Frequently Asked Questions:

TESTING

Question: What tests should be done to confirm if a person has Haemochromatosis?

Answer: Any doctor can organize the initial tests with any diagnostic lab. These should include:

1. Serum Ferritin, which is the form in which excess iron is stored in the body. This is similar to the way the body stores excess energy in the form of fat. Normal ferritin range is 20 - 250 ug/L. The serum ferritin can be falsely elevated at times of illness (such as viral infection) so it is best not to have a test at that time.

2. Iron saturation, which is an indication of the total body iron. Normal iron saturation range is 20 - 45%. The saturation can be increased after a meal containing meat – so it is best to have a fasting blood test.

3. To help interpret the above test it is helpful if a full blood count & liver function test are done at the same time.

If 1 and 2 are significantly above the normal range then the doctor will refer the person to a Haematology specialist for assessment & possible gene test to confirm hereditary Haemochromatosis.

Question: I was recently tested for the Haemochromatosis gene and this shows I have C282Y gene mutation. Is this a cause for concern?

If gene test states you are heterozygous for the HFE (C282Y) gene mutation which means you are a carrier for Haemochromatosis, that is, you probably do not have the disorder but can pass it on through the generations. Some people who are carriers have Haemochromatosis symptoms or a moderately raised serum ferritin but generally you are OK. Your siblings & other close relatives on the side of the family of the parent who passed the gene to you should be checked. Your Haematology specialist will be able to give you more information on being a carrier.

Question: I have recently been diagnosed with Haemochromatosis and I am anxious. My doctor tells me that I am a carrier and all I have to do is to have annual blood tests. He suggests that my brothers and sisters should be tested. Should my children also be tested? As a carrier, how will I be affected?

Answer: Sounds like good advice from your doctor. Yes your brothers & sisters should have a blood test and it would probably be a good idea if the father of your children does as well - if he is also a carrier then there is a 25% chance your children will have Haemochromatosis (full) and 50% chance of them being a carrier, so if he is positive then I would suggest your children should be checked as well, though it is not usually necessary for them to have tests until they are young adults. If their father is OK then there is a 50% chance that your children are also carriers. Check out http://www.ironz.org.nz/what_is.htm for a diagram regarding gene inheritance. It's a good idea for your children to be aware of the situation & to know if they are carriers or not.

My mother and father are both carriers and so is my sister, none of them undergo any treatment (in fact they don't have blood tests for ferritin on a yearly basis either). I have Haemochromatosis and give 500ml of blood every 2 months and have a regular diet & life the same as everyone else. Not that much of an inconvenience as far as I am concerned.

Question: When is NZ going to start nation wide screening for Haemochromatosis?

Answer: Governments all over the world have deemed that screening is too expensive, and that is why we have to rely on testing all family members of those with the gene mutation which is hit and miss.

Question: When should our children be tested for Haemochromatosis?

Answer: First degree relatives including children of individuals with definite Haemochromatosis are tested when convenient...unless there are rarer forms of Haemochromatosis in the family which can affect children (not in your case), the best time is in the 20s. Population screening is recommended at about 30 yrs of age (but we know the children of an affected person will be at least carriers - so I suggest that blood is taken in such children when they are having tests for something else).

Question: I have recently visited a haematologist and was advised that I should have a liver biopsy. Can I have some information on this procedure, its risks and benefits?

Answer: It’s best you discuss this with your Haematology specialist. Normally a liver biopsy is only required if liver damage is suspected. There is a risk of complications with liver biopsies and it is not a procedure undertaken on a whim.

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VENESECTING

Question: I have been diagnosed with Haemochromatosis after six months of feeling unwell and not knowing the cause. My ferritin levels are between 404-563ug/L, my iron saturation is at 36%. I have been referred to the Gastro department but won’t be seen until the end of March. I’ve been told by my doctor that I don’t need to start venesecting until my ferritin is over 1,000ug/L but I feel tired and lethargic. I have donated blood and felt much better but would prefer to start venesecting immediately. What do you suggest?

