Treatment

Venesection (the medical removal of blood) is the most effective and the treatment of choice for GH.

Venesection helps to reduce excess iron stores through a repeated process of blood removal - and the body's subsequent replacement of the blood removed. Every time a unit of blood is removed, the body works to replace that blood. To do so requires the components needed for red cell generation - and this includes iron, which is drawn out of the soft tissues, organs and macrophages. By regularly removing units of blood, the body draws down its excess iron stores until the patient is termed "in maintenance". From this point, venesection is required less frequently and may also include or be replaced by blood donation through the donor services.

The standard amount for each venesection is 450 ml (this can range from 200ml - 500ml), this will contain 200mg of iron. Once the removal of iron has begun many symptoms start to reduce in severity.

There are two stages:

1. The Iron Removal Phase (de-ironing) - venesection is undertaken every week or every two weeks until SF is within a range of 50-100mcg/l. Once at this level it leads onto
2. Maintenance phase (where approximately 2-4 venesections are required a year)

With patients who particularly complain of arthropathy, keeping their TS below 50% can be beneficial. However, it is noted to do this can lead to anaemia and should only be undertaken if the patient remains asymptomatic of anaemia.

Above : a patient receiving venesection treatment in a typical East Midlands oncology unit

The HUK provide all their members with a personal diary in which they can record their starting ferritin, transferrin saturation and the amount of blood removed each time. This can keep patients proactive and mentally prepared for their venesections, as calculations can determine an estimate of how many are needed to reach the maintenance phase. Haemochromatosis UK provides paper-based venesection diaries to help patients track their treatment (order free copies online here). 

If patients have been diagnosed with liver cirrhosis, then they will require close follow up in the hepatology unit. They should be offered 6 monthly USS and a blood test to check a tumour marker called Alpha Feta Protein (AFP). They may also require an endoscopy to check for varices.

 

Learning points

• Venesection is the most effective treatment for GH. The De-ironing stage is where the procedure is undertaken weekly or every two weeks to bring the ferritin level to below 50-100mcg/l 

• If patients have arthropathy, keeping TS below 50% can be beneficial towards symptom management

Next steps

Dietary advice