Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management

Updated: May 01, 2020
  • Author: Srikanth Nagalla, MD, MS, FACP; Chief Editor: Emmanuel C Besa, MD  more...
  • Print
Treatment

Approach Considerations

Most individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency do not need treatment. However, they should be taught to avoid drugs and chemicals that can cause oxidant stress. Patients should also avoid broad beans (ie, fava beans). Favism occurs primarily in the Mediterranean variety of G6PD deficiency.

Identification and discontinuation of the precipitating agent is critical to manage hemolysis in patients with G6PD deficiency. Anemia should be treated with appropriate measures, recognizing that hemolysis is self-limited and often resolves in 8 to 14 days. Transfusions are rarely indicated. Splenectomy is usually ineffective.

Infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy with a bili light (see Neonatal Jaundice). Exchange transfusion may be necessary in cases of severe neonatal jaundice or hemolytic anemia caused by favism. However, Samanta and colleagues found that in neonates with idiopathic hyperbilirubinemia, transfusion with G6PD-deficient blood was significantly less effective than transfusion with G6PD-normal blood. [32]

Patients with chronic hemolysis or non-spherocytic anemia should be placed on daily folic acid supplements. Consultations with a hematologist and a geneticist should be sought.

Next:

Deterrence/Prevention

Persons with G6PD deficiency need to avoid foods, drugs, and chemicals that can precipitate hemolysis. The risk posed by those substances is determined in part by the person's G6PD variant (see Etiology) and thus the degree of enzyme deficiency.

Fava beans are the food best known for precipitating hemolysis; indeed, favism—the term for symptomatic attacks of hemolytic anemia from eating fava beans—has been recognized since antiquity. [10] Other foods that some persons with G6PD deficiency may prefer to avoid include the following [33] :

  • Red wine
  • All legumes
  • Blueberries
  • Soya products
  • Tonic water

For most persons with G6PD deficiency, the following drugs pose a definite risk: [1]

  • Dapsone and other sulfones (higher doses for dermatitis herpetiformis more likely to cause problems)
  • Methylthioninium chloride
  • Niridazole
  • Nitrofurantoin
  • Pamaquin
  • Primaquine (30 mg weekly for 8 weeks has been found to be without undue harmful effects in African and Asian people)
  • Quinolones (ciprofloxacin, moxifloxacin, nalidixic acid, norfloxacin, ofloxacin)
  • Rasburicase
  • Sulfonamides (including co-trimoxazole; however, some sulfonamides, such as sulfadiazine, have been found not to be hemolytic in many G6PD-deficient individuals)

Drugs that pose a possible risk in some persons with G6PD deficiency include the following:

  • Aspirin (acceptable up to a dose of at least 1g daily in most G6PD-deficient individuals)
  • Chloroquine (acceptable in acute malaria and malaria chemoprophylaxis)
  • Menadione (vitamin K3) and water-soluble derivatives (eg, menadol sodium phosphate)
  • Quinidine (acceptable in acute malaria)
  • Quinine (acceptable in acute malaria)
  • Sulfonylureas
  • Naphthalene in (mothballs)

Intravenous vitamin C is currently undergoing testing as adjunctive treatment for sepsis. Case reports describe episodes of hemolysis in patients with G6PD deficiency who received high doses of intravenous (IV) vitamin C (> 60 g). However, Marik cites dramatic reductions of methemoglobinemia and hemolysis that have been reported in patients treated with IV vitamin C in low to moderate doses (1-10 g every 6 hours), and proposes that those data suggest that vitamin C at a dosage of 6 g/day should not be considered contraindicated in patients with known or suspected G6PD deficiency—that indeed, IV vitamin C may be the treatment of choice in G6PD-deficient patients with drug-induced hemolysis.  Sepsis can cause methemoglobinemia, and methylene blue—the first-line treatment for acquired methemoglobinemia—is contraindicated in patients with G6PD deficiency. [34]

 

Previous