Abstract

Walter Clement-Noel, a dental student from Grenada, reported the first case of sickle cell disease (SCD) in Chicago in 1910. The hallmark of this genetic disorder is vasoocclusive crises associated with severe pain. SCD occurs in 1 of every 365 black or African American births and 1 in 16,300 Hispanic American births. SCD affects an estimated 100,000 Americans. During the last century, significant advances have been made in understanding the disease and its management, thereby leading to longer life expectancy. For patients, families, and physicians this chronic disease remains a major health problem with significant morbidity because of acute and chronic complications that can affect many, if not all, organ systems. SCD results in frequent hospitalizations and leads to decreased quality of life, challenges in pain management, psychosocial issues, and a significant mortality rate. SCD is a major cost to the healthcare system because of the high utilization of medical sources, with more than 200,000 emergency department (ED) SCD-related visits each year and an annual cost of medical care of patients exceeding $1 billion. Most studies also have noted that the high proportion of the SCD-related healthcare costs results from frequent ED visits and hospitalizations, which could be reduced by preventing complications such as pain crises and infections.ri