Healthy individuals with the genetic defect can prevent iron overload through regular blood donation. There is ongoing treatment for HH through ‘blood-letting’ or ‘phlebotomy’. This procedure reverses the build-up of excess iron and will help reduce or prevent complications. The frequency of the blood-letting will depend on the level of iron in the body and, initially, may need do be done every week.
To prevent iron stores re-accumulating it is necessary to continue with life long phlebotomy therapy every two to three months to keep the Transferrin saturation at an acceptable level (30%).
Initially treatment consists of regular removal of blood, known as venesection / phlebotomy (weekly). Every pint removed contains a gram of iron. The body then uses excess stored iron to make new red blood cells. Performed once a week until degree of iron overload decreases. Treatment continues until Serum Ferritin level reaches 20ug/l.
Maintenance therapy is usually needed every 3 to 4 months; continuous monitoring of TS and SeFn (maintain TS at below 50% and SeFn below 50ug/l) is important.
PARTNERING WITH THE SOUTH AFRICAN NATIONAL BLOOD SERVICES (SANBS)
The HSSA and the SANBS have agreed policies relating to removal of blood from HH patients. In summary, during the inital de-ironing phase the SANBS will remove blood in terms of the patient\’s medical advisor\’s instructions at cost. During the maintenance phase, providing the bleeds are at intervals longer than 56 days and other normal blood donor criteria are met, the SANBS will remove the blood at no charge.
There is no cure for Haemochromatosis; treatment is ongoing. But, especially when detected early, a series of phlebotomies or “bloodlettings” (venesection is another term used to describe the procedure) can greatly reduce the build-up of excess iron in the body, thus alleviating many of the complications. Venesection allows tissue iron to be mobilised and iron stores will return to normal. However, it will not cure clinical conditions already present, such as diabetes, which is why early diagnosis is vital.
FOLLOWING A TREATMENT PROGRAM
- Fatigue and abdominal pain should decrease
- Cardiomyopathy should improve if cardiac damage was not severe
- Bronzing of skin should fade
- Cirrhosis may improve slightly or stay the same
- Sexual dysfunction and arthritis do not usually improve (arthritis may still appear later despite treatment)
- Hypogonadism and arthropathy do not improve, and hepatoma may develop even years later if the liver disease is far advanced.