A 24-year-old man with sickle cell disease (hemoglobin SS) comes to the emergency department with chest pain and shortness of breath. He has felt unwell for the last several days with increasing extremity, abdominal, and chest pain. The patient took extra doses of oral morphine with little relief. He stopped taking hydroxyurea 4 months ago due to stomach upset.
His blood pressure is 122/72 mm Hg, pulse is 108/min, respirations are 22/min, and oxygen saturation is 84% on room air. Bilateral crackles are heard on chest auscultation. A 2/6 midsystolic murmur is heard at the left upper sternal border. The abdomen is diffusely tender without rebound. There is no swelling of the extremities.
Laboratory results are as follows:

Chest x-ray reveals bilateral dense infiltrates involving both upper and lower right lung fields and the lower left lung field. Intravenous fluids, supplemental oxygen, and antibiotics are initiated.
Which of the following is the best next step in management of this patient?

Question

A 24-year-old man with sickle cell disease (hemoglobin SS) comes to the emergency department with chest pain and shortness of breath.  He has felt unwell for the last several days with increasing extremity, abdominal, and chest pain.  The patient took extra doses of oral morphine with little relief.  He stopped taking hydroxyurea 4 months ago due to stomach upset.
His blood pressure is 122/72 mm Hg, pulse is 108/min, respirations are 22/min, and oxygen saturation is 84% on room air.  Bilateral crackles are heard on chest auscultation.  A 2/6 midsystolic murmur is heard at the left upper sternal border.  The abdomen is diffusely tender without rebound.  There is no swelling of the extremities.
Laboratory results are as follows:

Chest x-ray reveals bilateral dense infiltrates involving both upper and lower right lung fields and the lower left lung field.  Intravenous fluids, supplemental oxygen, and antibiotics are initiated.
Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec193a_961b_e20d_853d_2951329a7536_MD0002_00 This patient with sickle cell disease has features characteristic of severe acute chest syndrome (ACS): multiple lobar infiltrates on chest imaging associated with chest pain, and dyspnea with hypoxemia. Initial supportive measures in ACS include intravenous hydration, analgesics, broad-spectrum antibiotics, incentive spirometry, and supplemental oxygen. chest imaging In addition, definitive management of moderate or severe ACS includes blood transfusions, which acutely increase oxygenation. Moderate cases (<2 pulmonary lobes involved on imaging and SaO₂ >85% on room air) may be managed with conventional red cell transfusions. However, most experts advise exchange transfusion for severe ACS (>2 lobes involved or SaO₂ <85%) to enable rapid replacement of red cell mass without causing hemoconcentration and hyperviscosity. < (Choice B) Glucocorticoids as a treatment for ACS have been shown to reduce length of hospitalization. However, concern for significant complications, including rebound vaso-occlusive events, increased risk of avascular necrosis, and an association with hemorrhagic stroke, has limited the clinical utility of glucocorticoids. (Choice C) Hydroxyurea is recommended to decrease the frequency of pain crises and acute chest syndrome in sickle cell disease, but it would not be helpful in the acute setting. (Choice D) Low-dose naloxone, an opioid antagonist, can be used occasionally to relieve opioid-induced nausea and pruritus without affecting the analgesic benefit of opioids. However, this approach is not recommended routinely. (Choice E) Noninvasive ventilation has been suggested as an additional supportive measure in ACS. However, in trials, noninvasive ventilation failed to show improvement in hypoxemia, transfusion rates, pain, or length of stay. Educational objective: Acute chest syndrome in sickle cell disease is characterized by pulmonary infiltrates associated with fever, pain, hypoxemia, and pulmonary crackles. Management includes supplemental oxygen, intravenous hydration, broad-spectrum antibiotics, and pain medication. Mild cases may be managed without transfusion. Moderate cases require conventional red cell transfusion, and exchange transfusion is recommended in severe cases. __________ References: Pulmonary complications of sickle cell disease. (http://www.ncbi.nlm.nih.gov/pubmed/22447965)

A 24-Year-Old Man with Sickle Cell Disease (Hemoglobin SS) Comes