Introduction: Acute Chest Syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. The current practice guidelines lack sufficient detail on the evidence behind our standard of practice. Materials and Methods: A comprehensive literature search was accomplished with PubMed with a particular focus on literatures published between 2015-2021 with the keywords ACS management, vaso-occlusive crisis, sickle cell, antibiotics, opioids, transfusion, fluid, steroids, bronchodilators, incentive spirometry, anticoagulation, oxygen, prevention, and SARS-CoV2. We included literatures both in the adult and pediatric population. Results/Discussion: ACS is defined as a new infiltrate in a chest radiograph involving at least one broncho-pulmonary segment in an individual with one additional clinical finding. There are multiple causes of ACS which include infections, pulmonary edema, pulmonary infection, hypoxemia and hypoventilation. The goal is to quickly diagnose and treat this condition to prevent irreversible lung damage and mortality. In this narrative review, we discuss why a balanced approach to fluid and pain management will provide better outcome for patients, and the evidence behind using antibiotics, steroids, bronchodilators, nitric oxide, incentive spirometry, as well as the current management of patients with concomitant ACS and SARS-CoV2.
Conclusion:All patients admitted with ACS should be started on a third-generation cephalosporin and macrolide or a fourth-generation fluoroquinolone, incentive spirometry, thromboprophylaxis, oxygen and simple or exchange transfusion if needed, with a balanced approach to fluid resuscitation and pain control.