What is the management of pneumonia or acute coronary syndrome (ACS) in patients with sickle cell disease (SCD)?

From the Guidelines

Management of pneumonia or acute chest syndrome (ACS) in sickle cell disease (SCD) patients should prioritize early initiation of empiric antibiotic therapy, supplemental oxygen, pain management, and hydration, with consideration of blood transfusion therapy, particularly automated or manual red cell exchange (RCE) for severe cases, as suggested by the American Society of Hematology 2020 guidelines 1. For suspected pneumonia or ACS, empiric antibiotic therapy should be initiated immediately with a third-generation cephalosporin (ceftriaxone 1-2g IV every 24 hours or cefotaxime 1-2g IV every 8 hours) plus a macrolide (azithromycin 500mg IV/PO daily).

• If MRSA is suspected, add vancomycin 15-20mg/kg IV every 12 hours.
• Supplemental oxygen should be provided to maintain oxygen saturation above 95%.
• Pain management with opioids is essential, typically morphine 0.1mg/kg IV every 2-4 hours as needed.
• Hydration with IV fluids at 1.5 times maintenance rate is crucial, but avoid overhydration to prevent pulmonary edema.
• Early blood transfusion therapy should be considered, with simple transfusion for mild cases and exchange transfusion for severe cases (PaO2 <70 mmHg, progressive respiratory failure, or multi-organ failure), as automated or manual RCE may be preferred over simple transfusions in patients with severe ACS, according to the ASH guideline panel 1.
• Incentive spirometry every 2 hours while awake is vital to prevent atelectasis.
• Bronchodilators should be used if there is evidence of reactive airway disease.
• Close monitoring of vital signs, oxygen saturation, and respiratory status is essential, as SCD patients are particularly vulnerable to respiratory infections due to functional asplenia and impaired immunity, and any respiratory infection can trigger sickling in the pulmonary vasculature, leading to a vicious cycle of hypoxemia, increased sickling, and worsening ACS.

From the Research

Management of Pneumonia or ACS in SCD patients

• The management of acute chest syndrome (ACS) in patients with sickle cell disease (SCD) is crucial, as ACS is a leading cause of morbidity and mortality in this patient population 2, 3.
• Early diagnosis and timely management of ACS result in better outcomes, but the effectiveness of most treatment modalities for ACS management has not been established 2.
• Treatment options for ACS include broad-spectrum antibiotics, pain control, and blood transfusions 4, 5.
• Incentive spirometry, positive expiratory pressure device, intravenous dexamethasone, oral vs. intravenous morphine, inhaled nitric oxide, unfractionated heparin, and blood transfusion have been evaluated in randomized controlled trials, but more high-quality studies are needed to support recommendations with stronger evidence 2.
• Mycoplasma pneumoniae infection is commonly associated with ACS in patients with SCD, and aggressive treatment with broad-spectrum antibiotics, including a macrolide, is recommended 4.
• Streptococcus pneumoniae is also a potent mediator of ACS in SCD patients, and its virulence factors, such as hydrogen peroxide and pneumolysin, contribute to lung injury 6.
• Universal vaccinations and antibiotic prophylaxis play a significant role in decreasing mortality from pulmonary infections in patients with SCD 5.
• Pulmonary complications, such as pulmonary hypertension and venous thromboembolism, should be managed according to established guidelines, and sleep disorders of breathing should be considered in patients with unexplained nocturnal and daytime hypoxemia or recurrent vaso-occlusive events 5.