Sickle Cell Disease (SCD) and Perioperative Pain Management

Sickle cell diseaseSickle cell disease (SCD) is an autosomal recessive disorder that involves the inheritance of a mutant Beta globin gene. The Beta globin gene is encoded at chromosome 11. The defective hemoglobin S (HbS) results from a substitution mutation that occurs at the sixth amino acid where Glutamine is substituted for Valine on chromosome 11 (Kumar et al, Robbins basic pathology, Elsevier, Saint Louis, pp 411–3, 2012 [1]; Lanzkron, Sickle cell anemia straight to the point of care [Internet], 2023 [2]). At least one HbS allele must be present to develop sickle cell disease. In cases where only one HbS allele exists, co-inheritance of defective Beta hemoglobin such as occurs in the setting of Beta thalassemia or Hemoglobin C disease may result in clinical manifestations of sickle cell disease. Inheritance of a hemoglobin S allele from both parents results in the most severe form of sickle cell diseaseSickle cell disease (SCD) also known as sickle cell anemia (Lanzkron, Sickle cell anemia straight to the point of care [Internet] 2023, [2]). Patients with sickle cell trait have one normal gene (HbA) and another sickle gene and are commonly asymptomatic or have symptoms of diminished magnitude.