My father has been diagnosed with Primary hemochromatosis. What are the implications?

Primary hemochromatosis is caused by a problem with your genes or by receiving a large number of blood transfusions which boost your iron levels.

Hemochromatosis occurs when too much iron builds up in the liver. This leads to liver enlargement. The disease may lead to the development of diabetes, skin coloring changes, heart problems, arthritis, testicular atrophy, cirrhosis of the liver, liver cancer, hypopituitarism, chronic abdominal pain, severe fatigue, and an increased risk of certain bacterial infections.

Yes like superstar says it is too much iron in the liver. The earlier oyu make a diagnosis and start treatment, the better. If treatment is started before any organs have been affected, diseases such as liver disease, heart disease, arthritis, and diabetes can usually be prevented.
Am sure your doctor has probably advised you that the whole family will have to be screened to check for Hemochromatosis.

Hemochromatosis is a progressive increase in total body iron stores with abnormal iron deposition in multiple organs. Primary hemochromatosis is a genetic disorder, whereas secondary hemochromatosis can be the result of a variety of disorders, most commonly chronic hemolytic anemia.

Doctors can treat hemochromatosis safely and effectively by removing blood from your body on a regular basis, just as if you were donating blood. But in this case, the goal is to reduce your iron levels to normal.

Hereditary hemochromatosis is an adult-onset disorder characterized by inappropriately high iron absorption resulting in progressive iron overload. The organs involved are the liver, heart, pancreas, pituitary, joints, and skin.

So if you father has been diagnosed, then I'd suggest everyone in the family get tested too.
Symptoms usually begin between age 30 years and age 50 years, but they may occur much earlier. Clinical manifestations include the following:
Liver disease
Skin pigmentation
Diabetes mellitus
Arthropathy
Impotence in males
Cardiac enlargement, with or without heart failure or conduction defects
Severe fatigue
Impotence
Arthralgia
Most importantly Cirrhosis being one of the most common disease manifestations of the tissue damage caused by hemochromatosis.

The goal of therapy in patients with iron overload disorders is to remove the iron before it can produce irreversible parenchymal damage. Treatment is simple, inexpensive, and safe. Encourage patients to have weekly therapeutic phlebotomy of 500 mL of whole blood. Most patients require maintenance phlebotomy in which 1 unit of blood is removed every 2-3 months. Therapeutic phlebotomy may improve or even cure some of the manifestations and complications of the disease, such as fatigue, elevated liver enzymes, hepatomegaly, abdominal pain, arthralgias, and hyperpigmentation.

That's because hereditary hemochromatosis causes your body to absorb too much iron from the food you eat. The excess is stored in your organs, especially your liver, heart and pancreas. Sometimes the stored iron damages these organs, leading to life-threatening conditions such as cancer, heart problems and liver disease.

Signs and symptoms of hereditary hemochromatosis usually appear in midlife, although they may occur earlier. The most common complaint is joint pain, but hereditary hemochromatosis can also cause a number of other symptoms, including fatigue, abdominal pain and impotence. Though not always easy to diagnose, hereditary hemochromatosis can be effectively treated by removing blood from your body to lower the level of iron.


You can't prevent hemochromatosis, but if you have the disease, the following measures may help:

Avoid iron supplements and multivitamins containing iron. These can increase your iron levels even more. It's also best to avoid a diet high in iron-rich foods, such as red meat, dried peas and beans, and iron-enriched breads, cereals and pastas.
Avoid taking vitamin C supplements, especially with food. Vitamin C increases absorption of iron. Try to drink vitamin C-rich juices, such as orange juice, between meals. Whole fruits and vegetables that contain vitamin C are fine.
Avoid alcohol. Alcohol and iron combine to cause more liver damage.
Avoid eating raw shellfish. People with hemochromatosis are susceptible to infections, especially those caused by certain bacteria in raw shellfish.