Author: Charles Frank

Medications to Avoid in Patients with G6PD Deficiency Due to Risk of Hemolysis

This means there is a high risk of hemolytic anemia when exposed to triggers, often requiring medical intervention and treatment (3, 5). Because of these factors, people assigned male at birth are more likely to receive a diagnosis of symptomatic G6PD deficiency. On average, people assigned female at birth are more likely to have the G6PD deficiency gene but less likely to develop serious symptoms (1, 12).

Additionally, if only the father carries the gene, sons are unaffected, and daughters remain carriers. The type of mutation inherited will determine the activity level of the G6PD enzyme and the resulting severity of symptoms. Once G6PD deficiency has progressed to hemolytic anemia, however, more aggressive treatment may be required. This sometimes includes oxygen therapy and a blood transfusion to replenish oxygen and red blood cell levels.

In a 2018 review, no other foods found in a typical U.S. diet were linked to G6PD deficiency-related symptoms (11). G6PD deficiency is a manageable condition, and if a person is not exposed to triggers, they may never experience any symptoms. If you or a family member has G6PD deficiency or is a known carrier of the condition and you are thinking of having children, you can talk with your doctor or a genetic counselor.

The numerous reticulocytes present during the hemolytic crisis contain normal levels of G6PD, causing a false negative. If highly suspected, the testing should be repeated when a patient is at baseline status. Females are usually affected if there is a mutation present in both copies of the gene, though in some cases, females with one G6PD mutation can also experience symptoms. Since females have two X chromosomes, males are affected by G6PD deficiency much more frequently than females. Problems may occur if you are exposed to medicines or foods that may harm your blood cells.

How do healthcare providers diagnose G6PD deficiency?

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 ). Hence, men are more likely to suffer from this disorder than women. 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 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. Reticulocytes are immature red blood cells sent from the bone marrow in response to the anemia.

Differential Diagnosis

Knowing and avoiding the triggers is the best way to prevent issues that can arise from G6PD deficiency. Certain medications can also trigger episodes of hemolytic anemia in those with G6PD deficiency. Some of these medications are commonly used in the United States (1, 12). This is the most common genetic enzyme disorder and is typically diagnosed in childhood. An estimated 330 million individuals are affected worldwide — approximately 4.9% of the global population (2, 5, 9, 10, 11). Glutathione is an important antioxidant, a compound that guards your cells against damage from free radicals and oxidative stress.

1. G6PD deficiency (also known as favism or G6PDD) is hereditary, meaning it is passed down in families.
2. Do your best to eat a healthy diet that includes a variety of nutrient-rich foods.
3. Talk with your doctor if you’re concerned about your risk for the condition.
4. A child with one X and one Y chromosome (usually assigned male at birth) will develop G6PD deficiency if they receive this gene.
5. Still, more studies, particularly in humans, are needed to determine whether any supplements would benefit those with this condition.
6. Certain medications can also trigger episodes of hemolytic anemia in those with G6PD deficiency.

The gene for G6PD is located on the X chromosome, making males most susceptible to G6PD deficiency (X-linked disorder). G6PD deficiency protects people from being infected with malaria, so it is more commonly seen in areas with high malaria infection rates, such as Europe, and Asia. In the United States, 10% of African-American males have G6PD deficiency.

Other Substances to Avoid

Some infants may also be affected by jaundice due to G6PD deficiency (G6PDD), which is quite risky. You should be cautious about the consumption of food colored with a reddish orange agent (1-phenylazo-2-naphthol-6-sulphonic acid), which is even banned in many countries. 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 to Avoid

In some instances, though, G6PD deficiency can cause serious medical conditions such as hemolytic anemia in adults and severe jaundice in newborns. Sometimes, anemia symptoms develop very quickly, causing hemolytic crisis symptoms that require immediate medical attention. Other diagnostic tests that may be done include a complete blood count, serum hemoglobin test, and a reticulocyte count. All these tests give information about the red blood cells in the body. G6PD is an enzyme that protects your red blood cells from oxidative stress and damage.

G6PD is an enzyme that protects your red blood cells from harmful substances. Deficiency happens when the gene that drives the G6PD enzyme mutates or changes so the enzyme can’t protect red blood cells. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic condition that can be passed down from parents to their children. It’s a type of hemolytic anemia, meaning oxygen-carrying red blood cells break down too fast (called hemolysis), leading to a lack of red blood cells. G6PD is a genetic disorder that happens when your body doesn’t have enough glucose-6-phosphate dehydrogenase (G6PD) enzyme. G6PD helps red blood cells work and protects them from harmful substances.

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. Other studies suggest that L-cysteine — the precursor to glutathione — could help increase glutathione in G6PD-deficient cells (5, 19). If you’re not sure whether fava beans are safe to include in your diet, talk with your doctor. Qualified healthcare professionals must test for and diagnosis the condition.

To diagnose G6PD deficiency, a healthcare professional will take a blood sample to determine the level of G6PD in your blood. That’s why a deficiency (lack) of the enzyme can cause immune system impairment and an increased risk of infections and non-nutritional anemia (3, 14). 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). 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.