CLINICAL QUERIES: Understanding sickle cell disease
Bridget Parsh EdD, RN, CNS
Devina Kumar RN

August 2012 
Volume 42  Number 8
Pages 64 - 64
  PDF Version Available!

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.2-4 Complications of SCD include acute chest syndrome, hepatic and splenic sequestration, hemolytic anemia, pulmonary embolus, pulmonary hypertension, cholecystitis, iron overload, retinal detachment, hemorrhage,

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