Hemochromatosis: What You Need To Know

The importance of iron

Iron is known as a key mineral for human metabolism, and most of the iron found in the body is found in red blood cells which are responsible for carrying oxygen to different parts of the body.

Without healthy red blood cells, the body will not get enough oxygen. As a result, an individual may become fatigued, which may cause a malfunction in the body’s immune system.

Aside from the above, iron is needed to maintain healthy cells, skin, hair, and nails.

Each person’s need for iron varies, depending on age and gender. It may also depend on the amount of iron that is stored inside the body. When an individual has high iron storage, the body will naturally absorb less iron from food consumed. On the other hand, the body will absorb more iron.

Below are some of the recommended daily allowances of iron in people:
Premenopausal women (ages 19-50) : 18 milligrams
Postmenopausal women: 8 milligrams
Pregnant women: 27 milligrams
Breastfeeding women: 9 milligrams
Men: 8 milligrams

Iron overload

As the saying goes – too much of anything is not always good. When a person absorbs too much iron from the food and drinks they consume, it may lead to an iron overload which causes hemochromatosis.

Extra iron is stored in the liver, heart, pancreas and other organs in the body. When it is left untreated, this can cause serious damage to these organs.

What is Hemochromatosis?

Hemochromatosis is known as an iron overload disorder. In this case, it is the increase of total body iron stores and deposition of iron which may result in organ dysfunction. Healthy people can absorb 8 to 10 percent of the iron they consume through their diet, but those who have hemochromatosis tend to absorb beyond normal.

It may either be primary or secondary, the former being a genetic disorder while the latter a result of various diseases.

Primary hemochromatosis
Primary hemochromatosis is known as an autosomal recessive condition where the HFE gene, the protein product responsible for iron absorption from the gastrointestinal tract, is abnormal.

Most primary hemochromatosis types are due to mutations of the HFE gene. Two common mutations include the C282Y and the H63D. A person who has this condition usually inherits a copy of the defective gene from each parent, while a person who only inherits one copy of the mutated gene is only a carrier who does not experience symptoms.

Primary hemochromatosis has two subtypes:

Juvenile hemochromatosis
This subtype causes symptoms that are similar to primary hemochromatosis, but this usually only affects people between the ages of 15 and 30.

Instead of the HFE gene, this form of hemochromatosis is caused by the mutation of the hemojuvelin gene.

Neonatal hemochromatosis
This form causes a severe increase of iron in an infant’s liver, which may be lethal to the child.

Secondary hemochromatosis
This is rare and is usually due to diseases that cause hemosiderosis, an accumulation of hemosiderin, which occurs in the reticuloendothelial system (RES). This may result in some kinds of anemia such as thalassemia, chronic liver diseases such as chronic hepatitis C infection or alcoholic liver disease.

Anybody who is diagnosed with hemochromatosis must seek further treatment to avoid further iron accumulation in the body.

Who is at higher risk to develop hemochromatosis?

Primary hemochromatosis is common among Caucasians of Northern European descent. It is rare in African Americans, Asian Americans, Hispanics, and American Indians because HFE mutations are usually not the cause of this condition in these populations.

Although both men and women may inherit the gene defects of hemochromatosis, not everyone will develop the symptoms.

Risk factors
Below are some factors that may be the cause for hemochromatosis:

  1. Genetic factors – An individual who possesses two copies of mutated high iron or HFE may have higher chances of having hereditary hemochromatosis.
  2. Gender – As earlier stated, men have a higher risk of developing hemochromatosis than women. Signs and symptoms may begin to emerge between the ages of 40 and 60 years, while women are likely to develop it after experiencing menopause. Women who menstruate regularly are at lower risk of this due to their regular loss of blood which lowers the amount of iron in their body.
  3. Family history – If an individual has a parent, child or sibling with hemochromatosis, they are most likely to develop it.
  4. Ethnicity – People of British, Scandinavian, Dutch, German, Irish and French descent have a higher chance of having the HFE mutation which will lead to hemochromatosis.

Symptoms of hemochromatosis
Most of the time, people at risk of this condition do not experience noticeable symptoms. More so, when symptoms emerge, they vary for every individual.

Some symptoms include:

  • Fatigue
  • Low sex drive
  • Impotence
  • Abdominal pain
  • Low energy
  • Joint pain
  • Weight loss
  • Heart palpitations
  • Shortness of breath
  • Irritability
  • Depression
  • Loss of body hair

In more serious cases, an individual’s skin may take a bronze or yellowish color. Due to organ failure, a person may become confused, frequently bleed, have an accumulation of fluid in the legs and abdomen, have black or bloody stools and have jaundice.

