Suzetrigine for Pediatric Sickle Cell Disease Vaso‐Occlusive Crisis: Is There a Role?
Abstract
Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by a single HBB gene mutation yielding abnormal β-globin alleles. The homozygous variant, HbSS, portrays the most severe form of the disease, with heterozygous genotypes producing enough HbS to cause clinical pathology. Deoxygenation of HbS leads to intraerythrocytic polymerization with resultant sickling of red blood cells. The occlusion of intravascular beds by sickled cells causes ischemia, tissue damage, reperfusion injury, and end-organ dysfunction. Systemic microvascular injury alongside a cascade of immunologic responses leads to acute painful vaso-occlusive crisis (VOC), representing the most common recurring sequalae from infancy into adolescence and adulthood [1]. The mainstays of acute VOC treatment include protocolized care with aggressive analgesia, individualized hydration, oxygenation and non-pharmacologic interventions [1, 2]. Albeit, achieving adequate pain control in acute VOC remains elusive. Non-steroidal anti-inflammatories and opioids are the recommended first-line analgesics. Nervous system sensitization alongside the adverse drug reactions associated with opioid therapy have propelled the utilization of adjuncts targeting alternative nociceptive receptors. Subanesthetic ketamine, lidocaine, gabapentin, midazolam, and regional anesthesia have been investigated among pediatric and adult cohorts [1-3]. Suzetrigine is a non-opioid selective inhibitor of Nav1.8 voltage-gated channels…
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