Hemochromatosis – How it is Treated

The mainstay of hemochromatosis treatment is bloodletting also known as phlebotomy or venesection. This treatment is life long after diagnosis.

Severe cases of hemochromatosis may need up to and sometimes more than the 10g of iron removed by phlebotomy. In very severe cases phlebotomy may need to initially occur once or twice a week. Each unit of blood is usually 500mls and contains 250mg of iron. So it takes the removal of 2 liters of blood to rid the body of 1g of excess iron.

The ongoing need for phlebotomy is highly variable. Small patients may only tolerate phlebotomies of 250 mls at a time. Maintenance phlebotomies may be in the order of 3 or 4 a year in many cases. Some patients are well maintained by becoming blood donors. The aim is to keep the ferritin level at between 25 to 75 ug/L. Ferritin level is used to monitor the iron level. The need for phlebotomies may change in patients with hemochromatosis.

If a patient stops drinking alcohol they may need fewer phlebotomies to maintain a low ferritin level. Similarly an increase in alcohol consumption may increase the need for phlebotomies. Regular exercise may decrease the need for phlebotomies.

If there is no contraindication to blood donation many blood agencies around the world now accept blood from patients with hemochromatosis. This blood may be actually better blood for persons in need of transfusions as it will contain more young blood cells if the patient is being regularly bled. Sometimes they will not accept blood if the patient has an infectious disease such as hepatitis C and other arrangements have to be made.

Sometimes erythropoietin may need to be used to stimulate the production of new blood cells if there is severe iron overload and the patient is being bled very frequently eg 2 or 3 times a week. Such treatment is uncommon and needs close medical management.

Iron chelators are sometimes used to treat patients with hemochromatosis. Such treatment has to be carefully monitored.

With ongoing venesections it often becomes difficult to access damaged veins. So it is important to preserve the veins from the beginning. Cold packs applied immediately after phlebotomy are helpful.

When preparing for a phlebotomy it is important to be well hydrated before. The patient should drink fluids such as juice or tea or coffee afterwards and most people should arrange for someone else to drive them home.

If hemochromatosis is advanced other problems also need to be treated. These can include arthritis, diabetes, heart problems, impotence and cirrhosis. These problems are treated separately but the hemochromatosis is also included in the overall management. For example phlebotomies usually improve diabetes secondary to hemochromatosis. However the diabetes rarely completely clears. Similarly impotence caused by deposition of iron in the hypothalamus/anterior pituitary and causing testicular failure and impotence is usually not reversed by phlebotomy treatment. With successful deironing or removal of excess iron liver fibrosis may improve. Cirrhosis of the liver usually does not improve.