What is the management approach for a patient with sickle cell disease experiencing shortness of breath?

From the Guidelines

Management of a patient with sickle cell disease experiencing shortness of breath requires prompt evaluation for acute chest syndrome (ACS), which is a life-threatening complication, and initial steps should include administering supplemental oxygen to maintain saturation above 94-98% as recommended by the BTS guideline for oxygen use in adults in healthcare and emergency settings 1. The clinical approach to such a patient involves several key steps:

• Administering supplemental oxygen to maintain the recommended saturation level
• Providing adequate analgesia with opioids such as morphine
• Ensuring proper hydration with IV fluids
• Obtaining immediate blood tests and chest X-ray to evaluate for pulmonary infiltrates
• Starting early broad-spectrum antibiotics to cover common pathogens
• Considering simple or exchange blood transfusion if there is clinical deterioration, with a goal of reducing HbS to less than 30% as suggested by the American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support 1
• Using incentive spirometry to prevent atelectasis
• Close monitoring in an intensive care setting due to the potential for rapid deterioration of respiratory status

It's also important to consider the risk of pulmonary hypertension in patients with sickle cell disease, as it is a well-established risk factor for mortality, and guidelines recommend risk stratification using noninvasive measurements such as tricuspid regurgitant jet velocity via Doppler echocardiography or serum N-terminal pro–brain natriuretic peptide level, and consideration of hydroxyurea for patients with increased risk for mortality 1. Key aspects of management include:

• Prompt recognition and treatment of acute chest syndrome
• Aggressive supportive care including oxygen therapy, analgesia, and hydration
• Consideration of blood transfusion for clinical deterioration
• Monitoring for and management of pulmonary hypertension
• Use of hydroxyurea for patients with increased risk for mortality due to pulmonary hypertension.

From the Research

Causes of Shortness of Breath in Sickle Cell Disease

• Shortness of breath in sickle cell disease can be caused by various factors, including acute chest syndrome (ACS) 2, 3, 4, 5.
• ACS is a leading complication of sickle cell disease, characterized by chest pain, cough, dyspnea, fever, abnormal lung examination, leukocytosis, hypoxia, and new radiographic opacities 2, 4, 5.
• The pathophysiology of ACS involves vaso-occlusion in pulmonary vessels, resulting in hypoxia, release of inflammatory mediators, acidosis, and infarction of lung tissue 5.
• Infections, rib infarction, and fat emboli are common precipitants of ACS 5.

Management Approach for Patients with Sickle Cell Disease Experiencing Shortness of Breath

• The management of ACS includes hydration with IV crystalloid solutions, antibiotics, judicious analgesia, oxygen, and, in severe cases, transfusion 5.
• Early recognition and initiation of treatment are crucial to prevent acute respiratory failure and minimize irreversible lung damage 2.
• The use of transfusions should be targeted to the severity and likelihood of ACS progression, with stable, non-hypoxic patients with favorable hematologic and radiographic findings usually not requiring transfusion 4.
• Exchange transfusion is recommended for severe progressive ACS 4.
• A quality improvement framework, such as the Emergency Department Sickle Cell Assessment of Needs and Strengths (ED-SCANS), can guide emergency department team members in the assessment and management of patients with sickle cell disease 6.

Key Considerations for Emergency Department Practitioners

• Emergency medicine practitioners should keep ACS as a high-consequence differential diagnosis for all patients with sickle cell disease presenting to the emergency department 5.
• A detailed medical history, physical examination, and laboratory and imaging studies are essential for diagnosing and managing ACS 5.
• Appropriate disposition to the intensive care unit after consultation with hematology may be necessary for patients with severe ACS 5.