Apparent desaturation on pulse oximetry because of hemoglobinopathy

Hemoglobinopathies are an uncommon cause of cyanosis and low oxygen saturation on pulse oximetry. However, when they do occur, they can present a complex clinical scenario for the emergency physician. We report the index case of a previously undescribed hemoglobinopathy that presented to the pediatric emergency department. The evaluation and management of the cyanotic/hypoxic child and review of hemoglobinopathies are presented here.