Health Library

What Is Hereditary Hemochromatosis?

Hereditary hemochromatosis (hee-muh-kro-muh-TOE-sus) is a disease that causes the body to absorb and store more iron than it should. The iron comes from the foods we eat.

With hemochromatosis, iron builds up slowly in the joints and organs such as the liver, heart, brain, pancreas, and lungs. This can cause liver disease, diabetes, heart disease, joint pain, fatigue (extreme tiredness), and other problems as a child grows up. When doctors diagnose hemochromatosis early, these problems can be avoided.

What Are the Signs of Hereditary Hemochromatosis?

Kids don’t usually show signs of having hereditary hemochromatosis. That’s because it can take many years for excess iron to build up in the body and cause symptoms.

What Causes Hereditary Hemochromatosis?

Hereditary hemochromatosis is a genetic condition. For kids to get it, both of their parents must have the gene that causes the condition. But many kids who inherit the gene from their parents do not develop any problems. They may absorb and store extra iron, but not enough to cause health problems.

How Is Hereditary Hemochromatosis Diagnosed?

To diagnose hereditary hemochromatosis, a doctor may recommend:

• Blood tests: Different blood tests help doctors find out if a child has hereditary hemochromatosis. These tests measure how much iron is in the blood.
• Other tests: Sometimes a doctor will need to do special tests to see how much iron is in the liver and how well the liver is working.

How Is Hereditary Hemochromatosis Treated?

It is very rare for a child to need treatment for hereditary hemochromatosis. If it is treated, a doctor may recommend phlebotomy (flih-BOT-uh-mee). This is a process to remove some blood from the body on a regular basis to lower iron levels.

To remove the blood, a phlebotomist inserts a needle into a vein in the arm. It’s the same process as when someone donates blood. The amount of blood removed, and how long this takes, depends on the patient.

What Can Parents Do?

If your child has been diagnosed with hereditary hemochromatosis, you can help slow iron buildup by:

• Avoiding foods with iron. Talk to your child’s doctor or a dietitian to learn more about foods that contain lots of iron and ways to avoid them.
• Limiting vitamin C. Vitamin C makes the body absorb more iron, so make sure any vitamin C supplements your child takes are under 100 milligrams a day.
• Using iron-free vitamins. If your child takes a children’s multivitamin, make sure it is iron-free.

What Else Should I Know?

Make sure your child is vaccinated against hepatitis A and hepatitis B. These diseases can damage the liver. A damaged liver might not be able to manage iron well. This can lead to excess iron in the liver, causing more liver damage.

Kids and teens should not drink alcohol — but sometimes they do. If your child or teen has hereditary hemochromatosis, they need to know that drinking alcohol is harmful. Alcohol makes the disease worse, and can put people who have it at risk for liver disease.