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LEARNING OBJECTIVES

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LEARNING OBJECTIVES

Upon completion of the chapter, the reader will be able to:

  1. Explain the underlying causes of sickle cell disease (SCD) and their relationship to patient signs and symptoms.

  2. Identify the typical characteristics of SCD as well as symptoms that indicate complicated disease.

  3. Identify the desired therapeutic outcomes for patients with SCD.

  4. Recommend appropriate pharmacotherapy and nonpharmacotherapy interventions for patients with SCD.

  5. Recognize when chronic maintenance therapy is indicated for a patient with SCD.

  6. Describe the components of a monitoring plan to assess effectiveness and adverse effects of pharmacotherapy for SCD.

  7. Educate patients about the disease state, appropriate therapy, and drug therapy required for effective treatment and prevention of complications.

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KEY CONCEPTS

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  • Image not available. Sickle cell disease (SCD) is an inherited disorder caused by a defect in the gene for hemoglobin. Patients may have one defective gene (sickle cell trait [SCT]) or two defective genes (SCD).

  • Image not available. Although most often seen in persons of African ancestry, other ethnic groups can be affected.

  • Image not available. SCD involves multiple organ systems.

  • Image not available. Prophylaxis against pneumococcal infection reduces death during childhood.

  • Image not available. Hydroxyurea has been shown to decrease the incidence of painful crises. However, the patient population that receives hydroxyurea should be carefully monitored.

  • Image not available. Chronic transfusion therapy programs have been shown to be beneficial in decreasing the occurrence of stroke in children with SCD.

  • Image not available. Patients with fever greater than 38.5°C (101.3°F) should be evaluated, and appropriate antibiotics should include coverage for encapsulated organisms, especially pneumococcal.

  • Image not available. Pain episodes can usually be managed at home. Hospitalized patients usually require parenteral analgesics. Analgesic options include opioids, nonsteroidal anti-inflammatory agents, and acetaminophen. The patient characteristics and the severity of the crisis should determine the choice of agent and regimen.

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INTRODUCTION

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Image not available. "Sickle cell syndrome" refers to a collection of autosomal recessive genetic disorders that are characterized by the presence of at least one sickle hemoglobin gene (HbS).1,2

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Sickle cell disease (SCD) is a chronic illness that is associated with frequent crisis episodes. Acute complications are unpredictable and potentially fatal. Common symptoms include excruciating musculoskeletal pain, life-threatening pneumonia-like illness, cerebrovascular accidents, and splenic and renal dysfunction.2 As the disease progresses, patients may develop organ damage from the combination of hemolysis and infarction. Because of the complexity and severity of SCD, it is imperative that patients have access to comprehensive care with providers who have a good understanding of the countless clinical presentations and the management options of this disorder.

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EPIDEMIOLOGY AND ETIOLOGY

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Sickle cell trait (SCT) is the heterozygous form (HbAS) of SCD in which a person inherits one normal adult hemoglobin (HbA) gene and one sickle hemoglobin (HbS) gene. These individuals are carriers of the SCT and are usually asymptomatic.2 Symptomatic disease is seen in homozygous and compound heterozygous genotypes of SCD. Sickle cell anemia (SCA) is the homozygous (HbSS) state of SCD.2 It ...

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