Answer: I do not know why your ferritin is high, but it sounds as if it may be secondary to some problem with your liver. The ferritin can be "falsely" increased in a number of conditions. Another situation which increases the serum ferritin is a high serum cholesterol.

You mentioned Haemochromatosis, but normally you would be referred to Haematology for that condition. If you have had the gene test, and if you do have full blown Haemochromatosis, I would agree with you that venesections would be appropriate.

In the meantime, until this is sorted out, it would be important to avoid any drugs or alcohol as these could interfere with liver function.

Question: How much iron is removed in each venesection?

Answer: 1 venesection of 500ml removes _ of a gram of iron from the blood. This is the equivalent of 250 days or 750 meals of a normal balanced diet.

Question: I have recently been diagnosed as a carrier of the HFE gene. Although I do not have Haemochromatosis I have had high iron saturation levels in my blood this year. I do not take iron supplements. I often feel tired and wonder if being a carrier is the cause. My doctor tells me not to be concerned about the level of iron in my blood. What do you think?

Answer: It is unusual for anyone to have a high saturation unless they have Haemochromatosis, are on iron medication (and you state that you are not), or are unable to utilise the iron e.g. in situations of bone marrow disease. The saturation level is not an indication however of iron overload, we use the ferritin to measure that. What is your ferritin level?

I presume your gene test has been done and you are negative for the common gene, that you are not anaemic, and that you are not on iron currently (I have seen patients with iron deficiency and high saturation levels who have Haemochromatosis). If your ferritin is currently normal then you should have it measured annually. Should you discover that your ferritin levels are normal there may be another explanation for your tiredness. In your situation we would consider testing for other gene mutations that can cause iron overload in carriers. These tests are often useful not only for you, but your family. Is there a history of Haemochromatosis in the family?

Question: We have a visitor that has Haemochromatosis. Where can he venesect while he is on holiday?

Answer: The blood donation centres offer venesections for those with Haemochromatosis. Check out http://www.nzblood.co.nz/?t=18 for details on where to give blood. Alternatively, the Haematologist looking after your visitor (or your GP) can write to the Haematologist at your local hospital who can arrange venesections. Depending on their nationality, the treatment may be free or there may be a small charge for this service (and any blood tests associated).

Question: My serum ferritn has now dropped to 321ug/L. Is it true that when it drops to around 100ug/L I will only need venesections 2 yearly?

Answer: For most patients the normal method is to have regular blood tests, say every 2 months, and to venesect according to the results of those blood tests. Normal practice when someone is diagnosed with Haemochromatosis is to venesect frequently (weekly, fortnightly or monthly depending on the initial diagnosis level) until the level reaches 100ug/L, or a level recommended by your Haematology specialist, then monitor from that point with blood tests. For example, in my case I presented with a ferritin level of approx 600ug/L at the age of 27, I was venesected fortnightly for approx one year until a ferritin level of 100ug/L was reached and now venesect 2 – 3 monthly depending on the results of blood tests.

Please note that some clinics both in New Zealand and overseas use maintenance levels different from the 100ug/L quoted above.

Question: The hospital is removing 600ml from me every week, I read in most articles that the normal is 500ml's, are they taking to much?. I am 6'2'', weigh 21 stone and am physically active. I have a very good blood flow and normally fill the 600ml bag in four minutes or less.

Answer: 450 - 500mls is a normal amount to remove but a general rule of thumb is no more than 80ml per 10kg of body weight (10% of your blood volume) so with your body weight of 21 stone equating to 134kg 600ml is not excessive.

Question: My husband has been recently diagnosed with Haemochromatosis. We are aware that in order to control this condition he should be venesected however he also has high blood pressure and is not advised to give blood donations. Any recommendations on how he can manage his Haemochromatosis?

