Mexiletine: An adjuvant option for refractory pain in patients with sickle cell disease and comorbid autism spectrum disorder

Abstract

Sickle cell disease (SCD) is a hemoglobinopathy that causes structural alterations to the beta-globin component of hemoglobin, leading to formation of abnormally rigid, sickle-shaped red cells. 1,2 This causes recurrent vaso-occlusive crises (VOC) secondary to vessel blockage, ischemia, and microinfarction, manifesting as excruciating pain episodes. The resulting inflammation, necrosis, and nerve damage causes acute pain, but also leads to chronic centralized/neuropathic pain over time. 3 Effectively managing pain in this population represents a significant clinical challenge.Management of acute VOC includes hydration, nonsteroidal antiinflammatory drugs (NSAIDs), and opioids. For chronic pain, antide-