www.Nursing2017.com January l Nursing2017 l 67 SICKLE CELL DISEASE (SCD) is a group of chronic genetic disorders that affects an estimated 70,000 to 80,000 Americans.1 SCD is characterized by abnormal… Click to show full abstract
www.Nursing2017.com January l Nursing2017 l 67 SICKLE CELL DISEASE (SCD) is a group of chronic genetic disorders that affects an estimated 70,000 to 80,000 Americans.1 SCD is characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in red blood cells that distort the cells’ normal rounded shape into a sickle or crescent shape.2 (See Sickle cells bend the rules.) Because sickle-shaped cells can’t flow freely through blood vessels, they may cause vascular occlusion resulting in severe pain and other signs and symptoms. Patients with SCD are at high risk for acute and chronic complications that may result in disability or death.3 Acute vaso-occlusive crisis (VOC), the most common complication of SCD, results from tissue ischemia related to vascular occlusion.3 It’s characterized by excruciating pain— usually of sudden onset, although gradual onset is also possible. Nearly all patients with SCD experience VOC at some point, but the frequency is variable and unpredictable.3 VOCs lead to 197,000 ED visits and 356 million dollars spent on pain management every year.4 Many patients with SCD visit the ED repeatedly for pain management because VOCs are typically recurrent. However, despite their severe pain, patients with VOC are too often undertreated for reasons discussed in this article. This article also proposes ways to improve pain management for patients experiencing this painful disorder.
               
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