Favism: Causes, Symptoms, and Daily Precautions

Favism

What is Favism?
Favism is a condition that occurs due to a deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD).
This enzyme deficiency can lead to hemolytic anemia, especially after the consumption of fava beans or exposure to certain infections, medications, or chemicals.
Factors contributing to Favism include genetic predisposition, as it is an X-linked recessive disorder, meaning it primarily affects males and can be passed down through families.
Daily life precautions for individuals with Favism include avoiding fava beans and other foods that may

Xue Lin, 20~29 year old female. Ask Date: 2003/12/26

Dr. Jian Yingxiu reply Rare Disease

The formal medical term for Favism is "Glucose-6-Phosphate Dehydrogenase Deficiency," abbreviated as G6PD deficiency.
G6PD is an enzyme that assists in the metabolism of glucose in the human body, producing a substance that protects red blood cells.
Patients with Favism lack this enzyme, making their red blood cells susceptible to destruction and hemolysis when exposed to certain chemicals or medications.
In severe cases, this can lead to acute hemolytic anemia.
The incidence of Favism is higher in males than in females.
When specific oxidizing agents enter the body, red blood cells are easily damaged, resulting in hemolysis.
At this point, patients may exhibit symptoms such as pallor, fatigue, poor appetite, jaundice, and yellowing of the whites of the eyes and skin, as well as dark-colored urine.
In severe cases, patients may become comatose and face life-threatening risks.
If a child shows these symptoms, it is crucial to take them to the emergency department promptly to assess the extent of hemolysis and anemia.
Remember to inform the healthcare staff of the history of G6PD deficiency to ensure timely and accurate diagnosis and treatment.
By consistently avoiding substances that can trigger hemolysis and providing appropriate treatment during hemolytic episodes, no long-term complications should arise.

Reply Date: 2003/12/26

More Info

Favism, also known as G6PD deficiency, is a genetic condition that affects the red blood cells. It is particularly prevalent in certain populations, especially those of Mediterranean, African, and Asian descent. The condition is caused by a deficiency in the enzyme glucose-6-phosphate dehydrogenase (G6PD), which plays a crucial role in protecting red blood cells from oxidative damage. When individuals with G6PD deficiency are exposed to certain triggers, they can experience hemolytic anemia, where red blood cells are destroyed faster than they can be produced.

Causes of Favism
Favism is primarily caused by a genetic mutation that leads to a deficiency in the G6PD enzyme. This condition is inherited in an X-linked recessive manner, meaning that it predominantly affects males, while females can be carriers. The deficiency can be triggered by various factors, including:
1. Certain Foods: The most notable trigger is the consumption of fava beans (broad beans), which can lead to a severe reaction in susceptible individuals. Other legumes may also pose a risk.

2. Medications: Certain medications can trigger hemolysis in individuals with G6PD deficiency. These include:
- Sulfonamides (antibiotics)
- Aspirin (in high doses)
- Non-steroidal anti-inflammatory drugs (NSAIDs)
- Some antimalarial drugs (e.g., primaquine)
3. Infections: Viral or bacterial infections can also precipitate hemolytic episodes due to increased oxidative stress on red blood cells.

4. Chemical Exposure: Exposure to certain chemicals, such as naphthalene (found in mothballs), can trigger hemolysis.

Symptoms of Favism
Symptoms of favism typically arise after exposure to a triggering factor and can vary in severity. Common symptoms include:
- Fatigue: Due to anemia, individuals may feel unusually tired or weak.

- Pallor: A noticeable paleness of the skin due to reduced red blood cell count.

- Jaundice: Yellowing of the skin and eyes, indicating an increase in bilirubin levels due to the breakdown of red blood cells.

- Dark Urine: The presence of hemoglobin or bilirubin in the urine can cause it to appear dark.

- Shortness of Breath: This can occur during physical activity due to reduced oxygen-carrying capacity of the blood.

- Rapid Heart Rate: The heart may beat faster to compensate for the reduced oxygen levels in the blood.

Daily Precautions for Individuals with Favism
For individuals diagnosed with G6PD deficiency, it is essential to take specific precautions to avoid triggers and manage the condition effectively:
1. Dietary Restrictions: Avoid fava beans and other legumes that may cause a reaction. It is also advisable to read food labels carefully to avoid hidden sources of these ingredients.

2. Medication Awareness: Always inform healthcare providers about G6PD deficiency before starting any new medication. Carry a medical alert card that indicates the condition and lists medications to avoid.

3. Infection Management: Promptly treat any infections and maintain good hygiene practices to reduce the risk of illness.

4. Avoidance of Chemicals: Stay away from naphthalene and other harmful chemicals that can trigger hemolysis.

5. Regular Monitoring: Regular check-ups with a healthcare provider can help monitor the condition and manage any potential complications.

6. Education: Educate family members, friends, and caregivers about G6PD deficiency to ensure they understand the condition and can help avoid potential triggers.

In conclusion, understanding favism is crucial for individuals affected by G6PD deficiency. By recognizing the causes, symptoms, and necessary precautions, individuals can lead a healthy life while minimizing the risk of hemolytic episodes. If there are any concerns or symptoms arise, it is essential to seek medical advice promptly.

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