Treatment

Venesection

The usual, relatively simple, and highly effective treatment for genetic haemochromatosis consists of regular removal of blood. Known as venesection, venesection therapy or phlebotomy, the procedure is the same as for blood donors; every pint (450ml) of blood removed contains about a quarter of a gram of iron.

After venesection, the body then uses some of the excess stored iron to make new red blood cells.

VenesectionInitially, venesection will usually be performed once a week, depending on the degree of iron overload. Treatment may need to be continued at this frequency for up to two years, occasionally longer. During the course of treatment, serum ferritin levels are monitored, indicating the size of the remaining iron stores. Treatment will usually continue until the serum ferritin level indicates minimal iron stores.

This is not the end of the story however; excess iron will continue to be absorbed so the individual will need occasional venesections, referred to as maintenance therapy. Typically this means every 3 to 4 months, for the rest of his or her life. Monitoring of transferrin saturation and serum ferritin is used to assess whether venesection is required more or less often. Generally (but take individual advice) transferrin saturation should be maintained below 50% and the serum ferritin below 50µg/l.

The graph below shows how blood test results may change during treatment. Serum ferritin decreases steadily, but transferrin saturation remains high until iron deficiency occurs, then falls sharply.

Graph

Your doctor or consultant will also monitor levels of haemoglobin during venesection therapy to avoid iron levels falling too rapidly. Venesection therapy may be paused if this is the case.

If you join Haemochromatosis UK we will provide you with a record card so you can keep a note of your venesection dates and ongoing blood test results, and a copy of The Haemochromatosis Handbook.

Chelation

For people who have severe difficulty or other medical problems giving blood, there are drugs that will remove iron from the body. This is called chelation therapy and is normally used to treat iron overload caused by the multiple blood transfusions necessary for the treatment of inherited anaemias.

The most common chelating drug, desferrioxamine, is less effective than venesection and must be administered by continuous infusion, which is not pleasant for the patient. Although oral iron chelators are being developed venesection remains more effective and is therefore the preferred treatment for haemochromatosis.

Erythrocytapheresis

Erythrocytapheresis is a process by which blood is removed, red blood cells are extracted, and the remaining fluids are returned to the patient. Some researchers suggest this is more effective than venesection but this is debatable. The process is much more complex and introduces some risks; it is not generally used in the UK.

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