Problem: A 12-year-old female with known sickle cell disease (SCD) present to the Emergency Room in sickle cell crisis. The patient is crying with pain and states this is the third acute episode she has had in the last nine months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made. Appropriate therapeutic interventions were initiated by the APRN and the patient's pain level decreased, and she was transferred to the pediatric intensive care unit (PICU) for observation and further management.
What is the pathophysiology of acute SCD crisis and why is pain the predominate feature of acute crises?
Discuss the genetic basis for SCD.