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doi: 10.1097/01.NURSE.0000415854.17386.06

Understanding sickle cell disease

Parsh, Bridget EdD, RN, CNS; Kumar, Devina RN

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Bridget Parsh is an assistant professor of nursing at California State University Sacramento, where Devina Kumar recently graduated from the LVN-to-RN program.

The authors thank California State University Sacramento for supporting the school of nursing's publication efforts.

The authors have disclosed that they have no financial relationships related to this article.

A young adult with sickle cell disease was recently admitted to our ED with an acute pain episode. What's the underlying pathophysiology of this pain?—W.L., MISS.

Bridget Parsh, EdD, RN, CNS, and Devina Kumar, RN, respond: Sickle cell disease (SCD), the most common inherited blood disorder in the United States, is caused by a mutation in the hemoglobin-beta gene found on chromosome 11.1 SCD is actually a group of disorders characterized by abnormal hemoglobin molecules called hemoglobin S.2 The abnormal hemoglobin shortens the lifespan of red blood cells (RBCs), causing anemia. SCD also causes RBCs to form a crescent or sickle shape and to become less flexible. The resultant vasoocclusion and subsequent tissue ischemia and inflammation cause acute pain and possible organ damage.24 Complications of SCD include acute chest syndrome, hepatic and splenic sequestration, hemolytic anemia, pulmonary embolus, pulmonary hypertension, cholecystitis, iron overload, retinal detachment, hemorrhage, and leg ulcers.5

Although many adults with SCD lead normal, healthy lives most of the time, when they're under stress—for example, from dehydration, infection, physical exertion, or exposure to extreme temperature—they can experience an intensely painful crisis requiring expert nursing assessment and care.6

Your nursing assessment should include a comprehensive pain assessment, including pain triggers, if any. Be sure to ask the patient what she or he feels is an acceptable pain intensity level. Perform medication reconciliation and determine what nonpharmacologic and pharmacologic interventions have helped manage the patient's pain in the past to help guide current treatment.4

Your patient who comes to the ED with severe pain may need I.V. morphine or hydromorphone. Reassess the patient's pain 15 to 30 minutes after administering the dose. The patient who has continued pain may need additional doses every 20 to 30 minutes and may be hospitalized for around-the-clock I.V. therapy if more than one additional dose is required. If one dose has handled the pain adequately, the patient may be discharged home with long-acting opioids such as oxycodone and a prescription to be filled in case of break-through pain. However, if the patient doesn't usually require long-acting opioids at home, these may not be prescribed.4

Positive relationships with healthcare providers are important for managing this disease, but unfortunately some providers are unsympathetic to patients with sickle cell pain. Healthcare professionals may not adequately perceive the severity of SCD and the pain associated with it. Some studies have found that healthcare professionals may characterize SCD patients as drug dependent or drug seeking.7 A conflict between the patient's and health professional's viewpoints can cause friction and prevent proper care and understanding of the pain associated with a vasoocclusive crisis.

Figure. Sickle cell ...
Figure. Sickle cell ...
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Good communication and mutual respect and understanding, along with appropriate nursing and medical care, may facilitate positive outcomes for patients with SCD.

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1. National Human Genome Research Institute. Learning about sickle cell disease. 2011.

2. Genetics Home Reference. Sickle cell disease. 2007.

3. Vichinsky EP. Overview of the clinical manifestations of sickle cell disease. UpToDate. 2012.

4. DeBraun MR, Vichinsky EP. Acute pain management in adults with sickle cell disease. UpToDate. 2012.

5. Tanabe P, Reddin C, Thornton VL, Todd KH, Wun T, Lyons JS. Emergency Department Sickle Cell Assessment of Needs and Strengths (ED-SCANS), a focus group and decision support tool development project. Acad Emerg Med. 2010;17(8):848–858.

6. Brown M. Managing the acutely ill adult with sickle cell disease. Br J Nurs. 2012;21(2):90–92, 95–96.

7. Wright K, Adeosum O. Barriers to effective pain management in sickle cell disease. Br J Nurs. 2009;18(3):158–161.

© 2012 Lippincott Williams & Wilkins, Inc.