History

A 34 year-old woman with a known history of sickle cell disease (HbSS) presents to the emergency room with a one-day history of back, shoulder and abdominal pain. The pain came on quite suddenly and was severe, deep and gnawing in nature. She took several dilaudid at home, but experienced little relief. She states that several family members at home recently contracted upper respiratory tract symptoms, but she denies any such symptoms herself. She was diagnosed with sickle cell disease as a child and she has a history of 1-3 pain crises per year, for which she is usually hospitalized for a brief period. She has never had acute chest syndrome. She has received periodic transfusions for symptomatic anemia, but no exchange transfusions. She is up to date with her vaccinations. She has no other medical problems, and all of her past hospitalizations have been related to her sickle cell disease. There is no family history of sickle cell disease on either her mother’s and father’s side. She is a non-smoker and denies any alcohol intake. She works as a bartender. She takes hydroxyurea 500 mg daily and folic acid. She has no known allergies to drugs.

When taking the history from a patient with potential vaso-occlusive crisis (VOC), it is important to consider the following:

  • In some cases, VOC is preceded by an obvious precipitating factor, including:
    • Dehydration
    • Stress (physical or emotional)
    • Infection
    • Asthma
    • Acidosis
    • Sleep apnea
    • Climate (especially sudden changes in temperature)
    • Pregnancy
    • Menstruation 
  • It is important to rule out other causes of pain, including:
    • Pneumonia
    • Acute chest syndrome
    • Papillary necrosis
    • Osteomyelitis
    • Avascular necrosis
    • Hepatic or splenic sequestration, or other hepatobiliary complications
    • Trauma
    • Causes not related to SCD such as migraine headache, menstruation, or septic arthritis