From the Guidelines
The workup for sickle cell acute chest syndrome (ACS) involves a comprehensive evaluation, including a complete blood count, reticulocyte count, and blood cultures, as patients with SCD are at high risk of developing severe disease 1.
Key Components of Workup
- Patients should receive empiric antibiotic therapy with ceftriaxone and azithromycin for a minimum of 7-10 days, as ACS is a leading cause of death in patients with SCD 1.
- Oxygen therapy should be initiated to maintain oxygen saturation between 94-98% or at the patient's usual saturation level, as hypoxia can indicate severe disease 1.
- Pain management should be provided with morphine as needed, and chest radiography and pulmonary function tests may also be performed to assess the extent of pulmonary involvement.
Additional Considerations
- Baseline information about the patient, such as SCD genotype, prior splenectomy, and chronic complications, should be readily available to healthcare professionals treating acute illness 1.
- Strategies for ensuring the availability of baseline information include computerized patient databases and medical alert cards.
- Older adolescents and adults with SCD who present with chest pain and shortness of breath should be evaluated for acute myocardial infarction (AMI) or myocardial ischemia, as AMI can occur at a relatively early age in patients with SCD without traditional risk factors 1.
From the Research
Diagnostic Criteria for Acute Chest Syndrome
The diagnostic criteria for acute chest syndrome (ACS) in patients with sickle cell disease (SCD) include:
- A new infiltrate on pulmonary imaging combined with any of the following: fever > 38.5°C (101.3°F), cough, wheezing, hypoxemia (PaO2 < 60 mm Hg), tachypnea, or chest pain 2
- The presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray 3
Workup for Acute Chest Syndrome
The workup for ACS in patients with SCD includes:
- Physical exam
- Blood work
- Chest imaging, with chest radiograph considered the gold standard for imaging modality 2
- Laboratory studies, such as complete blood count, comprehensive metabolic panel, brain natriuretic peptide (BNP), lactic acid, procalcitonin, EKG, troponin level, and d-dimer 2
- Point-of-care lung ultrasonography, which has been shown to be a feasible alternative to chest radiography for screening for ACS in young patients with SCD 4
Management of Acute Chest Syndrome
The management of ACS in patients with SCD includes:
- Hydration with IV crystalloid solutions
- Antibiotics
- Judicious analgesia
- Oxygen
- Transfusion therapy, including blood transfusion or exchange transfusion 3, 2, 5
- Incentive spirometry and positive expiratory pressure device 6
- Bronchodilators, if there is a history of asthma or in the presence of acute bronchospasm 3
- Treatment with hydroxyurea, which should be considered for prevention of recurrent episodes 3