Gene mutations that affect the synthesis or structure of hemoglobin are present in 7% of the world’s population. These mutations lead to variant hemoglobinopathies with phenotypes that range from clinically insignificant to fatal. Because monitoring oxygen saturation is standard of care and critical to detecting hypoxemia, it is crucial to recognize factors that might interfere with the measurement of oxygen saturation or decrease oxygen delivery. We discuss the management of a patient who presented to a freestanding surgical center with the rare variant hemoglobin Kansas, clinical cyanosis, and an initial pulse oximetry reading of 68% on room air.
From the Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland.
Accepted for publication November 30, 2018.
The author declares no conflicts of interest.
Address correspondence to Yael S. Varnado-Rhodes, MD, Department of Anesthesiology and Critical Care Medicine, Johns Hopkins Outpatient Center, Johns Hopkins University, c/o Yvette James, 601 N Caroline St, JHOC B165, Baltimore, MD 21287. Address e-mail to email@example.com.