
• O2 is not useful in the nonhypoxic patient

Anemia

• Transfusion
  • Indications:
    • Aplastic crisis
    • Sequestration crisis
    • hemoglobin <6 with inappropriately low retic count
    • hemoglobin <10 with acute crisis
  • Transfuse 10 mL/kg over 2hr period

Disposition

• Consider admission to the hospital if:
  • Acute chest syndrome is suspected
  • Sepsis, osteomyelitis, or other serious infection is suspected
  • Priapism, aplastic crisis, hypoxia
  • WBC >30K
  • Platlet <100K
  • Pain is not under control after 2-3 rounds of analgesics in ED
  • <1yr old
• Consider discharge if:
  • Pain is under control and patient can take oral fluids and medications
  • Ensure appropriate oral analgesics are available
  • Provide home care instructions
  • Ensure resource for follow-up