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INTRODUCTION

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Sickle-cell disease (SCD) is the most prevalent single-gene disorder, affecting about 100,000 Americans. Sickle-cell disease is a debilitating condition associated with chronic anemia, stroke, splenic and renal dysfunction, susceptibility to bacterial infections in children, acute chest syndrome, and pain crises. Each year in the United States, an average of 75,000 hospitalizations are due to SCD, costing approximately $475 million. Despite a decrease in mortality in children with SCD over the last 25 years, median life expectancy for most SCD patients remains less than 50 years of age. Although tremendous resources have been invested in finding a cure, barriers to analgesia in SCD remain and stereotyping of patients persists. Major barriers to pain management are misconceptions regarding patients’ report of pain, use of opioids and other pain medications, and perception of addiction. This chapter discusses the correlation between SCD pain and mortality; mistrust between patients and health care providers (HCPs) as one reason for undertreated pain; patients’ perceptions of health care system response to their needs; and the need for clinical guidelines to provide appropriate comprehensive care to patients with SCD.

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HISTORICAL PERSPECTIVES

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Sickle-cell disease was identified in the United States in 1910, when sickle-shaped red blood cells were found in the blood of a medical student from Africa. Autosomal dominant inheritance was identified in 1923, and oxygen deprivation of tissues was shown to provoke sickling in 1927. Single amino acid substitution of glutamic acid for valine in the sixth amino acid position of the B chain of hemoglobin (Hb) was demonstrated to be responsible for physicochemical features of altered Hb. Further studies demonstrated that both altered erythrocytes and endothelial cells express surface molecules that mediate intracellular adhesions, leading to polymerization of deoxygenated sickle-cell hemoglobin (HbS) and vaso-occlusion. Advances in basic and clinical research did not significantly change the outcomes for patients until recently, when antibiotics, hydroxyurea, and bone marrow transplantation (BMT) were introduced. Bone marrow transplantation, first performed in 1998, is currently available as a cure for a selected group of patients only. Pain management and supportive care have been the cornerstones of the treatment of SCD since its discovery and remain the best intervention to reduce morbidity and improve the quality of life for most patients.

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PREVALENCE

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The Centers for Disease Control and Prevention (CDC) estimates that

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  • SCD affects 90,000 to 100,000 Americans.

  • SCD occurs in about 1 in every 500 black or African-American births.

  • SCD occurs in about 1 in 12 blacks or African Americans.1

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MORTALITY

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In 1973, the average life span for individuals with SCD was only 14 years.2 Current life expectancy for SCD patients is 50 years and more.3 Women with SCD live longer than men.

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Vaso-occlusion resulting from Hb polymerization and erythrocyte rigidity, as well as chronic anemia, hemolysis, and vasculopathy, is central to the pathophysiology of ...

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