What is the management of chest pain in sickle cell disease?

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Last updated: June 2, 2025 • View editorial policy

From the Guidelines

Chest pain in sickle cell disease is primarily caused by vaso-occlusion from sickled red blood cells, leading to tissue hypoxia, inflammation, and potential pulmonary complications, and its management requires prompt evaluation and treatment, focusing on both pain control and addressing the underlying cause, particularly acute chest syndrome (ACS). The management of chest pain in sickle cell disease involves administering analgesics such as morphine or hydromorphone, along with NSAIDs like ketorolac if not contraindicated, as noted in studies such as 1. Oxygen supplementation should be provided to maintain saturation above 95%.

Key Considerations

  • Chest pain in sickle cell patients may indicate ACS, which requires aggressive management including IV fluids, incentive spirometry, and broad-spectrum antibiotics, as discussed in 2.
  • Blood transfusion therapy, either simple or exchange transfusion, should be considered for moderate to severe ACS, aiming for hemoglobin of 10 g/dL, with consultation from hematology being essential.
  • The pathophysiology involves vaso-occlusion from sickled red blood cells, which can lead to tissue hypoxia, inflammation, and potential pulmonary complications, as mentioned in 3.
  • Close monitoring for respiratory deterioration is crucial, as ACS can progress rapidly and may require intensive care support, highlighting the importance of prompt and aggressive management.

Management Approach

  • Initial management includes pain control with analgesics and addressing the underlying cause, particularly if ACS is suspected.
  • The use of hydroxyurea has been shown to decrease vaso-occlusive complications, including episodes of painful events and acute chest syndrome, as noted in 3.
  • Recent guidelines, such as those from the American Society of Hematology in 2020 2, emphasize the importance of early recognition and treatment of ACS, as well as the consideration of blood transfusion therapy in moderate to severe cases.
  • The 2019 guidelines by the American Society of Hematology 4 also highlight the challenges in developing guidelines for rare diseases like SCD but emphasize the need for expertise in SCD care to improve patient outcomes.

From the Research

Causes of Chest Pain in Sickle Cell Disease

  • Chest pain in sickle cell disease can be caused by several serious conditions, including chest syndrome, pulmonary embolism, and infectious complications 5
  • Acute chest syndrome (ACS) is a common complication and reason for hospital admission in patients with sickle cell disease (SCD) and is also the most common cause of death in this patient population 6
  • Infection is the most common identifiable cause for ACS, but other important triggers include vaso-occlusive crisis (VOC) and asthma 6
  • Other risk factors that precipitate ACS include hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use 7

Management of Chest Pain in Sickle Cell Disease

  • The key to the successful treatment of ACS is early recognition and initiation of treatment without delay 7
  • The main goal is to prevent and treat acute respiratory failure and, thus, minimize irreversible lung damage 7
  • Painful vaso-occlusive crisis (VOC) remains the most common reason for presenting to the Emergency Department and hospitalization in patients with sickle cell disease (SCD) 8
  • Adequate pain relief and monitoring of ventilatory patterns during the treatment of VOC are crucial, as pain-associated shallow breathing and maldistribution of ventilation may contribute to the pathogenesis of acute chest syndrome 9
  • Opioid analgesia can help reduce the differences in breathing patterns between patients with chest pain and those with pain at other sites 9

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Chest pain in sickle cell disease.

The Netherlands journal of medicine, 2013

Research

Acute chest syndrome: sickle cell disease.

European journal of haematology, 2011

Research

Acute chest syndrome in sickle cell disease.

Hospital practice (1995), 2018

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.