Acute Chest Syndrome

• 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)
  • Chest Pain
  • Cough
  • Wheezing
  • Hypoxemia
• 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 Hematologist.

References

NHLBI.  Acute chest syndrome and other pulmonary complications.  Management of Sickle Cell Disease.  June 2003; 25 – 29.  http://www.nhlbi.nih.gov/health/prof/blood/sickle/index.htm.

Kathleen Ryan, RN, MPH, Anju Chawla, MD and Matthew Heeney, MD. Management of Acute Chest Syndrome in Sickle Cell Disease. New England Pediatric Sickle Cell Consortium. 2005.