Sickle cell crisis

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Background

  • Sickle cell disease is the most common monogenetic disorder worldwisde affecting 30 million people
  • Disease can manifest with vaso-occlusion(pain), acute chest syndrome, stroke, aplastic anemia, priapism

Precipitating Factors

  1. For vaso-occlusion:
    1. cold weather
    2. hypoxia
    3. infection
    4. dehydration
    5. acidosis
    6. alcohol intoxication
    7. emotional stress
    8. pregnancy
    9. exertional stress
  2. For aplastic crisis:
    1. parvovirus B19 infection
    2. folic acid deficiency
  3. For acute chest syndrome:
    1. fat embolus
    2. infection
    3. pain crisis
    4. asthma

Diagnosis

Insert

Work-Up

  1. CBC / stat hemaglobin
    1. Assess change in anemia
  2. Chemistry / LFT / lipase
    1. If abd pain, may develop cholecystitis, mesenteric ischemia, or perforation
  3. Reticulocyte count (if aplastic crisis considered) -rare in adults
  4. CXR
    1. If cough, sob, or febrile
  5. O2
    1. If pain crisis or hypoxic; otherwise may inhibit erythopoesis
  6. ECG
  7. T&S/T&C
  8. BCx2
  9. VBG
  10. UA
  11. Urine pregnancy
  12. Head CT/MRI if symptoms of stroke

DDx

  1. ACS
  2. anemia (acute, chronic)
  3. appendicitis
  4. cholecystitis
  5. gout
  6. hepatitis
  7. meningitis
  8. osteomyelitis
  9. pancreatitis
  10. PID
  11. pneumonia
  12. priapism
  13. pulmonary embolism/infarction
  14. rheumatic fever
  15. sepsis
  16. stroke (ischemic or hemorrhagic)
  17. UTI
  18. URI

Treatment

  1. Abd Pain
    1. Surgery if abdominal injury
  2. Aplastic Anemia
    1. Exculde reverible causes and tranfuse for severe anemia (Hb <6-7)
  3. Pain crisis
    1. IVF, pain meds, O2 if hypoxic
  4. Priapism
    1. Pain meds, exchange tranfusion before surgery, urology consult
  5. Acute chest syndrome
    1. Admit if infiltrate, treat pain, give IV Abx
  6. Stroke
    1. Head CT +/- LP, IVF, keep HbS <30% total blood vol

Disposition

  1. Admission if:
    1. Acute Chest Syndrome - pain/pulmonary infiltrates from infection or pulm infarct
    2. Stroke, priapism, serious bacterial infection, aplastic crisis, hypoxia, acidosis
    3. Unable to tolerate PO or inadequate pain control, abnormal vital signs
    4. Consider if pregnant (in crisis), or uncertain dx of SCD

See Also

Acute Chest Syndrome

Source

DONALDSON 6/07 SOTELO 10/09

TUNG 7/10

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