X-linked Dominant Severe Hemolytic Anemia due to G6PD Deficiency

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic problem in red blood cells. The G6PD enzyme helps red cells make NADPH, which protects them from “oxidative” damage. When the enzyme is low, red cells break down too early (hemolysis). Most G6PD deficiency is X-linked recessive (it mainly affects males). However, some families show very severe, ongoing hemolysis (called “chronic nonspherocytic hemolytic anaemia,” or CNSHA). In some girls and women, skewed X-inactivation (lyonization) means the healthy X chromosome is turned off in many cells. That can make the condition look “dominant” in a family because females can also be quite symptomatic. So, “X-linked dominant” here usually means severe forms or female expression due to skewed X-inactivation, not a different gene. ASH Publications+2NCBI+2

G6PD deficiency means the red blood cells do not have enough of the G6PD enzyme. This enzyme makes a chemical called NADPH that protects red blood cells from “oxidative” damage. Without enough NADPH, certain medicines, foods (especially fava beans), infections, or chemicals can damage and burst red cells (hemolysis). This can cause sudden anemia, dark urine, jaundice, tiredness, shortness of breath, and in newborns, severe jaundice. Most people are well between attacks, and the most important step is avoidance of triggers. PMC+1

Safety note you should always keep in mind: before giving primaquine or tafenoquine (antimalarials) or rasburicase (a cancer-care drug for high uric acid), quantitative G6PD testing is required; these drugs are contraindicated in G6PD deficiency because they can cause dangerous hemolysis. CDC+2CDC+2

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Other names

• G6PD deficiency; G6PDD; Glucose-6-phosphate dehydrogenase deficiency.

• Favism susceptibility (hemolysis after eating fava beans).

• Class I G6PD deficiency (the most severe WHO class; often causes CNSHA).

• Congenital nonspherocytic hemolytic anemia due to G6PD.
  These names all describe the same underlying enzyme problem with different clinical patterns or triggers. MedlinePlus+2NCBI+2

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Types

Doctors group G6PD variants by enzyme activity and clinical severity (WHO classes):

1. Class I: severe deficiency with chronic hemolysis (CNSHA).
2. Class II: severe deficiency (<10% activity), usually acute hemolysis when stressed.
3. Class III: moderate (10–60%); hemolysis with stronger triggers.
4. Class IV–V: near-normal or increased activity (usually no problems).
   Severe family patterns (including symptomatic females) usually involve Class I variants. Medscape+2Mayo Clinic Laboratories+2

Red cells need G6PD to keep glutathione in a reduced, protective form. Without enough G6PD, oxidants from infections, drugs, or foods damage hemoglobin, forming Heinz bodies (clumps of damaged hemoglobin). The spleen “bites” these out, making bite cells. Damaged cells then burst in the bloodstream or are removed in the spleen, causing anemia, jaundice, and dark urine. In severe (Class I) forms, this can happen all the time, not just after a trigger. NCBI+2Merck Manuals+2

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Causes (triggers and contributors)

In Class I disease, hemolysis can be chronic; in other classes, the items below usually trigger attacks.

1. Fava beans (favism): natural oxidants (vicine/convicine) damage red cells. PMC

2. Primaquine and other 8-aminoquinoline antimalarials (e.g., tafenoquine): must check G6PD before use because they can cause life-threatening hemolysis. CDC+1

3. Dapsone (for skin/infections): strong oxidant; well-known hemolysis risk. Taylor & Francis Online

4. Nitrofurantoin (for UTIs): reported hemolysis in G6PD deficiency. Baebies

5. Sulfonamide antibiotics (e.g., trimethoprim-sulfamethoxazole): classic oxidant drugs. MSD Manuals

6. Rasburicase (for tumor lysis): contraindicated—generates hydrogen peroxide. FDA Access Data+1

7. Methylene blue (for methemoglobinemia): contraindicated; can worsen hemolysis and may fool pulse-ox readings. FDA Access Data