Checking for hemochromatosis

When the above symptoms come up, it may turn out to be similar to some other health conditions, making it challenging to diagnose. To detect this condition, several tests are necessary for confirmation.

Medical and family history
A health care provider may recommend an individual to take a medical and family history to diagnose the condition. They may look into clues that may lead to hemochromatosis. Some may include arthritis in family members or mysterious liver diseases.

Physical exam
A physical exam allows a medical professional to examine an individual’s body and listen to bodily sounds that may indicate any clues for hemochromatosis.

Blood testing
Iron levels can be checked through a blood test. There are a few common blood tests that may help in detecting hemochromatosis:

  • Serum Iron – This test checks the current level of iron in an individual’s bloodstream. It checks for the quantity of iron in the blood.
  • Unsaturated/Unbound Iron-Binding Capacity (UIBC) – This checks for the free iron in the blood, those that are unbound and cause oxidative damage.
  • Total Iron-Binding Capacity (TIBC) – This measures the available capacity of the blood to bind iron.
  • Ferritin Level – This lab test monitors the state of an individual’s condition and the progress of the treatment combating hemochromatosis. This will alert an individual regarding the burden of iron in the cells.
  • Transferrin Saturation – This helps find out how much iron is bound to the blood protein transferrin. High transferrin saturation is connected to hemochromatosis.

DNA testing
A doctor may recommend a DNA test to check for any mutations in the individual’s HFE and hemojuvelin genes.

Liver biopsy
This is the process of removing a piece of tissue from the liver of an individual for pathology lab testing. In this way, the presence of iron may be detected as well as check for liver damage. As the liver is the main storage of iron, it is usually the first organ that is damaged due to iron increase.

Imaging methods
Apart from those stated above, because the disease is usually silent, it can be found through imaging methods – such as MR imaging.

To detect iron overload, computed tomography (CT) and MR imaging are utilized. These types of methods may reveal the diagnosis of iron overload.

Treatment
To avoid the serious consequences of hemochromatosis, an individual must seek medical attention and treatment as soon as possible. However, when serious damage and complications occur to the individual, this condition may be long-lasting.

Normally, to treat hemochromatosis, periodic removal of blood known as phlebotomy is necessary. This may be as frequent as once a week until an individual’s iron levels return to normal. The phlebotomy must be done every two to four months to keep the iron levels stable. Phlebotomy may also help in improving issues like fatigue and abdominal pain.

To stay in check, a medical professional may monitor the individual’s blood count to ensure that blood removal is not excessive. Aside from that, some modifications in the diet may be necessary.

Complications
When left untreated, the individual may pose higher risks for the following conditions:

  • Arthritis
  • Cirrhosis of the liver
  • Diabetes
  • Enlargement of the liver
  • Heart disease
  • Heart failure
  • Endocrine problems
  • Pancreatitis

Managing symptoms and complications
Unfortunately, hemochromatosis is a lifelong condition. Without treatment, the iron buildup can lead to serious health problems as stated above which can threaten an individual’s life.

The best way to manage this is to keep a close eye on what the individual is consuming.

Hemochromatosis diet

An individual experiencing this condition must consider consuming food that is low in iron but must also ensure that they do not restrict themselves too much. They must practice a well-rounded, balanced and nutritional diet.

It is common for people who are diagnosed with hemochromatosis to think that they must stay away from food that is rich in iron. They may feel anxious to think that they may never be able to consume the same food again.

Restrictions
It is important to take note that the greater the level of iron overload there is in an individual’s body, the more it is important for them to be more cautious with the food they consume.

Before further expounding on the following, it is important to highlight some of the most important restrictions one must keep in mind:

  1. Avoiding iron supplements – Supplements contain higher levels of iron than food does. Often, supplemental iron is designed to be absorbed more by the body, which is what people facing hemochromatosis must avoid.
  2. Avoiding raw seafood – A bacteria known as Vibrio Vulnificus is present in raw seafood which grows when it is in an environment that is high in iron. When it mixes with hemochromatosis, it may lead to very serious health complications and though rare, may even cause death.
  3. Reducing or avoiding alcoholic beverages. – The liver is the most sensitive organ to iron overload and alcohol may compromise its health. Additionally, alcohol tends to enhance the iron absorption of food, which may cause serious damage.

For those facing more severe symptoms, it is important to reduce the following iron-rich foods:

  1. Non-Heme Iron-containing food – Non-Heme is derived from plant-based food.
  2. Heme Iron-containing food – Heme Iron is derived from animal food such as meat and seafood. This is more bioavailable than nonheme iron, so the body absorbs this better.
  3. Iron Fortified foods.

