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How do you treat iron storage disease? Essential guide to management

4 min read

According to the Mayo Clinic, the most common treatment for iron storage disease is therapeutic phlebotomy, which involves the regular removal of blood to lower iron levels. So, how do you treat iron storage disease and manage your condition for better long-term health?

Quick Summary

The management of iron storage disease primarily involves therapeutic phlebotomy for hereditary types, while chelation therapy is an alternative for specific cases. This includes dietary modifications and lifelong monitoring to control excess iron and prevent organ damage.

Key Points

  • Phlebotomy is the primary therapy: For hereditary hemochromatosis, regular therapeutic blood removal is the standard, safest, and most effective treatment to reduce excess iron stores.

  • Chelation therapy is an alternative: Medication that binds and removes iron is used when phlebotomy is not an option due to conditions like anemia or heart complications.

  • Dietary changes are supportive: While not a cure, modifying diet to avoid iron supplements, excess alcohol, and raw shellfish helps manage iron levels.

  • Lifelong maintenance is required: After an initial induction phase, long-term, periodic blood removal or chelation is necessary to keep iron levels in a safe range.

  • Early diagnosis is crucial: Initiating treatment before organ damage occurs can prevent serious complications and lead to a normal life expectancy.

  • Iron levels must be monitored: Regular blood tests are essential to track iron levels and adjust treatment frequency and dosages accordingly.

  • Red meat and vitamin C should be limited: High-heme iron foods and supplemental vitamin C can increase iron absorption and should be moderated under a doctor's guidance.

In This Article

Understanding Iron Storage Disease

Iron storage disease, also known as hemochromatosis, is a condition where the body absorbs and stores too much iron. This excess iron can accumulate in vital organs, including the liver, heart, and pancreas, leading to severe and life-threatening complications like liver cirrhosis, heart failure, and diabetes. The primary goal of treatment is to reduce the iron levels in the body to prevent or reverse organ damage and alleviate symptoms such as fatigue, joint pain, and abdominal pain. The treatment approach depends largely on the underlying cause of the iron overload, which can be either hereditary or acquired.

Primary Treatment: Therapeutic Phlebotomy

For hereditary hemochromatosis, therapeutic phlebotomy is the standard and most effective treatment. The procedure is similar to a routine blood donation and serves to lower the body's iron stores by removing iron-rich red blood cells.

The Phlebotomy Process

  1. Induction Phase: Initially, a patient will have blood drawn more frequently, often once or twice a week. This intensive phase continues until the iron levels, typically measured by serum ferritin and transferrin saturation, return to a normal, or near-normal, range. The volume of blood removed is usually around one pint (approx. 470 ml) per session.
  2. Maintenance Phase: Once iron levels are within a safe range, the frequency of phlebotomies is reduced. Most patients transition to a maintenance schedule of blood removal every two to four months, depending on how quickly their iron levels rise. This lifelong therapy is essential to prevent iron from building up again.

Benefits and Monitoring

Therapeutic phlebotomy is a safe and inexpensive treatment that can relieve symptoms like fatigue and prevent serious complications if initiated early. Regular blood tests are necessary throughout both phases to monitor ferritin and hemoglobin levels to avoid complications like anemia.

Alternative Treatment: Chelation Therapy

In cases where therapeutic phlebotomy is not a suitable option, such as for patients with anemia, heart problems, or poor venous access, a doctor may recommend chelation therapy. This treatment uses medication to bind to the excess iron, allowing the body to excrete it through urine or stool.

Types of Chelating Agents

  • Deferoxamine (Desferal®): This agent requires administration via injection, either intravenously or subcutaneously, over several hours. Its parenteral delivery can be a drawback for some patients but is effective for severe iron overload, especially in the heart.
  • Deferasirox (Exjade® or Jadenu®): An oral chelator taken once daily. It is often preferred for its convenience and is particularly effective for removing iron from the liver.
  • Deferiprone (Ferriprox®): Another oral chelator, usually taken three times a day. It is known for its efficacy in removing cardiac iron.