8. Naphthalene (mothballs): causes oxidative hemolysis; infants are especially vulnerable. CDC+1

9. Severe infections (bacterial or viral): infection-driven oxidants trigger hemolysis. NCBI

10. Acute hepatitis or other inflammatory illnesses: increase oxidant stress. MSD Manuals

11. Certain analgesics in high doses (e.g., salicylates): possible oxidant effect. MSD Manuals

12. Phenazopyridine (urinary analgesic): reported oxidant hemolysis. MSD Manuals

13. Henna/aniline dyes and related oxidants (rare exposures). ATSDR

14. Severe metabolic stress (e.g., diabetic ketoacidosis): amplifies oxidative load. MSD Manuals

15. Nitrates/nitrites (high exposures; food or industrial): oxidant stress. ATSDR

16. Herbal/cultural products containing oxidants (varies by region). MSD Manuals

17. Neonatal life (newborn period): higher bilirubin risk even without clear triggers. AAFP

18. Skewed X-inactivation in females (biologic contributor to severity): fewer normal G6PD-expressing cells → greater hemolysis. MedlinePlus

19. Co-existing red-cell disorders (e.g., unstable hemoglobins): add to oxidative damage. Medscape

20. Some antimalarial regimens without prior testing or monitoring: preventable risk when G6PD status is unknown. CDC

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Common symptoms and signs

1. Sudden tiredness and weakness during attacks (from low hemoglobin).

2. Shortness of breath on exertion; sometimes at rest in severe episodes.

3. Rapid heartbeat (tachycardia) as the body tries to deliver oxygen.

4. Pale skin and inner eyelids (anemia).

5. Yellow eyes/skin (jaundice) from bilirubin buildup.

6. Dark “tea-colored” urine (hemoglobin/hemolysis by-products).

7. Back or abdominal pain during acute hemolysis.

8. Headache, dizziness, lightheadedness.

9. Low energy, poor feeding in babies; may appear sleepy or irritable.

10. Scleral icterus (yellow whites of the eyes).

11. Fever if an infection is the trigger.

12. Enlarged spleen (sometimes, especially in chronic forms).

13. Gallstone-type pain in long-standing hemolysis (pigment stones).

14. Neonatal jaundice that starts early or lasts longer than expected.

15. No symptoms between attacks in most non-Class-I variants.
    These features come from the anemia itself and the oxidative injury to red cells; babies with G6PD deficiency have special risk for significant jaundice, so careful newborn follow-up is essential. MSD Manuals+2AAFP+2

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Diagnostic tests

A) Physical examination

1. General look and vitals: checking heart rate, breathing, and blood pressure; fast heart rate and fast breathing suggest the body is compensating for low oxygen-carrying blood. NCBI

2. Skin and mucosa: paleness (anemia) and yellowing (jaundice) are key bedside clues. MSD Manuals

3. Abdominal exam: feeling for an enlarged spleen (can happen with chronic hemolysis). MSD Manuals

4. Newborn jaundice assessment: early or strong jaundice raises suspicion. AAFP

B) Manual/bedside tests

5. Transcutaneous bilirubin (TcB) scan: a small sensor on the skin estimates bilirubin; helps decide if a baby needs a blood test or treatment. Renaissance School of Medicine

6. Urine dipstick: looks for blood/hemoglobin; dark urine during hemolysis supports the diagnosis. StatPearls

7. Point-of-care hemoglobin (HemoCue): quick finger-stick estimate of anemia. StatPearls

C) Laboratory & pathology tests

8. Complete blood count (CBC): shows low hemoglobin during attacks; often a high reticulocyte count (young red cells) as the marrow responds. Medscape

9. Peripheral blood smear: shows bite cells/blister cells; with a special supravital stain, Heinz bodies appear—hallmarks of oxidant injury in G6PD deficiency. Merck Manuals+1

