Author: Alex Thompson

12 Things to Avoid if You Have G6PD Deficiency

If you’re not sure whether fava beans are safe to include in your diet, talk with your doctor. To diagnose G6PD deficiency, a healthcare professional will take a blood sample to determine the level of G6PD in your blood. Also, G6PD deficiency can sometimes happen in people who do not have the gene for the condition. This type is caused by other conditions, such as diabetes and hyperaldosteronism (2, 13). This article explains what G6PD is, the impact of G6PD deficiency, and triggers to avoid. People assigned male at birth are more likely to develop noticeable symptoms of G6PD deficiency.

Some foods, medications, and other substances can trigger a hemolytic crisis — a rapid breakdown of red blood cells — in people with G6PD deficiency. In summary, we found only a limited number of publications describing foods or chemicals which are known to increase the risk of haemolysis in G6PD‐deficient individuals. G6PD deficiency (also known as favism or G6PDD) is hereditary, meaning it is passed down in families. It is caused by an abnormality in the activity of red blood cell enzymes. This enzyme deficiency may provoke the sudden destruction of red blood cells and lead to hemolytic anemia with jaundice. This may be triggered by consuming fava beans, certain legumes and medicines.

While anyone can develop G6PD deficiency, the gene is more common in certain groups. People of African, African American, Middle Eastern, Mediterranean, and Asian descent may be more likely to have the gene for G6PD deficiency (1, 5). In this case, the gene causing the deficiency does not come from a parent — the genetic change happens by chance (1).

What to Avoid

Typically, a child has one biological parent with two X chromosomes and one biological parent with one X chromosome and one Y chromosome. A child receives an X chromosome from one parent and either an X or a Y chromosome from the other parent. To understand how X-linked disorders are passed down in families, let’s take a closer look at X and Y chromosomes. Although it’s found in all your cells, G6PD is especially high in the testes and immune cells, and it helps regulate your immune system (3, 7).

In the United States, African American males are most likely to be affected (1). Your doctor will provide a detailed list of medicines and substances that may trigger hemolysis that you should avoid. If you’ve been diagnosed with G6PD deficiency, you may feel better knowing most people with G6PD deficiency never have symptoms.

This means there is a high risk of hemolytic anemia when exposed to triggers, often requiring medical intervention and treatment (3, 5). Therefore, this disorder is transmitted through the faulty genes of mothers (who are usually healthy carriers) to their son (or daughter who again becomes another healthy carrier ). Infants rarely develop serious and obvious G6PD deficiency symptoms. The most common symptom is severe jaundice that appears within an infant’s first 24 hours and/or an infant has a bilirubin level that’s greater than the 95th percentile.

1. So, it’s likely safe for individuals with G6PD deficiency to use exercise to improve their quality of life (21).
2. Methemoglobin (MetHg) forms in red blood cells when the iron in the heme group of hemoglobin molecules undergoes oxidation from the normal ferrous (Fe 2+) state to the ferric (Fe3+) state.
3. Intravenously injected methylene blue is reduced to leucomethylene blue through NADPH-dependent mechanisms.
4. This is the most common genetic enzyme disorder and is typically diagnosed in childhood.

In areas where glucose-6-phosphate dehydrogenase (G6PD or G-6-PD) deficiency (G6PDD) is very common, the doctor will avoid giving G6PD-deficient blood to patients. If toxins or chemicals build up in your body, it may cause breakdown of red blood cells and destroy them, which is known as hemolysis. This process may reduce the level of hemoglobin and red blood cells in your body, which is known as anemia. For example, crises may present as hyperbilirubinemia — a buildup of bilirubin resulting from the breakdown of red blood cells — and severe jaundice, which may require phototherapy (5, 20).

