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LEARNING OBJECTIVES

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 to reduce the frequency of sickle cell crises.

PATIENT PRESENTATION

Chief Complaint

“I’m in pain all over and no one believes me! I can’t breathe ... and I don’t like being considered an addict!”

HPI

Danny Johnson is a 21-year-old African-American man with a history of sickle cell anemia who presents to the local community hospital ED frustrated and in 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 2 hours at that time with limited pain relief. When reaching out to his physician for additional pain medication, he was denied due to a disbelief of the patient’s recently reported pain. His primary physician said that he was taking much larger doses of opioids than the physician’s other patients. The ED physician questioned his level of pain and the opioid doses that he reported taking at home. This morning he had a temperature of 102°F, progressive shortness of breath, and priapism, which caused him to seek treatment at the ED. The patient acknowledged having sick contacts at his workplace.

PMH

Sickle cell anemia (hemoglobin SS disease) diagnosed before the age of 1 with approximately seven to eight 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

FH

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

SH

College student who is struggling to pass courses due to frequent missing of school for hospitalizations; depressed because sickle cell is affecting his life including limitation in sports and social activities. Struggling financially to keep up with hospital bills due to lapses in insurance.

ROS

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 ...

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