From the Guidelines
Glucose-6-phosphate dehydrogenase (G6PD) deficiency management requires avoiding triggers that can cause hemolytic anemia, and the most critical step is to avoid specific medications and foods that can induce oxidative stress, as recommended by recent guidelines 1. The management of G6PD deficiency involves a multi-faceted approach to prevent hemolytic episodes and improve quality of life.
Key Principles
- Avoiding medications that can trigger hemolysis, such as sulfonamides, nitrofurantoin, rasburicase, primaquine, dapsone, and methylene blue, is crucial, as these medications can cause severe hemolytic anemia in patients with G6PD deficiency 1.
- Certain foods, particularly fava beans, should be limited or avoided, as they can cause severe reactions in individuals with G6PD deficiency.
- High-dose vitamin C supplements should also be avoided, as they can induce oxidative stress and trigger hemolysis.
Acute Hemolytic Episodes
During acute hemolytic episodes, treatment involves:
- Discontinuing the triggering agent
- Providing supportive care with hydration
- Monitoring hemoglobin levels Severe cases may require blood transfusions to prevent morbidity and mortality.
Screening and Follow-up
Screening for G6PD deficiency is recommended upon entry into care or before starting therapy with an oxidant drug in patients with a predisposing racial or ethnic background, as suggested by the HIV Medicine Association of the Infectious Diseases Society of America 1. Family members should consider screening since G6PD deficiency is X-linked recessive, meaning males are more commonly affected while females can be carriers. Regular medical follow-up is essential to monitor the patient's condition and adjust the management plan as needed.
Quality of Life
Most patients with G6PD deficiency can lead normal lives by avoiding known triggers and following a comprehensive management plan. By prioritizing the avoidance of triggers and providing supportive care, patients with G6PD deficiency can reduce their risk of hemolytic episodes and improve their overall quality of life, as supported by recent evidence 1.
From the Research
Management of Glucose-6-phosphate (G6P) Deficiency
The management of Glucose-6-phosphate (G6P) deficiency, also known as Glucose-6-phosphate dehydrogenase (G6PD) deficiency, involves preventing hemolysis by avoiding oxidative stressors.
- The most effective management strategy is to prevent hemolysis by avoiding oxidative stressors, as stated in the study 2.
- Management for pain and anxiety should include medications that are safe and have not been shown to cause hemolytic crises, such as benzodiazepines, codeine/codeine derivatives, propofol, fentanyl, and ketamine 2.
- A real-world study found that certain medications, previously thought to be risky for G6PD-deficient patients, have been prescribed safely to these patients, providing reassurance to patients, prescribers, and regulators 3.
Medications and Hemolysis Risk
Certain medications can increase the risk of hemolysis in G6PD-deficient patients.
- A study found that nitrofurantoin, phenazopyridine, and a "pain killer" were associated with hemolysis in G6PD-deficient patients 3.
- Another study reported a case of a patient who developed intravascular hemolysis after taking a fixed-dose combination of isosorbide dinitrate and hydralazine, highlighting the need for a high degree of suspicion of G6PD deficiency in patients with unexplained signs and symptoms of intravascular hemolysis 4.
Treatment Strategies
Currently, there are no therapeutics for G6PD deficiency, but potential pharmacological pathways are being explored.
- A study discussed the role of G6PD in hemolytic and nonhemolytic disorders, treatment strategies attempted over the years, and potential reasons for their failure 5.
- The study also discussed potential pharmacological pathways, including glutathione (GSH) metabolism, compensatory NADPH production routes, and transcriptional upregulation of the G6PD gene, highlighting potential drug targets 5.