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

  • 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.


Chief Complaint

“I can’t breathe and my chest hurts.”


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.


  • 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


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


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


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.


  • Folic acid 1 mg PO daily

  • Hydroxyurea 1000 mg PO BID

  • Oxycodone 15 mg PO Q 4 H PRN pain


  • Sulfa (reported rash when very young)

  • Codeine (nausea and dysphoria)

Physical Examination


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


BP 115/72 mm Hg, P 110 bpm, RR 20, T 38.5°C; 72 kg; O2 sat is 84% in ...

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