- Acute chest syndrome (ACS) is the leading cause of death in sickle cell patients
- ACS is defined by the presence of a new infiltrate and one of the following: chest pain, wheezing, fever, tachypnea, or cough
- Early and aggressive therapy is needed to minimize mortality
- Up to 50% of patients develop respiratory failure
- Treatment
- Broad spectrum antibiotics - including a macrolide
- Pain control to reduce hypoventilation
- Early use of blood transfusion to improve O2 carrying capacity
- Incentive spirometry
- Bronchodilators if wheezing present
- Hematology consult
