Acute chest syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical Therapy
Acute Pharmacotherapy
- The primary goal is to prevent the development of ACS. It is therefore essential to vaccinate patients with the pneumococcal and haemophilus influenza B (HIB) vaccines and prevent hypoxemia.
- The acute treatment of ACS is primarily supportive, and depends on the likelihood of the above diagnoses.
- Antibiotics should be initiated, with the goal of covering the typical and atypical organisms that cause community acquired pneumonia.
- Supplemental oxygen to maintain a PaO2 >70.
- Hydration to prevent intravascular sickling are essential.
- Pain control and incentive spirometry to avoid splinting is also important.
- There is some data, however, that suggests that vigorous hydration in combination with narcotic analgesics can lead to pulmonary edema. Therefore, euvolemia should be the goal.
- Empiric anticoagulant therapy is currently not recommended because of the risk of intracranial and renal bleeding.
- Exchange transfusion is recommended in the setting of progressive infiltrates and hypoxemia, with a goal of reducing the Hb S level to below 30%, while keeping HCT ~30%.
- Hydroxyurea has been associated with a 50% reduction in the frequency of developing both pain crises and ACS, and studies are underway examining its use in the acute treatment of patients with ACS.
Chronic Pharmacotherapy
The primary goal is to prevent the development of ACS. It is therefore essential to vaccinate patients with the pneumococcal and HIB vaccines and prevent hypoxemia.