10. Bilirubin (total/indirect), LDH up, haptoglobin low: standard hemolysis pattern. StatPearls

11. Direct antiglobulin (Coombs) test: usually negative in G6PD deficiency (helps rule out immune causes). StatPearls

12. Urinalysis for hemoglobin/hemosiderin: supports intravascular hemolysis in severe episodes. Medscape

13. Quantitative G6PD enzyme assay: the key test; note it can appear normal during or just after an attack because young red cells have more enzyme—so repeat after recovery (often ~2–12 weeks). Merck Manuals+1

14. Fluorescent spot test (screen): quick field test but misses many heterozygous females; confirm with quantitative or advanced tests. AAFP+1

15. Newborn screening programs (where available): may flag infants early, but female results can be tricky and sometimes need repeat testing. Mayo Clinic Laboratories+1

16. Molecular (DNA) testing: pinpoints the variant and helps with family counseling when the clinical picture is unclear or in females. Wiley Online Library

D) Electro-diagnostic / monitoring

17. ECG (electrocardiogram): checks for fast heart rates or strain from severe anemia; useful in sick patients. NCBI

18. Pulse oximetry: tracks oxygenation; readings can be misleading in some drug contexts (e.g., methylene blue), so interpret with labs and the clinical picture. FDA Access Data

E) Imaging tests

19. Abdominal ultrasound: looks for pigment gallstones in people with long-standing hemolysis or recurrent attacks. SpringerOpen

20. Chest X-ray or echocardiography (selected cases): evaluates heart size/function if anemia is severe or prolonged. (Used case-by-case for complications, not to “diagnose” G6PD deficiency itself.) MSD Manuals

Non-pharmacological treatments (therapies & other measures)

Each item includes a short description, purpose, and “how it works”.

1. Trigger-avoidance education. Learn which drugs/foods/chemicals can trigger hemolysis and keep a personal “avoid list” (carry a card in wallet/phone). Purpose: prevent attacks. Mechanism: removing oxidant stress prevents red cells from breaking. (Examples to avoid: primaquine/tafenoquine, dapsone, nitrofurantoin, rasburicase, methylene blue; fava beans; naphthalene mothballs). Iris+1

2. Check medicines before use. Ask clinicians/pharmacists to screen prescriptions and over-the-counter products; avoid high-risk drugs and use alternatives. Purpose: safe care. Mechanism: avoids oxidant medications. CPIC PGx Files

3. Infection prevention & prompt treatment. Keep vaccines up to date and seek care early for fevers; infections themselves can trigger hemolysis. Purpose: reduce oxidant stress from inflammation. Mechanism: infections raise oxidative stress in red cells. PMC

4. Newborn screening & early jaundice care. If available, screen newborns; monitor for jaundice; use phototherapy early; consider exchange transfusion if severe. Purpose: prevent bilirubin brain injury (kernicterus). Mechanism: phototherapy lowers bilirubin; exchange removes bilirubin and damaged cells. Medscape

5. Hydration during acute episodes. Drink fluids (or IV fluids in hospital) to protect kidneys from pigment load during hemolysis. Purpose: prevent kidney injury. Mechanism: supports urine flow and clears hemoglobin. Medscape

6. Avoid naphthalene (mothballs). Do not store clothes with mothballs; they can trigger hemolysis, especially in infants. Purpose: remove a common household oxidant. Mechanism: naphthalene metabolites oxidize red cell hemoglobin. CPIC PGx Files

7. Avoid henna on infants with G6PD deficiency. Henna (lawsone) has been linked to hemolysis in susceptible babies. Purpose: prevent neonatal crises. Mechanism: lawsone is an oxidant. Semantic Scholar

8. Nutrition rich in natural folate and iron (when appropriate). Eat leafy greens, legumes (except fava beans), meats/fish; check iron status first. Purpose: support new red cell production. Mechanism: folate is essential for erythropoiesis; iron only if deficient. Wadsworth Center

