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LEARNING OBJECTIVES

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After completing this case study, the reader should be able to:

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  • Recognize the clinical characteristics associated with an acute sickle cell crisis.

  • Discuss the presentation of acute chest syndrome and treatment options.

  • Recommend optimal analgesic therapy based on patient-specific information.

  • Identify optimal endpoints of pharmacotherapy in sickle cell anemia patients.

  • Recommend treatment that may reduce the frequency of sickle cell crises.

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PATIENT PRESENTATION

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Chief Complaint

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“I can’t breathe and my chest hurts.”

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HPI

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Todd Jefferson is a 38-year-old African-American man with a history of sickle cell anemia who presents to the local community hospital ED with pain. On waking up three days prior to admission, he experienced a sudden onset of pain in his hands, legs, and lower back. He began taking oxycodone 15 mg every 4 hours at that time with minor pain relief. This morning he experienced a fever of 102°F, progressive shortness of breath, and priapism, which caused him to seek treatment at the ED. Patient acknowledged having sick contacts at his workplace.

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PMH

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  • Sickle cell anemia (hemoglobin SS disease) diagnosed before the age of 1 with approximately three to four crises per year requiring hospitalization

  • Acute chest syndrome 2 years ago that required intubation

  • Transfusion exchange with PRBC during the intubation admission

  • Several episodes of priapism, usually associated with sickle cell pain crisis

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FH

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Mother and father alive and well, both with sickle cell trait. Patient has one sister with sickle cell trait.

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SH

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Lives locally with his wife; currently works as chemical engineer. Reports no use of tobacco; occasional alcohol intake for social events.

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ROS

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Denies nausea, vomiting, or diarrhea. Cannot remember his last bowel movement but believes he has not had one in the last 3 days. Has had fever with some chills and sweats; no cough, nasal discharge, rashes, or skin lesions. Reports stuttering priapism with recurring episodes each lasting approximately 1 hour, with no intervention.

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Meds

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  • Folic acid 1 mg PO daily

  • Hydroxyurea 1000 mg PO BID

  • Oxycodone 15 mg PO Q 4 H PRN pain

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All

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  • Sulfa (reported rash when very young)

  • Codeine (nausea and dysphoria)

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Physical Examination

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Gen
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Thin, well-developed, diaphoretic African-American man in acute distress

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VS
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BP 115/72 mm Hg, P 110 bpm, RR 20, T 38.5°C; 72 kg; O2 sat is 84% in room air improving to 97% on 4 L O2

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HEENT
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PERRL; EOMI; oral mucosa soft and moist; normal sclerae and funduscopic examination; no sinus tenderness

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