Wednesday, January 28, 2009

Acute Chest Syndrome in Sickle Cell Disease

The Acute Chest Syndrome will unfortunately occur in up to 50% of patients with sickle cell disease. It is defined by:
  • Fever
  • Chest pain
  • New pulmonary infiltrate
  • Tachypnea, wheezing, or a cough
Underlying etiologies include Infection (community acquired pneumonia), viral infection, pulmonary infarction, fat embolism, but mostly this is idiopathic (over 40%). The infarction stems from RBC's sickling and clogging up the pulmonary microvasculature.

Treatment of this condition is pretty much supportive and includes:
  • Maintaining a PaO2 between 70 to 100 mmHg
  • Attaining and maintaining Euvolemia - crystalloids are best
  • Pain management - typically will require opioid analgesics
  • Coverage for community acquired pneumonia
Other things to consider:
  • Exchange transfusion - if there are progressive pulmonary infiltrates or severe dyspnea that is not responding to the above therapy.
One last word: patients should be vaccinated against encapsulated organisms (Pneumococcus, Meningococcus, H. Flu), and consideration should be given to placing patients on Hydroxyurea for the prevention of subsequent sickle cell crises.

  • a good review of hydroxyurea here.
  • the original article of hydroxyurea's effects from NEJM here.

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