What dietary recommendations should an adult with hereditary hemochromatosis follow?

Dietary Recommendations for Hemochromatosis

Patients with hemochromatosis should follow a generally healthy diet while strictly avoiding iron and vitamin C supplements, limiting red meat consumption, restricting alcohol intake, and avoiding raw shellfish—but dietary modifications are adjunctive only and cannot replace phlebotomy as the primary treatment. 1

Core Principle: Diet is Adjunctive, Not Primary Treatment

• Dietary modifications should never substitute for iron removal therapy through phlebotomy, which remains the cornerstone of treatment. 1, 2
• The impact of dietary iron restriction is modest (2-4 mg/day) compared to the amount removed by weekly phlebotomy (250 mg/week). 1
• Potential reduction in yearly phlebotomy requirements from dietary interventions ranges between 0.5 and 1.5 liters, depending on individual disease penetrance and adherence. 3

Critical Dietary Restrictions

Absolute Avoidances

Iron Supplements and Fortified Foods:

• Avoid all iron supplements and iron-fortified foods completely. 1, 2
• Check labels on cereals, breads, and processed foods for added iron. 1

Vitamin C Supplementation:

• Avoid all supplemental vitamin C, especially during the iron depletion phase and while undergoing phlebotomy. 1, 4
• Vitamin C is a powerful enhancer of non-heme iron absorption and can accelerate iron mobilization to dangerous levels. 1
• In iron-overloaded patients, vitamin C supplementation has been associated with acute cardiac deterioration, including arrhythmias and cardiomyopathy, due to increased iron availability and free radical generation. 4
• If vitamin C supplementation is absolutely necessary for other medical reasons after iron depletion is achieved, limit to a maximum of 500 mg daily and only after physician discussion. 1

Raw Shellfish:

• Completely avoid raw or undercooked shellfish due to risk of Vibrio vulnificus infection, which can cause serious systemic bacterial infection in iron-overloaded patients. 1
• Avoid direct handling of raw shellfish and wound exposure to seawater in certain geographical regions. 1

Alcohol:

• Restrict alcohol intake significantly, especially during the iron depletion phase. 1
• Patients with iron overload and/or liver abnormalities should avoid or consume very little alcohol. 1
• Patients with cirrhosis must completely abstain from alcohol. 1
• Chronic excess alcohol intake accelerates fibrosis and increases risk of cirrhosis, hepatocellular carcinoma, and liver-related mortality in hemochromatosis. 1
• Even moderate alcohol consumption (12-24 g ethanol/day in men, up to 12 g/day in women) has been associated with increased mortality from cirrhosis. 1

Foods to Limit

Red Meat:

• Limit red meat consumption from mammals, as it contains highly bioavailable heme iron. 1, 2
• Heme iron is well absorbed and its bioavailability is minimally affected by meal composition. 1
• Patients with hemochromatosis show greater dietary iron absorption for both heme and non-heme iron, with particularly weak regulatory feedback for heme iron. 1
• Choose lean white meat from poultry instead, limiting total meat consumption to no more than 200 g per week. 5

Citrus Fruits and Fruit Juices:

• Fruit juices and citrus fruits are best consumed in moderation and not in combination with other foods, as their vitamin C content can enhance iron absorption when consumed with meals. 1
• Consume these items alone, between meals rather than with meals. 1

Beneficial Dietary Strategies

Foods That Inhibit Iron Absorption:

• Drink black tea or coffee with meals, as polyphenols and tannins significantly inhibit non-heme iron absorption. 1, 2
• Certain dietary components like tea have demonstrated inhibitory effects on iron absorption in hemochromatosis patients. 1

Recommended Dietary Pattern:

• Follow a generally healthy diet similar to individuals without hemochromatosis. 1
• Eat at least 600 g of vegetables and fruits per day. 5
• Choose protein-rich pulses and legumes (e.g., kidney beans, soybeans). 5
• Select whole grain products in cereals and bread (avoid iron-enriched grains). 5
• Choose fish 2-4 times weekly (350-500 g per week, with half being fatty fish). 5
• Opt for vegetable oils and low-fat dairy products. 5
• A "veggie-lacto-ovo-poultry-pescetarian" diet appears optimal. 5

Fruit and Vegetable Intake:

• Fruit and vegetable intake does not need to be restricted overall. 1
• Fresh fruits should be eaten between meals to avoid enhancing iron absorption from other foods. 5

Monitoring Considerations During Dietary Management

Vitamin Monitoring:

• Periodically check plasma folate and cobalamin levels, especially in patients requiring numerous phlebotomies. 1
• Administer vitamin supplements if deficiencies develop (excluding iron and vitamin C). 1

Ferritin Targets:

• Maintain serum ferritin between 50-100 μg/L through regular phlebotomy. 1
• Monitor ferritin every 6 months during maintenance phase to ensure levels remain within target range. 1

Common Pitfalls to Avoid

Do Not Delay Phlebotomy:

• Never delay or reduce phlebotomy frequency based on dietary changes alone. 2
• Monitor ferritin levels as scheduled to maintain the therapeutic phlebotomy regimen. 2

Avoid Creating Iron Deficiency:

• The goal is controlled iron stores, not iron depletion or iron deficiency anemia. 2
• Symptomatic iron deficiency can develop in hemochromatosis patients if overtreated. 1

Check All Supplements:

• Do not assume all "healthy" supplements are safe—many multivitamins contain both iron and vitamin C, both of which should be avoided. 2

Avoid Unhealthy Dietary Adjustments:

• Patients sometimes independently implement unhealthy dietary restrictions with the goal of reducing iron intake. 1
• Discuss diet and lifestyle recommendations when hemochromatosis is diagnosed to prevent inappropriate self-restriction. 1

Clinical Context and Evidence Limitations

• Data on the clinical and quality-of-life benefit of dietary modifications in hemochromatosis patients are limited. 1
• No large prospective randomized studies specifically evaluate the effect of dietary interventions in hemochromatosis. 5, 3
• Despite limited quantitative evidence, dietary interventions that modify iron intake and bioavailability may provide additional measures to reduce long-term iron accumulation and the number of required phlebotomies. 1, 3