Hemochromatosis
We have written this pamphlet to help you understand and learn to live with the
disorder hemochromatosis.
What is hemochromatosis?
This is an inherited genetic disorder that causes the body to absorb too much
iron. Normally, any iron that is not needed leaves the body through the bowels. In
hemochromatosis, 2-3 times the normal amount of iron is absorbed and stored
throughout the body. The liver is the first organ to store iron and when its storage
capacity is full; the iron is deposited in the heart and other parts in the body. If
untreated, damage to the liver, heart and pancreas can occur. This can result in
liver and heart disease, diabetes, arthritis and impotence.
What causes hemochromatosis?
It is a hereditary disorder that is sometimes called hereditary hemochromatosis.
A person must inherit 2 defective genes, one from each parent to have the
disease. Brothers or sisters of an affected individual have a 25% chance of
getting this disease. It is the most common genetic disorder in the Western
World. About 1 in 300 Canadians of Northern European descent are affected.
What are the symptoms of hemochromatosis?
The liver begins to retain iron at birth, but it may take 20 - 30 years before
symptoms appear.
The symptoms may be:
• Chronic fatigue.
• Swelling in the joints (arthritis), mainly in the knuckles of the middle and index
fingers.
In the later stages of the disease, symptoms can include:
• Depression, mood swings, anger.
• Erectile dysfunction, loss of sex drive.
• Early menopause.
• Yellowing of the skin and eyes (jaundice).
• Distention of the abdomen.
• Diabetes.
• A bronze or gray color to the skin.
• Excessive thirst or hunger.
• Frequent passing of urine.
• Thyroid problems.
How is hemochromatosis diagnosed?
• The level of iron in the blood is measured.
• A liver biopsy can be done to check for the amount of iron
in the liver and for liver damage.
• A new genetic test is used since a gene for
hemochromatosis has been described.
Who should be screened?
As soon as a person is diagnosed with hemochromatosis, the spouse, brothers,
sisters and children over the age of 18 should be checked. Screening is most
important for siblings, since they have 25% chance of inheriting the defective
genes from their parents.
Very young children will not have had time to store enough iron to make an
accurate diagnosis. Their screening should be done when they are 18.
How is hemochromatosis treated?
There is no cure for hemochromatosis so life-long treatment is important.
A needle is placed into a vein allowing some of your blood to drain through a
plastic tube into a bottle. Blood is drawn to remove the excess iron from the body
so it will not build up in the organs. This is called a venesection or phlebotomy.
It is usually done weekly and is similar to a blood donation. Red blood cells
contain iron. The body will draw on the stored iron to replace the lost red blood
cells.
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Treatment may take months, or even up to 2 years, depending on how much iron
has been stored. Once the excess iron has been removed by phlebotomy, the
person must continue to have the amount of iron checked 3-4 times a year.
Phlebotomies may be needed 3-4 times a year for the rest of the person’s life.
Your doctor may arrange for you to have a phlebotomy in the Medical Day Unit
on the 4th Floor, Victoria Building, Victoria General Site, QEII.
How do I get ready for a phlebotomy?
To reduce the risk of complications during the phlebotomy, it is important that
you:
• Drink 8 glasses of fluid (water/milk/juice) the day before and the day of your
treatment.
• Do not drink coffee, tea, or colas the day of your treatment.
• Make sure you eat your breakfast and lunch before your treatment.
• Arrange to have someone bring you to and from your 1st appointment
• If you have cold or flu symptoms, please call the Medical Day Unit to have your
appointment rearranged.
Please follow these instructions before your treatment. If you have any questions
or concerns, please feel free to call the Medical Day Unit at 473-5492.
After your phlebotomy
• The nurse will check your blood pressure and give you
something to drink.
• You will need to stay in the Medical Day Unit for a short time
to make sure you do not become dizzy or faint.
• You may eat and drink as usual.
• Resume your usual activities the next day.
• If you had a Band-Aid™ applied, remove it the day after the phlebotomy.
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Is a special diet needed?
No. Lowering your iron intake to below normal levels does not affect treatment.
However do not eat more than normal amounts of iron. Do not use iron
supplements, multivitamins, mineral preparations, or iron fortified foods. Vitamin
C enhances the absorption of iron when taken with food. It should be avoided or
taken 4 hours before or after a meal. Do not drink alcohol as it enhances the
absorption of iron.
Resource
Canadian Hemochromatosis Society www.cdnhemochromatosis.ca
Toll free in Canada 1-877-BAD-IRON (1-877-223-4766)
If you have any questions, feel free to ask.
Looking for more health information?
Contact your local public library for books, videos, magazine articles and online health information.
For a list of public libraries in Nova Scotia go to HTTP://publiclibraries.ns.ca
Capital Health promotes a smoke-free and scent-free environment.
Please do not use perfumed products. Thank you!
Capital Health, Nova Scotia
www.cdha.nshealth.ca
Reviewed by: Colleen Colville RN, BN, CNE Hematology
Prepared by: Hematology Patient Education Working Group ©
Approved by: LA Fernandez MD, FRCPC, FACP
Illustrations by: LifeART Health Care 1 Images, Copyright © 1994, TechPool Studios Corp. USA
Designed and Printed by: QEII Audio Visual and Printing Departments
WH85-0262 Revised July 2007
The information in this pamphlet is to be updated every 3 years.
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