Comparison of Phlebotomy vs. Chelation Therapy

Feature Therapeutic Phlebotomy Chelation Therapy
Application Primary treatment for hereditary hemochromatosis. Alternative for patients intolerant to phlebotomy or with anemia.
Mechanism Removes iron-rich blood cells directly. Uses medication to bind and excrete excess iron.
Method Regular blood draws, similar to blood donation. Oral pills or subcutaneous/intravenous infusions.
Cost Generally simple and inexpensive. Can be expensive, especially for chronic use.
Efficacy Highly effective for hereditary iron overload. Effective for acquired forms, such as transfusion-related overload.
Adherence Requires consistent attendance for appointments. Can be challenging due to daily medication requirements and side effects.
Side Effects Mild fatigue, potential bruising. Can include gastrointestinal upset, skin rashes, and potential organ toxicity.

Dietary and Lifestyle Adjustments

While diet alone cannot treat iron storage disease, it plays a supportive role in management. By understanding which foods promote or inhibit iron absorption, patients can make choices to help regulate their iron levels. These adjustments are in addition to, not in place of, medical treatment.

What to Avoid or Limit

  • Iron Supplements and Iron-Fortified Foods: These should be avoided entirely to prevent increased iron levels. Check labels for added iron in cereals and multivitamins.
  • Vitamin C Supplements: High doses of supplemental vitamin C increase the absorption of non-heme iron. The vitamin C in whole fruits and vegetables does not pose a significant risk, but supplements should be limited.
  • Alcohol: Excessive alcohol consumption can accelerate liver damage, especially in patients with iron overload. Individuals with any degree of liver damage should abstain completely.
  • Raw Fish and Shellfish: People with hemochromatosis are at higher risk of infections from bacteria found in raw shellfish, such as Vibrio vulnificus. Thoroughly cooked seafood is generally safe.
  • Red and Organ Meats: These contain heme iron, which is absorbed more readily by the body. Limiting consumption can help manage iron intake.

Foods That Can Help

  • Polyphenol-Rich Foods: Tannins in black tea and coffee can inhibit iron absorption, so drinking these with meals may be beneficial.
  • Calcium-Rich Foods: Calcium, found in milk and dairy products, can decrease iron absorption when consumed with meals.
  • Foods with Phytates: Phytates in grains, legumes, seeds, and nuts can reduce iron absorption. A plant-rich diet, while not a cure, can have lower iron bioavailability.

Conclusion

The proper treatment for iron storage disease is critical to prevent irreversible organ damage and improve a patient's quality of life. For hereditary forms, therapeutic phlebotomy is the gold standard, effectively and safely removing excess iron over time. For cases where phlebotomy is not viable, chelation therapy offers a proven alternative using oral or infused medication. Supporting these medical treatments with careful dietary management—avoiding supplements and limiting high-iron, high-alcohol intake—is also an important part of long-term care. Lifelong monitoring of iron levels is crucial for all patients to ensure treatment remains effective. By working closely with a healthcare team, those with iron storage disease can manage their condition successfully and maintain a normal life expectancy. For more information, consult the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) website.

Frequently Asked Questions

The main treatment for hereditary hemochromatosis is therapeutic phlebotomy, a procedure that removes blood from the body on a regular basis to reduce iron levels.

Initially, phlebotomy may be done once or twice a week during the induction phase. Once iron levels are normalized, a patient typically enters a maintenance phase, requiring less frequent phlebotomies, usually every two to four months.

Chelation therapy is typically used for patients who cannot undergo phlebotomy, such as those with anemia, heart problems, or poor venous access. It is also the primary treatment for acquired iron overload from regular blood transfusions.

No, diet alone cannot cure or adequately treat iron storage disease. While dietary adjustments are a supportive part of management, they cannot replace medical treatments like phlebotomy or chelation therapy.

It is not necessary or advisable to avoid all iron-containing foods. Instead, you should focus on avoiding iron supplements and fortified foods, limiting red and organ meats, and moderating alcohol intake.

Yes, you should avoid iron supplements and high-dose vitamin C supplements. Vitamin C increases the absorption of non-heme iron, which can worsen iron overload.

Common side effects of therapeutic phlebotomy are generally mild and may include temporary fatigue, bruising at the injection site, or dizziness. These can often be mitigated by resting and staying hydrated.

References

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Medical Disclaimer

This content is for informational purposes only and should not replace professional medical advice.