Understanding and Managing Glucose-6-Phosphate Dehydrogenase Deficiency

• This article will raise awareness of G-6-PDD for nurses and NPs.
• The pathophysiology and various clinical manifestations of G-6-PDD are thoroughly explained along with screening tests.
• The role of the NP in the management and care of these patients is discussed.

Glucose-6-phosphate dehydrogenase deficiency (G-6-PDD) is a genetic defect that leaves erythrocytes vulnerable to hemolysis upon certain exposures. The resulting anemia, although generally self-limiting, may be severe enough to necessitate exchange transfusion. In addition, neonates are at risk from an unusually abrupt rise in bilirubin that is strongly associated with kernicterus. Although the condition is often benign, the potential for significant morbidity makes ignorance of the defect a threat to patients and quality of care. In this article we review the history, pathophysiology, clinical manifestations, differential diagnosis, diagnostic screening, and the role of the nurse practitioner in caring for patients with this disorder.