9. Travel planning for malaria areas. Choose G6PD-safe chemoprophylaxis (e.g., atovaquone-proguanil, doxycycline, mefloquine as appropriate) and avoid primaquine/tafenoquine unless G6PD-normal is proven. Purpose: safe malaria prevention. Mechanism: avoids oxidant antimalarials. CDC

10. Medical alert ID. Wear a bracelet or phone alert noting “G6PD deficiency—avoid oxidant drugs.” Purpose: inform emergency teams. Mechanism: prevents inadvertent high-risk drug use. PMC

11. Family counseling. Offer carrier testing to relatives in high-prevalence families; discuss inheritance. Purpose: identify at-risk family members. Mechanism: X-linked transmission education. MedlinePlus

12. Plan for surgery/anesthesia. Ensure the perioperative team avoids oxidant agents (e.g., methylene blue) and monitors hemolysis signs. Purpose: safe procedures. Mechanism: avoids triggers during stress.

13. Avoid smoking and strong oxidant exposures (e.g., certain dyes/chemicals). Purpose: reduce red cell oxidative load. Mechanism: lowers reactive oxygen species.

14. Home jaundice/urine color checks during illness. Look for yellow eyes/skin or tea-colored urine and seek care early. Purpose: early detection. Mechanism: earlier treatment reduces complications.

15. Fever control with G6PD-safe analgesics. Prefer acetaminophen/paracetamol as directed. Purpose: comfort and lower oxidative stress from fever. Mechanism: avoids aspirin at high doses (oxidant risk).

16. Work/school plans. Have a written plan for what to avoid and what to do in an acute episode. Purpose: reduce delays in care. Mechanism: faster trigger withdrawal/support.

17. Regular follow-up in those with chronic hemolysis variants. Occasional labs and folate support if advised. Purpose: maintain steady red cell production. Mechanism: prevents folate depletion.

18. Avoid unnecessary oxidative supplements. Do not self-start “mega-dose” antioxidants; evidence is limited and high doses (e.g., vitamin C) can complicate methemoglobinemia care. Purpose: safety. Mechanism: prevents unpredictable redox effects.

19. Point-of-care G6PD testing programs (where available). Supports safe drug use (e.g., malaria elimination settings). Purpose: identify deficiency before risky drugs. Mechanism: quantitative enzyme testing guides therapy.

20. Document past reactions precisely. Keep dates/drugs/symptoms to help clinicians choose safe options later. Purpose: better risk management. Mechanism: avoids repeat exposures.

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Drug treatments

Important truth: there is no disease-modifying drug for G6PD deficiency itself. Care focuses on stopping the trigger, supporting the patient, and treating the specific problem (e.g., infection, malaria) with G6PD-safe options. Doses below are typical adult references—clinicians individualize by weight, kidney/liver function, pregnancy, and local guidelines.

1. Folic acid — 1 mg by mouth daily (timing: long-term in chronic hemolysis variants or after a severe episode). Purpose: support new RBC production. Mechanism: supplies folate for erythropoiesis. Side effects: very well tolerated.

2. Acetaminophen (paracetamol) — 500–1,000 mg by mouth every 6–8 h (max 3–4 g/day). Purpose: fever/ache control. Mechanism: central COX action; not an oxidant at usual doses. Side effects: liver toxicity if overdosed or with heavy alcohol.

3. Amoxicillin (± clavulanate) — e.g., 500 mg every 8 h or 875/125 mg every 12 h for common bacterial infections. Purpose: treat infections (a hemolysis trigger). Mechanism: β-lactam; generally G6PD-safe. Side effects: GI upset, allergy.

4. Azithromycin — 500 mg on day-1, then 250 mg daily × 4. Purpose: respiratory infections when appropriate. Mechanism: macrolide antibiotic, generally safe. Side effects: GI upset, QT prolongation.

