Sickle cell disease was first described in 1910 by Herrick (1910) with the first molecular disease defined and described by Linus Pauling (1980). It was Sydenstricker who first used the word crisis to describe the abdominal pains and jaundice that occurs during the event. Ingram in 1956 examined the electrophoretic properties of normal and sickle hemoglobin and discovered the substitution of glutamic acid for valine at the sixth position of the hemoglobin chain which led to a change in ionic charge from neutral to phobic. Deoxygenation leads to crystal formation, tactoids, and stacking of red blood cells, such that sludging in the microvascula-ture occurs and results in ischemia and organ infarction. Hemolysis results in jaundice. The vaso-occlusive crisis of sickle cell disease is heralded by pain that may be localized or diffuse (Ballas 2002).
Over two million African-Americans and Hispanic-Americans are affected by sickle cell disease. Other people affected include those of Arab, Indian, and Asiatic descent or wherever there was malarial spread from equatorial Africa. Generally patients present with abdominal pain, back pain, or extremity pain, especially of the legs. Any one or combination of these regions may be affected during a vaso-occlusive crisis (VOC) (Wethers 2000, Serjeant and Serjeant 2004). Shapiro in the 1990s described the event as a painful episode to de-emphasize the emotional component in an effort to improve coping (Shapiro et al. 1995). Despite an attempt to control the behavioral facet by de-emphasizing the anxiety, it has been shown that many patients objectively test positive for anxiety and a sense of helplessness that may be a co-morbidity for this life-threatening disease. The unpredictability of onset and severity of pain coupled with the uncertainty of sequelae and shortened lifespan can be likened to living in a mine field.
Most patients with sickle cell disease have few crises that require hospitalization. Approximately 25% of all patients with sickle cell disease have frequent crises. Many VOC episodes are handled at home or in day hospitals which support the fact that early, effective intervention can curtail most uncomplicated crises (Benjamin et al. 2000). However, when a patient fails at home care, strong analgesics are indicated. Of patients who present to the emergency departments 50% do so for painful events. Approximately 30% present with febrile events and 20% for combined pain and fever (Frush et al. 1995). Patients often require large doses of opioids but obtain minimal relief due to the complex nature of the pain since rheological, inflammatory, and ischemic factors come into play. Elevated substance P levels appear to contribute to the acute episodes (Douglas 2008). Conversely, pain scores are not affected by the amount of opioid administration (Jacob et al. 2003). Since opioids do not have a ceiling effect of analgesia, increased doses are given in an effort to diminish the pain. Oftentimes, the sedation from the opioids can be severe, resulting in inadequate ventilation, hypoxia, and a worsening of the crisis pathophysiology. Acute chest syndrome, a complicated VOC presentation, has been associated with high-dose systemic exposure to morphine (Kopecky et al. 2004).
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