Editor’s note: This week’s column, usually by Dr. Vince Akimoto, features Dr. Chen Huang, Guam’s only female medical oncologist and a specialist in all types of blood disorders, including hemochromatosis. She also specializes in the diagnosis and treatment of all types of cancer and other benign and malignant tumors. Her clinic is located in the Oka Building in Tamuning and she is a member of the medical staff at the Guam Memorial Hospital.HEMOCHROMATOSIS is a disorder that results from too much iron being absorbed from the gastrointestinal tract and too much iron building up in the body causes subsequent organ damage.
There are two forms of hemochromatosis: primary and secondary.
Primary hemochromatosis is usually caused by a specific genetic problem that causes too much iron to be absorbed. When people with this condition have too much iron in their diet, the extra iron is absorbed in the gastrointestinal tract and builds up in the body tissues, particularly the liver. Primary hemochromatosis is the most common genetic disorder in the United States, affecting an estimated 1 of every 200 to 300 Americans.
Diseases such as thalassemia or sideroblastic anemia can cause secondary or acquired hemochromatosis, especially if the person has received a large number of blood transfusions. Occasionally, it may be seen with hemolytic anemia, chronic alcoholism, and other conditions.
Hemochromatosis affects men more than women. It is particularly common in Caucasians of western European descent. Symptoms are often seen in men between the ages of 30 and 50 and in women over 50, although some people may develop problems by age 20. You have a higher risk of hemochromatosis if someone else in your family has or had the condition. Symptoms include abdominal pain, fatigue, generalized darkening of skin color (often referred to as bronzing), joint pain or arthritis, lack of energy, loss of sexual desire, diabetes and dementia. Liver problems of unknown etiology should also trigger suspicion of hemochromatosis.
Recently, genetic defects have been found in many families with a history of hemochromatosis. Blood tests can be used to look for these genetic changes and confirm the diagnosis of hemochromatosis, as well as determine who may be at high risk of developing the disease.
The goal of treatment is to remove excess iron from the body and treat any organ damage. A procedure called phlebotomy is the best method for removing excess iron from the body. A half-liter of blood is removed from the body each week until the body iron level is normal.
This may require many months or even years to accomplish. After that, less frequent phlebotomy is needed to maintain normal iron levels.
How often you need this procedure depends on your symptoms and your levels of hemoglobin and serum ferritin, and how much iron you take in your diet.
Testosterone hormone therapy can help improve the loss of sexual desire and changes in secondary sexual characteristics. Diabetes, arthritis, liver failure, and heart failure should be treated as appropriate. Hemochromatosis patients should follow a special diet to reduce how much iron is absorbed. The diet prohibits alcohol, especially for patients who have liver damage. You will also be told to avoid iron pills or vitamins containing iron, vitamin supplements, iron cookware, raw seafood, or fortified processed foods such as 100 percent iron breakfast cereals.
Again, treatment of dementia, diabetes, impotence and liver disorder in hemochromatosis patients can be accomplished by judicious phlebotomy (or drawing blood) as this effectively relieves iron accumulation. If you suspect that you or a loved one has hereditary hemochromatosis, you can ask your doctor to check the iron stores in your blood. If this is unusually elevated, excess iron accumulation may be to blame. Further referral to a specialist that deals with blood and cancer problems might be needed to further investigate and treat.