[This article taken from a local newspaper, The Community Common, which hosts a column by Dr. Inoshita. Dr. Inoshita is a hematologist/oncologist at The Portsmouth Cancer Care Center, Portsmouth, Ohio.]

DOCTORS CHATTER

August, 1997

By T. Inoshita, M.D.

For our vacation we flew to New York and, then, to Boston this summer. We were scheduled to take a shuttle flight from New York to Boston. As we did not know that the Delta Shuttle Service used a totally different terminal than that of the regular Delta flights at the La Guardia Airport in New York, we had waited at the wrong terminal until thirty minutes before the scheduled departure time when we finally realized that we were at the wrong terminal. You can imagine what kind of experience we were forced to have, taking a maddening taxi ride from one terminal to the other and fumbling through all the pockets to take out all the metal objects to zoom through the security gate. Fortunately the metal detectors did not detain us and we made it to the plane in the nick of time. Imagine the situation where the metal detectors keep buzzing and you cannot pass through the security gate.

Believe it or not, this could happen. many years ago I read a story in a medical journal about a patient returning from Rochester, Minnesota, where the world-renowned Mayo Clinic is located. The patient, who removed every imaginable metal object from himself, still could not get the sanction of the metal detector to get on a plane. As it turned out, the patient was afflicted with what we call hemochromatosis, a hereditary disease characterized by accumulation of excessive iron in the body.

Iron is an essential element of the body. It is an integral component of hemoglobin, a pigment in the red blood cells, which is involved in the transport of oxygen to various parts of the body. The metabolism of iron is very delicate, however. If we do not take enough iron in our meals or the gut fails to absorb enough iron, we will be in trouble and everyone knows that this results in iron-deficiency anemia. If we take too much iron or the gut absorbs too much iron, on the other hand, we can be loaded with excess iron. This generally does not happen, for the gut is programmed to absorb the exact amount of iron that the body is in need of. There is a disease in which this mechanism is not functioning right, resulting in accumulation of too much iron, and this is called hemochromatosis. As unnecessary iron accumulates in various organs of the body, it can damage these organs eventually. The liver damaged by too much iron can result in cirrhosis. The damage to the pancreas results in diabetes mellitus, and that to the heart in congestive heart failure.

I used to think that this disease is relatively rare, but that does not appear to be the case. It is now known that one in three hundred persons in the United States are affected and one in nine is a carrier. This disease becomes symptomatic most often betweeen 40 and 60 years of age although some may become symptomatic by the age of 20. Some early clinical signs include impotence, premature cessation of periods, irritability, depression, joint pain and fatigue. Screening blood tests are available, and liver biopsy can be ultimately diagnostic. The treatment is to take out blood, a procedure called phlebotomy, just like donating blood for transfusion.

Obviously it is critically important to make this diagnosis before any major organ damages occur, for judicuious use of phlebotomies will prevent the full-blown manifestations of this disease. As this is a hereditary disease, it is also recommended to test family members of the patients afflicted with this disease. Now genetic tests to detect the presence of the genes responsible for this disease are available. This is quite a significant development, for now children destined to develop this disease can be identified even before any clinical signs or abnormal blood tests become apparent. This enables these children to be followed carefully and started on treatment early enough if necessary.