Acute Chest Syndrome
Acute Chest Syndrome
Background [1, 2]
- A leading cause of hospitalization and mortality in sickle cell patients
- Up to half of sickle cell patients will develop
- More common in HbSS but can occur in any phenotype
- Higher incidence in children but more severe and worse outcomes in adults
- Definition: fever and/or respiratory symptoms + radiodensity on chest x-ray
Children: Most commonly associated with infection or reactive airway disease
- Atypical bacteria or Respiratory Syncytial Virus (RSV)
- Inflammation results in pulmonary vaso-occlusion
Adults: Bone marrow/fat emboli
- Vaso-occlusion in bone marrow leads to release of bone marrow/fat emboli into circulation
- Mechanical obstruction occurs in small vessels of lungs
- Intra-pulmonary vascular obstruction, as a result of infection or emboli, causes hypoxia, inflammation, and acidosis that further worsen vaso-occlusion.
Supplemental oxygen and incentive spirometry
- Consider noninvasive positive-pressure ventilation if unable to maintain oxygen saturation > 92% with nasal cannula or non rebreather mask.
IV fluids to avoid hypovolemia
- D5 1/2 NS (resuscitate to euvolemia)
- Third generation cephalosporin + macrolide
- Exchange transfusion has potential advantage- higher volume of packed Red Blood Cells to lower the Hgb S percentage.
- Simple transfusion- should be used, regardless of severity, to achieve goal of Hgb > 10 g/dL if anemia present
- Caution over-sedation that can worsen respiratory drive
- Bronchodilators can be considered for patients with reactive airway disease.
- Glucocorticoids shown to decrease hospital stay, but associated with re-admission
Venous thromboembolism prophylaxis
- All patients with suspected Acute Chest Syndrome should receive prophylaxis of venous thromboembolism unless contraindicated.
- Acute Chest Syndrome occurs in both children and adults regardless of sickle cell disease phenotype.
- Treatment focuses on minimizing vaso-occlusion in pulmonary vasculature: Oxygen, IV fluids, antibiotics, blood transfusion
- Although pulmonary infection is less common in adults, cover with antibiotics since cannot distinguish from embolic cause in initial phase.
- Vichinsky EP, Styles LA, Colangelo LH, et al. Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course. Cooperative Study of Sickle Cell Disease. Blood. Mar, 1997; 89(5):1787-1792.[PubMed]
- Lovett PB, Sule HP, Lopez BL. Sickle Cell Disease in the Emergency Department. Hematol Oncol Clin N Am. 2017; 31: 1061-1079. [PubMed]
- Simon E, Long B, Koyfman A. Emergency Medicine Management of Sickle Cell Disease Complications: An Evidence-Based Update. J Em Med. 2016; 51(4):370-381. [PubMed]
- Porter M. Rapid Fire: Sickle Cell Disease. Emerg Med Clin N Am. 2018; 36: 567-576. [PubMed]
- Farooq S, Omar MA, Salzman GA. Acute chest syndrome in sickle cell disease. Hosp Prac. Apr, 2018; 46(3): 144-151. [PubMed]