What is Haemochromatosis?

Haemochromatosis is one of the most frequent genetic diseases found in white populations – affecting one in 200 people from northern European descent. It is an inherited disorder of iron metabolism and is characterized by the loading of excess iron in the liver, heart and pancreas. This results in iron overload and if left untreated can lead to organ failure, cirrhosis, liver cancer, diabetes mellitus, arthritis, endocrine and sexual dysfunction and decreased life expectancy.

Many people are aware having insufficient iron can be cause problems however few realise that too much iron is also a major concern. The good news is, that if found early enough hereditary haemochromatosis can easily managed, reducing the risk of health complications. 

Normal absorption of iron

Iron is a trace mineral that we obtain from the foods we eat. It is essential for the production of haemoglobin. Haemoglobin is a protein found inside red blood cells that is needed to transport oxygen around the body. Our bodies will usually only absorb the amount of iron we need. However, in people with haemochromatosis too much iron is absorbed and stored in body organs. 

Symptoms of Haemocromotosis 

Symptoms vary from person to person, with some people displaying no symptoms. In some cases, symptoms develop slowly over a period of time, as iron gradually accumulate in the body. The most common symptoms include fatigue, weakness and lethargy and joint pains leading to osteoarthritis. 

Other associated symptoms might include:

• Diabetes mellitus
• Abdominal pain
• Weight loss
• Liver disorders; enlarged, cirrhosis etc.
• Sexual disorders; loss of sex drive in both males and females, impotence in men, absent or scanty menstrual periods and early menopause in women
• Decrease in body hair
• Discoloured or bronzed skin
• Disease of the heart muscle (cardiomyopathy)
• Neurological/psychiatric disorders; mood swings, irritability, depression and impaired memory

Diagnosing Haemochromotosis

Hereditary haemochromatosis is diagnosed by simple blood tests. Usually, a doctor will order a test that measures transferrin saturation and serum ferritin.

If these tests exceed the norm, a genetic test that looks for specific genetic markers of haemochromatosis can be performed.

If you are aware that a close relative has this disorder, it is important to get tested yourself. This test can also determine if you are a carrier of this genetic disorder.

Treatment options

Your doctor will advise you on available treatment options. This often consists of removal of blood by venesection (similar to giving blood). Up to 500mls of blood is removed at regular intervals until iron levels have returned to within normal range. Once normal levels have been achieved, venesections are performed less frequently to maintain results.

Reducing iron in the diet

As people with hemochromatosis absorb iron easily from their foods reducing the amount of iron in the diet.  is often advised. The higher your iron levels are the more careful you need to be about the iron content in foods. Haem iron from animal sources is very well absorbed. It is found in high amounts in fish, liver and especially red meat. These foods can still be consumed as part of a healthy diet but for people with high levels of iron they should consider restricting their consumption to smaller servings or not at all if advised by their doctor. Non-haem iron is not absorbed as efficiently. This is the type found in green leafy vegetables, beans and lentils and usually not contribute to iron levels if eaten in moderation.

Avoid alcohol and raw seafood

Alcohol increases the absorption of iron from food. Excess alcohol consumption also increases the detoxification process needed to be performed by the liver. This very important organ is the most compromised organ in hemochromatosis. 

Raw seafood can contain bacteria which thrive in an iron-rich environment. Though very rare, these bacteria may cause serious complications and even death in individuals with hemochromatosis. To reduce risk avoid eating seafood raw. 

Supplements to avoid

Iron supplement are useful for those who need iron; however it is best to avoid iron in supplements (iron supplements and multivitamins with iron), and iron fortified foods. Supplemental Vitamin C and beta-carotene can also increase the absorption of iron. These are best taken away from foods that contain iron.

Inhibitors of iron absorption

Calcium

Calcium has been shown to have a negative effect on the absorption of iron from both plant and animal sources. Inhibitory effects were seen when calcium was supplemented at doses of 75-300mg and at doses of 165mg from dairy products. This suggests that eating dairy products in moderation and supplementing calcium may assist in reducing the amount of iron absorbed in the diet. 

Magnesium

Magnesium competes with iron for absorption. It is a mineral commonly deficient in Australians. It is available through the diet in dairy products, nuts, seeds and leafy greens; and in supplemental form. 

Polyphenols

Polyphenols occur in various amounts in plant foods and beverages, such as vegetables, fruit, some cereals and legumes, tea and coffee. The inhibiting effect of polyphenols from black and green tea on iron absorption is the strongest. One study found black tea consumed with a meal, reduced the increase in iron storage by one third compared to that of the control group. The researchers suggested that regular tea drinking reduces the frequency of having to donate blood in patients with hemochromatosis. Chili and turmeric are other dietary sources of polyphenols, with chili being shown to reduce iron absorption in one study. Turmeric has the added benefit offering antioxidant, anti-inflammatory and liver protecting actions. 

Milk thistle

One of the most popular liver herbs has been shown to reduce iron absorption in patients with hereditary hemochromatosis. Silybin, the main active constituent of St Mary’s thistle was taken at a dose of 140mg during a vegetarian meal containing iron. The silybin significantly reduced the amount of iron absorbed and has been suggested to be an effective treatment for people with hemochromatosis. It also has the additional benefit of protecting the liver

References

1. http://haemochromatosis.org.au/
2. Hopfner M, et al. Yersinia enterocolitica infection with multiple liver abscesses uncovering a primary hemochromatosis. Scand J Gastroenterol. 2001 Feb;36(2):220-4 https://www.ncbi.nlm.nih.gov/pubmed/11252417
3. Hurrell R, Egli I. Iron bioavailability and dietary reference values. Am J Clin Nutr. 2010 May;91(5):1461S-1467S http://ajcn.nutrition.org/content/91/5/1461S.long
4. Fan FS. Iron deficiency anaemia due to excessive green tea drinking. Clin Case Rep. 2016 Oct 5;4(11):1053-56 https://www.ncbi.nlm.nih.gov/pubmed/27830072
5. Kaltwasser JP, et al. Clinical trial on the effect of regular tea drinking on iron accumulation in genetic haemochromatosis. Gut 1998 Nov;43(5):699-704 https://www.ncbi.nlm.nih.gov/pubmed/9824354
6. Hutchinson C, et al. The iron-chelating potential of silybin in patients with hereditary haemochromatosis. Eur J Clin Nutr. 2010 Oct;64(10):1239-41https://www.ncbi.nlm.nih.gov/pubmed/20628405