Should an adult patient with a family history of hematochromatosis (a genetic disorder) follow a special diet to manage their condition?

Dietary Management in Hemochromatosis

Dietary modifications should not replace therapeutic phlebotomy as the primary treatment for hemochromatosis, but specific dietary restrictions are recommended to avoid worsening iron overload. 1

Primary Treatment Principle

• Phlebotomy remains the cornerstone of treatment—dietary changes are adjunctive measures only and cannot substitute for iron removal therapy. 1
• The most recent EASL guidelines (2022) explicitly state that dietary modifications should not substitute for iron removal therapy, though specific restrictions are warranted. 1

Critical Dietary Restrictions

Vitamin C Supplementation (AVOID COMPLETELY)

• All supplemental vitamin C must be avoided, especially during the iron depletion phase and in patients undergoing phlebotomy. 1, 2
• Pharmacological doses of vitamin C accelerate iron mobilization to dangerous levels that can saturate transferrin, causing increased pro-oxidant activity and free radical generation. 1, 2
• This poses serious cardiac risk: patients with iron overload who take vitamin C supplements face increased risk of cardiac arrhythmias, cardiomyopathy, and sudden death due to rapid iron mobilization. 1, 2
• If vitamin C is absolutely necessary for other medical conditions after achieving complete iron depletion, limit to maximum 500 mg daily and only after physician discussion. 2
• Dietary vitamin C from foods is acceptable—the concern is specifically about supplements, not citrus fruits or vegetables consumed as part of normal meals. 2

Iron-Fortified Foods and Supplements

• Avoid all iron supplements and iron-fortified foods (such as fortified cereals, breads, and grains). 1
• This is a strong consensus recommendation from both EASL and AASLD guidelines. 1

Red Meat Consumption

• Limit red meat intake from mammals, as it contains highly bioavailable heme iron (15-40% absorption vs. 1-15% for non-heme iron). 1
• Choose lean white meat from poultry instead, limiting total poultry consumption to no more than 200 g per week. 3
• Avoid processed meats, organ meats (offal), and blood-containing foods entirely. 3

Alcohol Restriction

• Alcohol intake should be restricted during both induction and maintenance phases of treatment. 1
• Alcohol stimulates iron absorption by inhibiting hepcidin expression and can worsen liver disease in patients with hemochromatosis. 4
• Abstaining from alcoholic beverages is recommended, with non-alcoholic alternatives preferred. 3

Raw Shellfish

• Avoid raw shellfish completely due to risk of Vibrio vulnificus infection, which is particularly dangerous in hemochromatosis patients. 1

Beneficial Dietary Strategies

Leverage Iron Absorption Inhibitors

• Drink tea or coffee with meals to reduce iron absorption—polyphenols and tannins in these beverages significantly inhibit non-heme iron uptake. 5, 6
• Consume calcium-rich dairy products with meals, as calcium decreases iron absorption. 4
• Eat foods high in phytic acid (whole grains, legumes) and polyphenols (vegetables), which inhibit iron absorption. 4

Optimize Meal Composition

• Choose a predominantly plant-based diet rich in vegetables, fruits, whole grains, and legumes—these contain non-heme iron with much lower bioavailability. 3
• Aim for at least 600 g of vegetables and fruits daily. 3
• Select fish (350-500 g per week, half being fatty fish) and eggs as primary protein sources instead of red meat. 3
• Choose whole grain breads and cereals (non-iron-fortified), preferably yeast-fermented rather than sourdough. 3

Timing Considerations

• Consume vitamin C-rich foods (citrus fruits, juices) between meals, not with iron-containing meals, to avoid enhancing iron absorption. 6
• Drink tea or coffee with meals, but consume fruit juices separately. 3, 6

Clinical Context and Limitations

The 2011 AASLD guidelines note that dietary iron restriction has limited impact because the amount of iron absorption affected by a low-iron diet (2-4 mg/day) is small compared to the amount removed by phlebotomy (250 mg/week). 1 However, more recent evidence suggests dietary modifications may reduce annual phlebotomy requirements by 0.5-1.5 liters per year, which can be meaningful for patient quality of life and treatment burden. 7, 8

Common Pitfalls to Avoid

• Do not delay or reduce phlebotomy frequency based on dietary changes alone—monitor ferritin levels as scheduled and maintain therapeutic phlebotomy regimen. 1
• Do not create iron deficiency—the goal is controlled iron stores (ferritin 50-100 μg/L), not iron depletion. 1
• Do not assume all "healthy" supplements are safe—many multivitamins contain both iron and vitamin C, both of which should be avoided. 1
• Patients often believe they need aggressive dietary iron restriction, but the primary focus should remain on avoiding supplements and fortified foods while following a balanced, predominantly plant-based diet. 1, 3