Favism is an inherited deficiency of an enzyme called glucose-6-phosphate dehydrogenase, or G6PD, the enzyme that protects red blood cells from oxidative stress. When it is deficient, red cells become fragile and break down suddenly on exposure to a trigger such as fava or broad beans, certain medicines and chemicals, or during an acute infection. The result is a hemolytic crisis: pallor, fatigue, breathlessness, yellowing of the eyes and dark urine, which can be severe, particularly in children and newborns. Diagnosis begins with a clinical history linked to a trigger and is supported by a blood count, blood film, reticulocyte count, bilirubin and LDH, followed by measurement of the G6PD enzyme level. One important detail is often overlooked: measuring the enzyme at the height of a hemolytic crisis can give a falsely normal result, because the weakest cells have already broken down and the new cells are rich in enzyme. The test is therefore repeated several weeks after the crisis has settled to confirm the diagnosis, and a negative result taken during a crisis should never be trusted. Treatment is not a medicine that restores the enzyme but a two-part strategy: lifelong avoidance of triggers, and supportive care when hemolysis occurs. A personal list of things to avoid is built for the patient, including fava and broad beans in all their forms, certain medicines, antibiotics and antimalarials specified by the doctor, naphthalene mothballs used to store clothes, and some herbal products, supplements and high-dose vitamin C. During a crisis the trigger is stopped and fluids are given to protect the kidneys, with monitoring of hemoglobin and kidney function, and blood is transfused in severe cases. Newborns need careful monitoring of jaundice, with phototherapy or exchange transfusion when indicated. The limits are clear. No supplement strengthens the enzyme or cures the deficiency, and avoidance is permanent rather than temporary. A widespread myth holds that well-cooked beans are safe for everyone, or that a small amount does no harm; in truth sensitivity differs between individuals and must never be tested by experiment. Iron does not treat favism, and it is given only if tests prove an accompanying iron deficiency, because this is a hemolytic anemia and not an iron-deficiency anemia. The deficiency need not prevent a fully normal life for someone who knows their list and follows it, and every doctor and pharmacist must be told the diagnosis before any new medicine. Your own assessment and personal list are determined by examination and testing with your doctor.
Procedure steps
- 1
Clinical assessment and linking the crisis to a trigger
The doctor asks about timing in detail: what food, medicine, supplement or herb was taken in the previous two to three days, whether there was infection or fever, and whether naphthalene was used in cupboards. Pallor, yellowing of the eyes, spleen size and urine colour are examined, with questions about newborn jaundice and family history.
- 2
Hemolysis tests and enzyme measurement at the right time
A blood count, blood film, reticulocyte count, bilirubin, LDH, haptoglobin and urinalysis are requested, then the G6PD enzyme is measured. If the measurement was taken during the crisis and came back normal, it must be repeated several weeks later, because the result can be falsely normal. Family members may be tested, since the condition is inherited.
- 3
Building the personal avoidance list
The patient and family receive a written list of foods, medicines and substances to avoid, with warnings about naphthalene, henna in newborns, certain supplements and high-dose vitamin C. Carrying an identification card is advised, along with informing every doctor, pharmacist and dentist before any new medicine or antibiotic is dispensed.
- 4
Managing the hemolytic crisis
The trigger is stopped at once and adequate fluids are given to protect the kidneys, with monitoring of hemoglobin, kidney function, and urine colour and volume, alongside treatment of any accompanying infection. In severe hemolysis or a rapid fall in hemoglobin, blood is transfused in hospital and the patient is observed until tests stabilise and signs of breakdown stop.
- 5
Newborn care and family education
Newborns are watched closely for early or severe jaundice and treated with phototherapy or exchange transfusion when needed, because severe jaundice can affect the brain. The family is taught to avoid naphthalene and henna and to watch skin and eye colour, siblings are tested, and genetic counselling is offered when planning further children.
Before the procedure
Bring a list of everything you ate and every medicine, supplement or herbal product you took in the days before symptoms began, and photograph the packaging of any supplement or herbal remedy if possible. Bring previous test results and any old G6PD enzyme level, and say whether it was measured during a crisis. Tell the doctor about severe jaundice at birth in yourself or a family member, and about known favism in relatives. Do not start any vitamin or supplement before consulting your doctor, and never try a small amount of beans as a test. If symptoms are acute, such as dark urine and pallor, do not wait for a clinic appointment but go to the emergency department.
After the procedure
Keep your avoidance list on your phone and with your family, and tell any doctor or pharmacist your diagnosis before any new medicine is dispensed, even a painkiller or antibiotic. Remove naphthalene mothballs from your wardrobes, avoid fava and broad beans in every form, and drink enough fluids especially during fever or infection. See your doctor early with any infection or fever, because infection itself can trigger hemolysis. **Go to the emergency department immediately with tea-coloured or cola-coloured urine, yellowing of the eyes or skin, sudden pallor with severe fatigue, breathlessness or palpitations, abdominal or low back pain, reduced or absent urine, fever with pallor, or confusion.** For newborns, seek care at once with early or deepening jaundice, marked drowsiness or poor feeding.
Expected duration
The assessment visit usually takes 20 to 30 minutes and tests need hours to days, while a hemolytic crisis usually requires monitoring and care lasting about two days to one week depending on severity.
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