What are the causes of acute chest pain in sickle cell disease (SCD)?

From the Guidelines

Sickle cell chest pain, also known as acute chest syndrome, requires prompt medical attention to prevent high morbidity and mortality rates, as highlighted in the 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR guideline for the evaluation and diagnosis of chest pain 1. The treatment typically involves hospitalization for oxygen therapy, intravenous fluids, pain management with opioids like morphine, and antibiotics such as ceftriaxone and azithromycin to cover potential infections. Some key points to consider in the management of sickle cell chest pain include:

• Blood transfusions may be necessary in severe cases, as noted in the American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support 1
• For pain management at home, patients can use oral analgesics like hydrocodone/acetaminophen or oxycodone/acetaminophen along with NSAIDs if not contraindicated
• Staying hydrated by drinking 8-10 glasses of water daily, using incentive spirometry every 2-3 hours while awake, and avoiding triggers like cold temperatures, high altitudes, and strenuous exercise is important
• Acute chest syndrome occurs when sickled red blood cells block small blood vessels in the lungs, causing tissue damage, inflammation, and potentially pneumonia, which can lead to respiratory failure if not treated promptly, as emphasized in the 2021 guideline 1
• Any sickle cell patient with chest pain, fever, cough, or breathing difficulties should seek immediate medical care to prevent serious complications. The clinical spectrum of acute chest syndrome is variable, and although there are no specific markers of disease severity, a significant decline in the hemoglobin concentration and/or oxygen saturations can suggest severe disease, as noted in the 2020 guidelines 1. Therefore, prompt recognition and treatment of sickle cell chest pain are crucial to improve outcomes and reduce morbidity and mortality rates, as highlighted in the 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR guideline 1.

From the Research

Sickle Cell Chest Pain

• Sickle cell chest pain, also known as acute chest syndrome (ACS), is a serious complication of sickle cell disease (SCD) that can lead to significant morbidity and mortality 2.
• The management of ACS is challenging, and there is limited evidence to support the effectiveness of various treatment modalities, including opioids, antibiotics, and blood transfusions 2, 3.
• Opioids, such as morphine, are commonly used to manage pain in SCD patients, but their use can be associated with significant side effects and may exacerbate existing organ damage 4.
• The use of antibiotics in ACS is often empirical, and there is no standardized approach to treatment, highlighting the need for further research to determine the efficacy and safety of different antibiotic regimens 3.
• Emergency department management of patients with SCD presenting with chest pain or other acute complications requires special consideration, and healthcare providers must be aware of the unique challenges and controversies surrounding the management of these patients 5, 6.

Treatment Options

• Morphine and other opioids may be used to manage pain in SCD patients, but their use should be carefully monitored due to the risk of side effects and organ damage 4.
• Antibiotics may be prescribed to treat ACS, but the choice of antibiotic and duration of treatment should be guided by clinical judgment and further research is needed to determine the optimal approach 3.
• Blood transfusions may be considered in certain cases, such as severe anemia or acute chest syndrome, but the decision to transfuse should be made on a case-by-case basis 5.
• Other treatment options, such as incentive spirometry, positive expiratory pressure devices, and inhaled nitric oxide, may be considered in the management of ACS, but further research is needed to determine their effectiveness 2.