5. Ceftriaxone — 1–2 g IV daily for serious infections. Purpose: sepsis/pneumonia/pyelo as indicated. Mechanism: cephalosporin; not a known oxidant trigger. Side effects: biliary sludging, allergy.

6. Doxycycline (malaria prophylaxis alternative) — 100 mg daily, start 1–2 days before travel, continue during travel and 4 weeks after. Purpose: malaria prevention when G6PD-deficient. Mechanism: antimalarial via apicoplast/protein synthesis; G6PD-safe. Side effects: photosensitivity, GI upset; avoid in pregnancy/young children.

7. Atovaquone-proguanil — 250/100 mg daily, start 1–2 days before travel, continue during travel and 7 days after. Purpose: malaria prophylaxis. Mechanism: mitochondrial electron transport + DHFR inhibition; safe in G6PD deficiency. Side effects: GI upset, mouth ulcers (rare).

8. Mefloquine — 250 mg weekly, start ≥2 weeks before travel, continue during travel and 4 weeks after. Purpose: malaria prophylaxis. Mechanism: heme detox interference; G6PD-safe. Side effects: neuropsychiatric (screen before use).

9. Chloroquine — where sensitive malaria occurs, 300 mg base weekly. Purpose: prophylaxis or treatment per local resistance patterns. Mechanism: lysosomal pH effects in parasite; does not require normal G6PD. Side effects: GI upset, pruritus, retinal toxicity long-term.

10. Artemether-lumefantrine (treatment of malaria) — per CDC tables (e.g., 6 doses over 3 days). Purpose: treat falciparum malaria without using primaquine/tafenoquine. Mechanism: endoperoxide radicals toxic to parasite; G6PD testing not needed. Side effects: headache, dizziness; drug interactions.

11. Ascorbic acid (vitamin C) for methemoglobinemia in G6PD deficiency — varied regimens (e.g., 1–2 g IV every 6–12 h in adults in reports) when methylene blue is contraindicated. Purpose: reduce methemoglobin when MB cannot be given. Mechanism: non-enzymatic reduction of methemoglobin. Side effects: GI upset; caution in G6PD patients generally—use under specialist care.

12. Riboflavin (adjunct, selected cases) — used anecdotally with ascorbate in G6PD-related methemoglobinemia. Purpose/Mechanism: cofactor support for redox enzymes. Side effects: harmless urine discoloration. (Evidence limited; specialist use only.)

13. Stop the offending drug (immediate action rather than a “drug,” but crucial). Purpose: halt hemolysis. Mechanism: removes oxidant source; hemolysis typically settles in 8–14 days. Side effects: none—this is the key first step.

14. Safe pain/fever options besides acetaminophen — ibuprofen at usual doses appears acceptable for many; avoid high-dose aspirin. Purpose: symptom control. Mechanism: non-oxidant dosing. Side effects: GI upset; avoid NSAIDs if kidney injury. (Always confirm locally.)

15. Antibiotics targeting the infection trigger — choose β-lactams/macrolides/tetracyclines when appropriate; avoid dapsone, sulfonamides, nitrofurantoin. Purpose: treat trigger safely. Mechanism: eradicates infection without oxidant drugs. Side effects: class-specific.

16. Avoidance of methylene blue (listed here to emphasize “do not use”): contraindicated in G6PD deficiency for methemoglobinemia; use alternatives. Purpose: prevent severe hemolysis/failure of therapy. Mechanism: MB requires NADPH from G6PD to work. Side effects: hemolysis if given.

17. Antiemetics and GI support (ondansetron 4–8 mg as needed). Purpose: maintain hydration and medicine tolerance during episodes. Mechanism: 5-HT3 blockade; no oxidant effect. Side effects: constipation, headache.

18. Proton-pump inhibitors/H2 blockers if needed (e.g., omeprazole 20 mg daily). Purpose: protect stomach if concurrent meds irritate GI tract. Mechanism: acid suppression. Side effects: headache, GI changes. (No G6PD-specific issues.)

