Monday, 17 September 2012

What is G6PD Deficiency?

What is G6PD Deficiency?

G6PD Deficiency (Glucose-6-phosphate dehydrogenase Deficiency) is the most common inherited enzyme deficiency in the world. G6PD deficiency affects an estimated 400 million people worldwide and there are over 400 genetic variants of the deficiency.  It is an inherited condition (passed on from previous generations) which requires genetic blood testing to check for the condition. It is a lifelong condition.

The enzyme, glucose-6-phosphate dehydrogenase is essential for normal red blood cell function and oxidizing processes. The deficiency may destroy red blood cells and lead to hemolytic anemia and jaundice after exposure to certain oxidative drugs, infections or fava beans (this also includes the pollen from the plant) and certain legumes. Most people with G6PD lead normal lives provided they avoid certain foods and drugs.

What to avoid

  • Sulphonamides (check with your doctor)
  • Co-trimoxazole (Bactrim, Septrin)
  • Dapsone
  • Chloramphenicol
  • Nitrofurantoin
  • Nalidixic acid
  • Always discuss with your doctor
  • Chloroquine
  • Hydroxychloroquine
  • Primaquine
  • Mepacrine
  • Napthalene (Mothballs)
  • Methylene blue
  • Fava beans - also called broad beans and other legumes
  • Sulfites and foods containing them. They are used in many foods so check labels carefully
  • Menthol and foods containing it. Candy, breath mints, chewing gum, mouth wash, toothpaste and many other products have menthol added to them.
  • Artificial blue food colouring. Other food colourings can also cause hemolysis. Natural food colour found in tumeric and grapes is ok.
  • Artificial Ascorbic acid commonly put in food and vitamins can cause hemolysis in large doses.
  • Tonic Water or energy drinks (contains Quinine)
  • Bitter Gourd/ bitter mellon and Garden Egg. Common foods in some parts of Africa and Asia
  • Some Chinese herbs: Rhizoma Coptidis (huang lin), Calculus Bovis (neu huang), Flos Chimonanthi Praecosis (leh mei hua), Flos Lonicerae (kam ngan fa) and Margarita or anything containing them.
Other Drugs
  • Sulphasalazine
  • Methyldopa
  • Large doses of vitamin C
  • Hydralazine
  • Procainamide
  • Quinidine
  • Some anti-cancer drugs
  • Menadione (Vitamin K3) When administered to newborns with G6PD Deficiency has been known to cause adverse outcomes including hemolytic anemia, neonatal brain or liver damage, or neonatal death.

Signs and symptoms

If you or your child has G6PD deficiency, you should get a check with your doctor if ever any of the following symptoms develop:
  • Pale skin (pallor)
  • Persistent / severe tiredness
  • Dark urine
  • Jaundice (yellow skin or eyes)
  • rapid heartbeat
  • rapid breathing or shortness of breath
  • dizziness or confusion
What to do incase of hemolytic crisis

If you detect the above symptom you should either call your doctor or paediatrition, or go directly to the nearest hospital. Avoid taking any drugs. You will probably be asked to list all foods and drugs taken in the preceeding 48 hours.


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