DOI: 10.36347/sjmcr.2013.v01i02.007

Pages: 42-43

Santa Ana hemoglobinopathy is a rare hemoglobinopathy which has been reported in the literature.We report a patient with Santa Ana hemoglobinopathy who presented for esophagogastroduedenoscopy (EGD).Santa anahemoglobinopathy is a genetic defect in the hemoglobin molecule which makes it unusually susceptible to thermal degradation. As the hemoglobin molecule denatures it changes conformation which means that pulse oximeters are not able to sense the hemoglobin and detect whether or not it is carrying oxygen. Such patients therefore have aberrantly low pulse oximeter measurements. If patients with this rare disorder are to be anesthetized, the pulse oximetry readings will not be useful to determine true oxygen saturation and alternative monitoring such as arterial line placement and repetitive arterial blood gas measurement will be necessary.