From the Guidelines
Acute chest syndrome in sickle cell disease presents with symptoms such as chest pain, fever, cough, shortness of breath, and low oxygen levels, and is a medical emergency requiring prompt treatment. Patients may also experience wheezing, chills, and increased respiratory rate [ 1 ]. The clinical spectrum of ACS is variable, and although there are no specific markers of disease severity, a significant decline in the hemoglobin concentration and/or oxygen saturations (SpO2 # 94% or several percentage points below the patient’s baseline) can suggest severe disease [ 1 ].
Key Symptoms
- Chest pain
- Fever
- Cough
- Shortness of breath
- Low oxygen levels
- Wheezing
- Chills
- Increased respiratory rate
According to the American Society of Hematology 2020 guidelines for sickle cell disease, early recognition and treatment of ACS are crucial, as it is one of the leading causes of death in patients with SCD [ 1 ]. The 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR guideline for the evaluation and diagnosis of chest pain also highlights the importance of prompt evaluation for patients with sickle cell disease who are experiencing chest pain [ 1 ].
Importance of Prompt Treatment
Prompt treatment is essential to prevent complications and improve outcomes in patients with ACS. This may include interventions such as antibiotics, oxygen, invasive and non-invasive respiratory support, bronchodilators, nitric oxide, and corticosteroids [ 1 ]. Clinicians should maintain a high index of suspicion for acute chest syndrome in sickle cell patients presenting with respiratory symptoms or chest pain.
From the Research
Symptoms of Acute Chest Syndrome (ACS) in Sickle Cell Disease (SCD)
The symptoms of ACS in SCD include:
- Respiratory symptoms such as fever, cough, chest pain, and shortness of breath 2, 3, 4, 5, 6
- Hypoxia and decreasing hemoglobin values 3
- Progressive multilobar pneumonia 3
- Neurologic events, including respiratory failure 3
- Abnormal lung examination and leukocytosis 6
- New radiographic opacities 2, 6
Clinical Presentation
Patients with ACS may present with varying degrees of severity, and the initial presentation can vary 2. The condition can progress to respiratory failure requiring mechanical ventilation in severe cases 2, 3. The mean length of hospitalization for ACS is approximately 10.5 days, with 13% of patients requiring mechanical ventilation and 3% resulting in death 3.
Risk Factors
Several risk factors contribute to the development of ACS, including: