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.
Links:- a good review of hydroxyurea here.
- the original article of hydroxyurea's effects from NEJM here.
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