How is severe hypoxia treated in patients with acute chest syndrome (ACS) and sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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For episodes of severe hypoxia, rapid progression, diffuse pulmonary involvement, and failure to improve, erythrocytapheresis is indicated. Intensive care is indicated for patients in severe hypoxia or respiratory distress, as respiratory decompensation can rapidly require mechanical ventilation.Treatment should also include oxygen therapy with close monitoring for hypoxemia with continuous pulse oximetry or frequent assessment of blood gases.

Administer oxygen if saturation is less than 94%. If that level cannot be maintained at a fraction of inspired oxygen (FiO2) of 0.4, provide simple transfusion (avoid raising hematocrit to more than 36%). If no improvement is seen, reduce the HbS level to 30% with erythrocytapheresis or exchange transfusion. The process can rapidly progress to respiratory failure. Ventilatory assistance may be required.

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