General Session: Sickle Cell Disease: Pathophysiology, Pain, and Prevention of Problems (103)

3:00 pm – 4:00 pm Friday, September 16

Sickle Cell Disease (SCD) is the most common inherited hemoglobinopathy in the United States; the etiology is a DNA mutation that results in the different amino acids at position 6 of the beta-globin chain, where valine is produced instead of glutamine. The sickling of the red blood cell sets off a cascade of pathology. Vaso occlusion causing ischemia to almost every body system accounts for the majority of morbidity and is the etiology of acute pain episodes. Acute pain episodes account for the majority of health care utilization by this population.

In the US, approximately 100,000 people live with Sickle Cell Disease. According to recent statistics from the Centers for Disease Control and Prevention: 1 in 365 African Americans and 1 in 16,300 Hispanic Americans are affected. Due to a better understanding of the disease, newborn screening in all 50 states, vaccines, antibiotics, and other medications and treatments, now more than 95% of children in the United States with SCD are surviving into adulthood.

Many patients with SCD in the US, both pediatric and adult, do not have access to Comprehensive Sickle Cell Centers, which tend to be located in urban areas and are often associated with academic centers. For patients in a more rural area, care may be rendered by providers not familiar with managing the disease or comfortable doing so. During this session, we will discuss the standard of care for treating a patient with SCD in any setting, whether a Comprehensive Sickle Cell Center or a Community Hospital. It is important for pediatric hematology oncology nurses to have an understanding of how this disease affects our patients and the various screenings and treatments which have been developed to optimize outcomes.