19. Topical agents for skin infections (e.g., mupirocin) when mild. Purpose: limit systemic antibiotics. Mechanism: local antibacterial action. Side effects: local irritation.

20. Transfusion support is a procedure, not a drug, but it often pairs with folate and infection treatment in severe anemia; include here to highlight its frequent role. Purpose: restore oxygen-carrying capacity. Mechanism: supplies healthy RBCs while hemolysis settles. Side effects: transfusion reactions (rare with screening).

Explicitly avoid: primaquine/tafenoquine unless G6PD-normal is confirmed; rasburicase; methylene blue. All three are contraindicated in G6PD deficiency.

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Dietary molecular supplements

Key principle: no supplement cures G6PD deficiency or reliably prevents hemolysis. Good nutrition matters, but evidence for specific pills is limited; most reports advise caution and avoiding mega-doses.

1. Dietary folate (food first; supplement only if prescribed). Leafy greens, beans (not fava), citrus; tablets 1 mg/day only when doctor advises. Function: supports red cell production. Mechanism: cofactor for DNA synthesis in marrow.

2. Vitamin B12 (if deficient). Dose per labs (e.g., 1,000 µg/day orally or injections). Function: erythropoiesis. Mechanism: cofactor for DNA synthesis.

3. Iron (only if iron-deficient). Typical 65 mg elemental iron 1–3×/day under guidance. Function: hemoglobin synthesis. Mechanism: provides iron substrate; do not use if iron stores normal.

4. Balanced multivitamin (standard dose). Function: fills minor dietary gaps. Mechanism: general micronutrient support; avoid “mega” antioxidant doses.

5. Omega-3 (food-based preferred). Function: general cardiovascular support; no direct anti-hemolysis proof. Mechanism: anti-inflammatory lipid effects. Evidence in G6PD: none specific.

6. Vitamin C: avoid high doses in G6PD. Low dietary amounts are fine; high IV/oral doses are not routine and, if used for special problems like methemoglobinemia, must be specialist-guided. Function: antioxidant. Mechanism: redox effects; dosing is tricky.

7. Riboflavin (B2) in diet. Eggs/dairy/leafy greens; supplements rarely needed. Function: redox cofactor. Mechanism: supports glutathione pathways.

8. Avoid unproven herbal products sold as “blood cleansers.” Function/Mechanism claims are untested; most herbs at normal doses have no clear evidence of harm or benefit in G6PD, but some products have caused problems.

9. Hydration as a “molecular” input. Water isn’t a supplement, but staying well hydrated supports kidneys during/after hemolysis. Mechanism: dilute/clear free hemoglobin.

10. Food variety over pills. Emphasize whole foods; supplements only to treat a documented deficiency. Mechanism: balanced nutrients support marrow recovery.

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Immunity-booster / regenerative / stem-cell drugs

1. No approved stem-cell or gene therapy exists for G6PD deficiency (outside research). Management is avoidance and supportive care.

2. Hematopoietic stem-cell transplantation (HSCT): not standard. It’s been discussed only for extremely rare, severe chronic non-spherocytic hemolysis; there are no clinical trials proving benefit just for G6PD.

3. Experimental small molecules (e.g., AG1) are preclinical. Not available for patients.

4. Erythropoietin: not a routine therapy; occasionally used in other anemias; no disease-modifying effect in G6PD deficiency.

5. IV immunoglobulin / steroids: not indicated for G6PD hemolysis (which is oxidative, not autoimmune).

6. Gene-editing concepts are being studied for other anemias; none are available for G6PD patients at this time.

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Procedures/surgeries

1. Phototherapy (newborns). Blue-light treatment converts bilirubin into forms the body can excrete. Why: treat neonatal jaundice safely and quickly.

