I have just been diagnosed with hemochromatosis. I understand the treatment. What, if any changes, should I make in my diet? Do I avoid or limit my intake of iron-rich foods? If so, how do I monitor my iron intake?

D.S.

Hemochromatos is an inherited condition that leads to an overload of iron in the body. This occurs because of an abnormality in the absorption of iron in the small intestine. In healthy people, the intestine stops absorbing iron when enough of the mineral is already present in the body. However, in patients with hemochromatosis, the iron-absorption switch is constantly turned "on."

Although the disease is present at birth, it usually does not cause symptoms until later in life. This is because large amounts of iron need to be absorbed and accumulate in various organs before health problems surface. Complications typically start when patients are in their 40s. Women tend to experience problems even later than men because they have less iron overload, due to their monthly menstrual loss of iron-rich blood.

Health difficulties caused by the excess iron stores may include the following: cirrhosis, heart failure, diabetes, arthritis, impotence and a bronze discoloration of the skin. The key to treating this disease is early diagnosis and therapy before complications develop.

With availability of screening blood tests, we can measure the amount of iron in the blood to evaluate for hemochromatosis. A patient who is suspected of having this disease then undergoes additional blood tests and possibly a liver biopsy to gauge the amount of excess iron in the body. The gene responsible for this condition has also been identified, and this advance aids in family screening.

The therapy for hemochromatosis involves eliminating the excess iron stores out of the body. This is accomplished by a blood donation, or phlebotomy. After the amount of excess iron has been determined, the amount of blood that needs to be eliminated is determined and regular weekly donations are performed. Periodically, blood tests are done to monitor the counts and iron levels. Once the excess iron has been eliminated, phlebotomy is done less often.

Given the effectiveness of phlebotomy in treating this disease, there is no current recommendation to change the diet in persons with hemochromatosis. However, it is important to maintain close follow-up with your doctor to monitor your therapy.