What is G6PD Deficiency
G6PD deficiency is a hereditary genetic abnormality resulting in insufficient amounts of an enzyme called glucose-6-phosphate dehydrogenase in a person’s blood. G6PD is an important protein or enzyme which is responsible for regulating numerous biochemical reactions within the body.
G6PD also helps ensure that a person’s red blood cells are healthy allowing them to function effectively and to survive as long as they should. If you are deficient in G6PD, these red blood cells will die prematurely. This premature destruction of blood cells is called hemolysis which can lead eventually to a condition known as hemolytic anemia.
Hemolytic anemia occurs when the red blood cells get destroyed quicker than they can be replaced. This results in less oxygen flowing to the tissues and organs. The resulting symptoms include fatigue, jaundice and a shortness in breath.
A person who is deficient in G6PD may develop hemolytic anemia after exposure to certain triggers. Some of the most common triggers are fava beans, various legumes and certain medications including common drugs like aspirin.
G6PD deficiency which is commonly referred to as favism is most prevalent in Africa and the Mediterranean. Worldwide, some 400 million people have the condition with as many as 20% of all Africans affected. As well as geography being a risk factor, it is far more common for men to be affected than women.
Tye majority of people with this deficiency do not experience any sort of symptom unless they are exposed to a trigger. Avoiding the food and medication that destroys the red blood cells is the best way to prevent complications. Even when a person becomes symptomatic, the symptoms typically resolve soon after the underlying has been removed.
Symptoms of Hemolytic Anemia
The majority of people with G6PD deficiency do not experience and symptoms but when exposed to one of the triggers, hemolytic anemia occurs. These are the most common symptoms:
- Jaundice which causes the skin and white parts of the eyes to appear yellow. G6PD deficiency is the most common cause of severe jaundice in newborns.
- Breath shortness and heavy or faster than normal breathing.
- Abnormally dark urine
- Increase in body temperature
- Faster than normal heart rate
- Confusion and problems concentrating
Causes of G6PD Deficiency
G6PD deficiency is a genetic condition meaning that it gets passed from both parents to their children. The condition is characterized by a genetic mutation to a G6PD gene. The abnormal gene which causes the deficiency is on one of the sex chromosomes – specifically the X chromosome.
Men have just a single X chromosome but women possess two of them. In males, just one mutated copy of this gene is sufficient to cause the deficiency. In females on the other hand, mutations need to have occurred in both of the copies.
Because it is far less likely for both copies of the gene to be altered, men are statistically far more prone to being G6PD deficient than their female counterparts. Sons with mothers who are healthy carriers of this mutation or are G6PD deficient themselves are the most likely people to inherit this condition.
Risk Factors for Complications
The biggest concern for people with G6PD deficiency is that they are more likely to develop hemolytic anemia. This condition can bring on a lot of adverse symptoms as listed above.
There are several triggers that people with G6PD deficiency need to avoid in order to prevent hemolytic anemia.
Consuming certain food can cause a negative reaction but not everybody will have the same reaction to the same food. The food to avoid includes fava beans (or broad beans) and certain legumes.
People with G6PD deficiency may react to a fairly long list of medications including aspirin and the anti-malarial drug – quinine.
Certain illnesses, infections and viruses can cause further complications for those with G6PD deficiency.
Treatment for G6PD Deficiency
When a person with G6PD deficiency experiences symptoms, the treatment involves the removal of whatever trigger caused them.
If the symptoms have been caused by some sort of illness or infection then the illness or underlying infection will be treated as usual.
If the symptoms have been caused by a particular medication, then the medication must be discontinued as soon as possible. The same is true where the symptoms are caused by a food that someone consumed. In such cases, the majority of people will make a full recovery by themselves.
Once the symptoms have progressed further and a patient has hemolytic anemia, the treatment may be more aggressive. Treatment for hemolytic anemia includes blood transfusions to replenish the patient’s red blood cells and oxygen. Oxygen therapy is also used to treat the condition. Hospitalization and close monitoring by medical professionals is important to ensure full recovery without any further complications.
Natural Ways to Avoid G6PD Symptoms
G6PD deficiency is a hereditary disorder so there is no actual cure for the condition itself. The important thing for people with the disorder is to avoid the triggers that can lead to complications.
1) Avoid Consuming Certain Foods and Drinks
Not everybody will have the same reaction to the same foods but there are a number of foods that are known to cause a reaction in many people with G6PD deficiency.
These are the main foods and beverages that people should try to avoid:
- Fava beans also known as broad beans. These seem to cause the greatest number of people to react adversely. Because of this, the condition is commonly referred to as favism.
- Legumes. While many people do not suffer an adverse reaction from other types of legumes, many do. If in doubt, avoid eating any legumes.
- Foods with soy content including tofu, tempeh, miso and soy milk.
- Vitamin C supplements and citrus fruit or other food rich in vitamin C.
- Food and drinks containing menthol.
- Food and drinks with an artificial blue dye.
- Tonic water because of its quinine content.
2) Medication to Avoid
There is a fairly extensive list of drugs that must be avoided by people with G6PD. Both of my children have the condition and carry the list with them wherever they go in case of an emergency. If you have children with G6PD, it is important to make the school aware of their condition.
The drugs to avoid include the following:
- Various NSAIDs
- Anti Malaria Medications
- Sulfa drugs
You can see a full list of the drugs to avoid by clicking on the following link. G6PD Drugs To Avoid.
3) A Healthy Lifestyle and Diet
While simply eating a good, clean diet and maintaining a healthy lifestyle will not actually cure the condition, it can help relieve any symptoms caused by complications like hemolytic anemia.
- Eat plenty of anti-inflammatory foods.
- Get plenty of rest and good quality sleep.
- Try to manage your stress naturally. You can read about natural remedies for stress by clicking this link,
4) Some Tips For Parents
It can be a worry for parents when they learn that their children have a medical condition like G6PD deficiency. It certainly worried us as parents when we found out that our children were both G6PD soon after they were born.
Rest assured that the vast majority of children with the condition lead normal and healthy lives, the biggest concern is to make sure that your children avoid the triggers that can lead to complications like hemolytic anemia.
Make sure that you inform your school, friends, carers and other family members that may be taking care of your child and teach them about the food and medicine that they must avoid.
Your child should always carry a copy if their ‘Avoid List’ of medications and food whether they are on a trip, a camp or simply visiting a relative.