An overview of sickle cell disease and chronic pain and perioperative considerations

This review aims to provide the practicing anesthesiologist with information on the pathophysiology, physiology, and management of patients with sickle cell disease. This includes the evaluation of common intraoperative management issues as well as perioperative concerns related to the disease. This review will also discuss the outpatient care of sickle cell patients concerning disease-modifying agents and chronic pain management.

There has been less funding for research on sickle cell disease and a relative deficit in treatment options for patients and their vaso-occlusive crises (VOC). In the chronic pain setting, adjuvants to therapy have been utilized, such as anti-inflammatories, neuropathic agents, and antihistamines; however, the mainstay of therapy for pain crises is the use of opioids. New medications for the treatment of VOCs include crinaluzimab and L-glutamine. In terms of intraoperative management, it is imperative to maintain homeostasis for the patients, and research does not show a benefit in preoperative blood transfusions when not clinically indicated.

The sociopolitical context and pathophysiology of sickle cell disease make it a unique disease to manage for the practicing anesthesiologist. Tailoring management via developing a patient-specific approach to maintain homeostasis and minimize the perioperative prevalence of VOCs.