•ACS is the leading cause of morbidity and mortality in children and adults with Sickle Cell Disease.
•Definition: ==> A new infiltrate on CXR (excluding atelectasis) PLUS one or more of the following: ◦Tachpnea
◦Fever (>101 degrees F)
•Treatment ◦Bronchodilators Trial of beta-agonists for clinical response is advocated even in those without wheezing.
◦Antibiotics Broad Spectrum: Ceftriaxone PLUS Azithromycin
Evidence demonstrates a significant amount of these patients have atypical bacterial infections
Vanco is warranted for severe disease unresponsive to therapy
◦Steroids Use for patients with Reactive Airway Disease or severe distress
They may cause a rebound of Vaso-occlusive Crisis and need to be tapered.
Prednisone 2mg/kg/Day x 5 then taper
◦Pain Control Need to optimize pulmonary toilet by providing adequate pain management, but avoid over-sedation leading to hypoventilation.
NSAIDs have proven to be useful in conjunction opiods.
◦Transfusion of PRBCs Simple For pts who have a >10-20% drop from their baseline Hgb
For pts who are symptomatic, but not in impending respiratory failure
Try not to EXCEED Hgb of 10g/dL post transfusion
Exchange For pts with impending respiratory failure
For pts with Hgb > 10g/dL and significant symptoms (to avoid hyperviscosity)
The decision to transfuse these patients needs to be made in conjunction with the consulting