Answer: If there are medical issues (potential or absolute) with blood removal, the staff performing the venesections are usually instructed to take less than 10% of the blood volume for the first few venesections to ensure that there are no ill-effects. Treated hypertension is not a contra-indication for blood donation, but may depend on the severity, any complications and sometimes the type of treatment. The more important issue here is not blood donation, but treatment of the affected individual. Blood removed from people with Haemochromatosis who are healthy and fulfill the criteria for blood donation can be used for donation, but not all the blood removed is suitable for that purpose.

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DIET

Question: I have recently been diagnosed with Haemochromatosis and with regular venesections have been able to reduce my ferritin and iron saturation levels to a satisfactory level. I’ve cut down on my intake of iron-rich foods however I am a very active person and I enjoy participating in competitive sports events. I’m concerned that I might be missing some of the nutrients my body needs during training. What dietary advice can you give me?

Answer: We recommend that people eat a balanced diet, including foods that are known to include iron. You should not take any supplements or vitamins that contain iron (there are very few that don’t have added iron & the iron content is high) or foods that have been fortified with iron (some breads, milk & other foods). Alcohol and vitamin C, especially when taken with food, can increase iron uptake in the gut.

Iron uptake from the gut is a slow process, iron builds in a Haemochromatosis patient over time at a rate that can vary depending on the person. You have not given me details of your ferritin or iron saturation levels so I cannot comment on your situation. In my case my ferritin is maintained at around 100 which generally requires bi-monthly venesections & I eat everything from steak to spinach & drink alcohol in moderate quantities. A serum ferritin about my level is more than adequate for any sport, as long as the haemoglobin is normal.

Question: Is it safe to eat shellfish?

Answer: People only need to avoid the raw seafood when they are iron overloaded, so treated patients on maintenance venesections should be OK. Pregnant women, with or without Haemochromatosis should avoid raw seafood because of the danger of contracting listeria. Listeria can be dangerous to the fetus.

Question: What dietary restrictions should be taken by those with Haemochromatosis?

Answer: IRONZ recommends that those who have Haemochromatosis eat a well balanced diet. Most literature suggests people with the condition avoid alcohol, raw shell fish, and dietary supplements containing added iron and foods that are fortified with iron. If you are healthy with a normal liver, and your Haemochromatosis is being treated, alcohol in moderation may be fine – check with your Haematologist.

If you search various websites you will find suggestions that drinking green tea and various other foods will reduce the intake of iron. This is not needed if you are on treatment and have well controller serum ferritin levels. Also, some of this information needs further scientific analysis and it is not the position of this group to recommend self treatment that is not recommended by our medical advisers who are Haematology specialists.

I suggest you check out our page of links for further references, the American organizations have a lot more money and have some quite detailed websites.

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SUPPLEMENTS

Question: My brother takes deer velvet pills. Is deer velvet a safe supplement for a person that has Haemochromatosis?

Answer: Deer velvet contains a small amount of iron. The NZ red deer antler has more iron at the tip than the base ( 462mg/kg vs. 179mg/kg ) with an average of 319mg overall.

If someone takes deer velvet pills, they would have to take one kilogram to equal one iron tablet eg. Ferrogradumet contains 325mg of elemental iron, so a tablet or two would be safe.

More information regarding deer velvet: http://www.positivehealth.com/permit/Articles/Nutrition/sut54.htm

Question: I used to take Bee Pollen capsules, I note these have Iron in them, are they safe to continue with?

Answer: Both IRONZ and medical practitioners do not recommend that people who have Haemochromatosis take supplements that have added iron as iron is stored in the vital organs of the body. In saying this I know that bee pollen is sometimes used to alleviate the symptoms of arthritis and if this is your case you will have to weigh up the options. If you are being venesected regularly to keep a low ferritin level then taking additional iron may not be such a problem.

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MANAGING HAEMOCHROMATOSIS

Question: I have been diagnosed with Haemochromatosis. At what age does damage to body organs occur?