Although most patients remain asymptomatic throughout their life, the clinical manifestations of G6PD deficiency depend on the age of the patient. Some infants may also be affected by jaundice due to G6PD deficiency (G6PDD), which is quite risky. It can be, but most people with G6PD deficiency never have symptoms. G6PD deficiency affects between 400 and 500 million people across the world. G6PD deficiency is more common among people living in sub-Saharan Africa, the Mediterranean and Southeast Asia regions.

What are G6PD deficiency symptoms?

A child with one X and one Y chromosome (usually assigned male at birth) will develop G6PD deficiency if they receive this gene. Most people with G6PD deficiency can manage the condition well by avoiding these triggers, and treatment is not usually necessary. Talk to your healthcare provider if you have G6PD deficiency and need to take antibiotics or malaria medications, as they can tell you what’s safe. This is not a complete list of items you should avoid if you have G6PD deficiency. There are other medications that only cause red cell breakdown if taken in high doses. Fortunately, once you remove and avoid the foods and medicines that are triggering these symptoms in you, hemolysis usually stops within a short period of time.

Left untreated, infants with severe jaundice can develop brain damage. While anyone can develop G6PD deficiency, people assigned male at birth are more likely to have symptoms of the condition. Triggers to be avoided include certain foods, such as fava beans; some medications; and some substances, including henna. Exercise causes oxidative stress, a potential trigger in people with G6PD deficiency. However, studies have not shown a significant difference in oxidative stress in people with and without the condition. This suggests that people with G6PD deficiency can engage in exercise.

However, patients who are deficient in G6PD lack sufficient NADPH to properly reduce methylene blue. Unreduced methylene blue can cause further oxidative damage in the G6PD-deficient patient resulting in hemolysis and even death. Therefore, it is important that patients who are known or suspected to have any degree of G6PD deficiency not receive methylene blue. Alternative therapies for G6PD deficient patients presenting with methemoglobinemia include transfusing packed red blood cells or providing hyperbaric oxygen therapy. Glucose-6 phosphate dehydrogenase (G6PD), also called the housekeeping gene, is an enzyme that protects your red blood cells against oxidative stress and related damage (2, 3, 4, 5).

Is G6PD deficiency a life threatening condition?

Knowing which triggers to avoid is the best way to prevent symptoms. A special situation exists in the management of G6PD patients with methemoglobinemia. Methemoglobin (MetHg) forms in red blood cells when the iron in the heme group of hemoglobin molecules undergoes oxidation from the normal ferrous (Fe 2+) state to the ferric (Fe3+) state. Symptoms of hypoxia begin to develop when the level of methemoglobin reaches 10%, and death can occur when the level reaches greater than 50%. Individuals with G6PD deficiency can continue to exercise to support their quality of life without an increased risk for hemolytic anemia. Exercise can induce oxidative stress, which is a trigger for hemolytic anemia in people with G6PD deficiency (5, 21).

However, many people with this deficiency do not develop symptoms after eating fava beans (1, 10). G6PD is an important enzyme (protein) in your body that protects your red blood cells from damage. However, if you have G6PD deficiency, your body makes a smaller amount of G6PD, which can lead to anemia. If you have mild deficiency symptoms, then transfusion is usually not recommended. In case of a severe hemolytic crisis, your doctor will give you a blood transfusion of either whole blood or packed cells.

More than 400 million people worldwide and about one in 10 African American men in the United States is affected by this disorder. So, it’s likely safe for individuals with G6PD deficiency to use exercise to improve their quality of life (21). Children with G6PD deficiency can lead a typical lifestyle, as long as they avoid any known triggers. The highest prevalence of hemolytic crises in G6PD deficiency is in children ages 1–3 (10). If you have G6PD deficiency, it’s important to talk with your doctor and pharmacist about which medications you should avoid or use with extra caution (12).

G6PD is an inherited form of hemolytic anemia that can cause damage to your red blood cells. There are some medications and foods you should avoid if you have this condition. In some instances, though, G6PD deficiency can cause serious medical conditions such as hemolytic anemia in adults and severe jaundice in newborns.