2. Exchange transfusion (severe neonatal jaundice). Replaces the baby’s blood to rapidly lower bilirubin and remove damaged RBCs. Why: prevent brain injury when bilirubin is very high.

3. Packed red blood cell transfusion (severe anemia in adults/children). Replaces lost oxygen-carrying capacity during acute hemolysis. Why: stabilize oxygen delivery.

4. Temporary dialysis/renal support (rare). Used if acute kidney injury develops after massive hemolysis. Why: support while kidneys recover.

5. Cholecystectomy (gallbladder removal) for pigment gallstones in those with recurrent stones and biliary symptoms. Why: hemolysis increases gallstone risk in some G6PD-deficient people.

Not recommended: Splenectomy is not a standard treatment for G6PD deficiency (unlike some other hemolytic anemias).

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Prevention

1. Know your diagnosis; keep a list of drugs to avoid and share it with every clinician.

2. Test G6PD status before primaquine/tafenoquine or rasburicase are prescribed.

3. Avoid fava beans, mothballs (naphthalene), and henna exposure in infants.

4. Treat infections early; follow vaccine schedules.

5. Use medical alert identification.

6. For travel, pick G6PD-safe malaria prophylaxis per CDC.

7. For newborns with risk factors, monitor jaundice closely and act early.

8. Maintain good hydration during illnesses.

9. Eat a folate-rich, balanced diet; only supplement iron/B12 if deficient.

10. Keep accurate records (dates/drugs/symptoms) after any hemolytic episode.

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When to see a doctor

• Sudden dark “tea-colored” urine, yellow eyes/skin, fast heartbeat, dizziness, or shortness of breath after a new medicine, food, or infection. These are classic hemolysis signs.

• Fever/illness when you have G6PD deficiency, especially if urine darkens or you feel unusually weak.

• Newborn jaundice within the first week of life—seek care urgently if the baby looks yellow or is feeding poorly/sleepy.

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What to eat & what to avoid

1. Avoid fava beans completely (all forms).

2. Enjoy other legumes (lentils/chickpeas/peas) as tolerated—no evidence they trigger hemolysis like fava beans.

3. Folate-rich foods (spinach, broccoli, asparagus, oranges).

4. Iron-rich foods if iron-deficient (red meat, fish, legumes, fortified grains) after your doctor checks iron levels.

5. Hydrate well, especially during illness.

6. Use acetaminophen for pain/fever as directed; avoid high-dose aspirin.

7. Avoid mothball-stored foods/utensils (naphthalene fumes).

8. Be careful with “herbal boosters.” Evidence is limited; avoid products claiming to “oxidize toxins.”

9. Cook meat/fish safely to reduce infection-triggered episodes.

10. Balanced meals over “mega-dose” supplements.

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FAQs

1. Is G6PD deficiency X-linked dominant? No—X-linked recessive. Many females are carriers; some can be symptomatic due to X-inactivation.

2. Why do red cells break in G6PD deficiency? They lack NADPH to control oxidants; oxidant stress (drugs, fava beans, infection) damages hemoglobin and membranes, causing hemolysis.

3. What are the biggest drug dangers? Primaquine, tafenoquine, rasburicase, and methylene blue—all can trigger severe hemolysis; test first (for PQ/TQ) and avoid if deficient.

4. Do all antibiotics cause problems? No. Many are safe (e.g., penicillins, cephalosporins, macrolides, doxycycline). Avoid sulfonamides and nitrofurantoin.

5. What about malaria prevention? Use atovaquone-proguanil, doxycycline, or mefloquine when indicated; avoid primaquine/tafenoquine unless G6PD-normal is proven.

6. Can I ever take methylene blue? No—it’s contraindicated; it doesn’t work without G6PD and can worsen hemolysis.

7. What if I get methemoglobinemia? Doctors can use ascorbic acid and supportive care; sometimes transfusion or hyperbaric oxygen is considered.

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: September 17, 2025.

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