Principles & Practice of Pain Medicine > Part V. Pain Syndromes > D. Pain Associated with Medical Illness > Chapter 52. Sickle Cell Disease Natalie Moryl and Richard Payne

Sickle Cell Disease: Introduction

"Sickle cell disease is one of the most prevalent single gene disorders, affecting over 50,000 Americans. Sickle cell disease is a debilitating condition that is associated with chronic anemia, stroke, splenic and renal dysfunction, susceptibility to bacterial infections, particularly in children, acute chest syndrome, and pain crises. Each year in the US, an average of 75,000 hospitalizations are due to sickle cell disease, costing approximately $475 million. Athough tremendous resources have been invested in looking for a cure and palliative care, the management of acute and chronic pain remains the major intervention, focusing on improving quality of life and decreasing morbidity. In spite of some decrease in mortality in children with sickle cell disease over the last 25 years, median life expectancy for most sickle cell patients remains below age 50 years. Pain, the most frequent symptom experienced by the patients with sickle cell anemia, profoundly impairs patient function at home and work, their social interactions, and their careers. Despite the high prevalence of pain episodes, correlating with morbility and mortality, sickle cell anemia pain remains underestimated and undertreated. There are still multiple misconceptions regarding the use of pain medications, including opioids, tolerance, and addiction...."