Answer: It is unlikely that damage happens before the age of 30, and is rare in patients with ferritin < 1,000ug/L. The risk of damage directly relates to the degree of iron overload, which is crudely assessed by the serum ferritin. In patients who have a high ferritin, we suggest a liver ultra-sound +/- biopsy (only if the liver functions and/or scan is abnormal), a glucose tolerance test to exclude diabetes, and sometimes special heart studies in those who have very high ferritin. The highest serum ferritin we have seen is 10,000 ug/L.

Question: I have been drinking water from a tank that is rusty. Will the inorganic iron worsen my condition?

Answer: Inorganic iron can be slightly absorbed, however if the water looks clean and tastes reasonable, it is unlikely that there is much iron present in the water. If you have concerns, you could have your water analysed by a chemical laboratory. You can tell if your condition has “worsened” by the results of the serum ferritin tests.

Question: Can Haemochromatosis cause a heart attack?

Answer: Haemochromatosis in situations of very high ferritin levels e.g. greater than 1000ug/L can cause reduced cardiac function which can be diagnosed on an echocardiogram. However, the blood vessels are not usually the main problem in Haemochromatosis, but the heart muscle.

Patients with high ferritin levels from other causes are more likely to have heart attacks than people with normal iron levels - these causes include inflammation and high cholesterol levels - the latter problem can cause a fatty liver and release of ferritin into the blood. Smoking is a higher risk than Haemochromatosis for a heart attack (caused by a blockage in one or more coronary arteries).

Question: Are Haemochromatosis and Iron Overload the same thing?

Answer: Haemochromatosis, if confirmed with genetic testing, often causes iron overload. Iron overload is not necessarily Haemochromatosis as other medical conditions can also cause iron overload.

Question: My son has a 2 year old niece with an internal haemangioma tumour - is this related to the Haemochromatosis?

Answer: No these tumours are usually benign (although I do not have the full details of your grand-niece's situation) and they occur sporadically in the population. They are not known to be related to Haemochromatosis.

What is important though, is that all first degree relatives of your son (parents, aunts, uncles, children, and cousins if their parents have the gene) should have the same test that clinched the diagnosis in his case (usually the HFE gene is analysed for a specific abnormality known as the Cys282Tyr mutation).

Question: Can you still be anaemic if you have Haemochromatosis?

Answer: Yes, individuals with this diagnosis can become anaemic - there are many causes of anaemia. Iron deficiency can also occur so it would pay to have your haemoglobin levels and ferritin checked. Pregnancy/lactation is a time when many women develop iron deficiency.

Question: Do you know anything about chelation treatment?

Answer: Excess iron can usually be removed from most people by a blood removal programme (venesections). Patients who have heart disease have to be managed carefully with this respect, however, we can usually remove just small volumes for these patients at each session. Almost all patients feel better once the iron levels are lower, however symptoms of arthritis do not always improve greatly.

Some patients with Haemochromatosis are unable to have blood removed, mainly those with anaemia from other causes. In this situation we use a drug called desferrioxamine to chelate the iron which has to be given in an injection form. Such treatment is expensive but is funded for selected cases, it can cost from $10,000 to $30,000/year.

Other types of chelation are controversial, although some patients do feel better after the treatment. I suggest you look at a website called www.quackwatch.com for another viewpoint.

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PREGNANCY AND BREASTFEEDING

Question: I am breastfeeding. Are you aware of any multi-vitamin supplements that are safe for women that have Haemochromatosis and are lactating?

Answer: There are a number of multivitamins without iron... just a plain multivitamin tablet would be fine. eg. Multivite 6 (Boots). Likewise minerals...the ideal approach would be to ask your pharmacist and/or read the bottle.

Question: I am breastfeeding and would like to take a supplement. Would it be a problem if I took a supplement that had iron in it? I plan to have another baby. Surely my iron count would lower as a result of a subsequent pregnancy.

Answer: It would not hurt for you to run a ferritin level up to 150ug/L, so if your ferritin is lower than that, it would be fine for you to have iron supplements at a low level. If your ferritin is <50ug/L, I would recommend a course of iron early in your pregnancy.

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