A combination of the following vitamins and minerals may enhance the effect of iron absorption. Thus, it is important to avoid these when consuming anything high in iron:

  • Vitamin C
  • Beta Carotene

The following nutrients help in reducing iron absorption from meals. Anybody who has hemochromatosis is encouraged to add this to their diets:

  • Turmeric
  • Milk thistle
  • Green tea
  • Calcium
  • Polyphenols and tannins
  • Oxalates and phytates

Food to eat

Fruits and vegetables
Aside from the above, individuals with hemochromatosis are encouraged to eat more fruits and vegetables. Antioxidants are important in protecting the body from the damage that can be caused by excessive iron.

Some vegetables are high in iron, which some may believe they must stay away from. However, as earlier mentioned, plant-based food is not as easily absorbed by the body, making vegetables a good idea.

Grains and legumes
Phytic acid is a unique natural substance that can be found in plant seeds. This substance is known to impair the absorption of iron, zinc, and calcium.

Grains and legumes are known to contain phytic acid, This helps an individual keep their body from over absorbing iron from food.

Lean protein
It is important to have protein in a healthy diet. Although a lot of meat contains iron, this does not necessarily mean one has to completely cut it out of their diet. Instead, it would be wise to plan meals around a protein that is lower in iron – this includes turkey, chicken, tuna and deli meat.

Eggs
Egg yolks contain a phosphoprotein called phosvitin, which inhibits the absorption of iron. It has been studied that rats that were fed with yolk protein absorbed less iron than those that were fed with soy protein.

Tea and coffee
Tea and coffee contain polyphenolic substances known as tannins, also known as tannic acid. This helps inhibit the absorption of iron.

Food to avoid

Excess red meat
As earlier mentioned, meat is important for a well-balanced diet. Because red meat is a source of heme iron, the body will be able to absorb it more easily. Eating this excessively may cause hemochromatosis to worsen, so it is important to keep this in moderation.

Raw seafood
Although seafood does not contain high levels of iron, as earlier mentioned, it contains vibrio vulnificus, a type of bacteria that infects shellfish in coastal waters, which also happens to contain high amounts of iron.

Vitamin A and C – rich food
Vitamin C and vitamin A are known to enhance the absorption of iron in the body. They are usually added as part of healthy diets, but for those who have hemochromatosis, it is important to consume these moderately.

Fortified food
Fortified foods are rich with nutrients such as calcium, zinc, and iron. Hence, this is an iron-rich source and may cause an increase in iron in the blood.

Excessive alcohol
Excessive alcohol intake will eventually lead to a damaged liver. Because iron overload can damage the liver as well, drinking alcohol can worsen the situation. Unless one has a serious liver condition, it is not necessary to completely cut alcohol out, but it is important to keep this in moderation.

Supplements

Because most supplements are designed to enhance the nutrients in the body, it is not advisable for individuals who have hemochromatosis. The following must be avoided:

  • Iron – This is a given, and will put an individual at high risk of gaining more iron in the body.
  • Vitamin C – This is usually taken by those who have iron-deficiency and acts to add more iron in an individual’s body. Instead of supplements, those with hemochromatosis can get their vitamin C through fruits and vegetables.
  • Multivitamins – These may contain high amounts of iron, vitamin C and a variety of nutrients that may enhance iron absorption.

Recipe

Just because one has hemochromatosis does not mean they do not get to enjoy delicious food. Below is a delicious recipe that those with the condition may enjoy:

Vegetable Quiche

  • 1 tbsp. olive oil
  • 1/2 cup green onion, chopped
  • 1/2 cup onion, chopped
  • 1/2 cup zucchini, chopped
  • 1 cup spinach
  • 3 eggs, beaten
  • 1/2 cup milk
  • 1 1/2 cups shredded cheese
  • 1 deep dish pie crust, precooked

Instructions: Preheat the oven at 350 degrees Fahrenheit. In a large skillet, add the green onion, onion, and zucchini and cook in olive oil for 5 minutes. Add the spinach and continue to cook for another 2 minutes. Set the vegetables aside as this will be used as the crust.

In a separate bowl, whisk the eggs, milk, half of the cheese, salt, and pepper. Pour this mixture into the pie crust and top with the remaining cheese. Bake for another 40-45 minutes.

Reminders

Although thorough research is highly accessible online, it is always important that an individual who believes they may be suffering from hemochromatosis must consult a medical professional as well as a nutritionist who can help design an ideal diet for them.