Perioperative considerations for patients with sickle cell disease: a narrative review

Approximately 200,000 individuals worldwide are born annually with sickle cell disease (SCD). Regions with the highest rates of SCD include Africa, the Mediterranean, and Asia, where its prevalence is estimated to be 2-6% of the population. An estimated 70,000-100,000 people in the United States have SCD. Due to enhanced newborn screening, a better understanding of this disease, and more aggressive therapy, many sickle cell patients survive into their adult years and present more frequently for surgery.

The authors identified relevant medical literature by searching PubMed, MEDLINE®, EMBASE™, Scopus™, Web of Science, and Google Scholar databases for English language publications appearing from 1972-September 2016. Case reports, abstracts, review articles, and original research articles were reviewed-with particular focus on the pathophysiology and medical management of SCD and any anesthesia-related issues.

Perioperative physicians should be familiar with the triggers of a sickle cell crisis and vaso-occlusive disease. Sickle cell disease affects various organ systems, including the central nervous, cardiovascular, pulmonary, genitourinary, and musculoskeletal systems. Preoperative assessment should focus on end-organ dysfunction. Controversy continues regarding if and when sickle cell patients should receive transfusions and which anesthetic technique (regional or general) confers any benefits. Timely, appropriate, and sufficient analgesia is critical, especially when patients experience a vaso-occlusive crisis, acute chest syndrome, or acute postoperative pain.

Effective management of SCD patients in the perioperative setting requires familiarity with the epidemiology, pathophysiology, clinical manifestations, and treatment of SCD.