Sunday, January 15, 2012

What is Hereditary Hemochromatosis?

If you have heard of Hereditary Hemochromatosis (HH or داء ترسب الأصبغة الدموية), it's probably because you or someone you know has HH! In Lebanon, awareness about this disease is kind of non-existing! HH is a genetic disorder of iron metabolism in which the body accumulates iron. It has a worldwide distribution and is the most common single-gene disorder in the US white population.

What happens exactly?
Genetic mutation causes the body to absorb and store more iron than it usually does, more like 2 to 3 times! Once absorbed, there is no physiological mechanism to get rid of this iron (other than through menstruation, giving birth or bleeding) so the body accumulates this iron leading to iron overload with decades.

Clinical signs
The issue with HH is that the early signs are so vague and non-specific, making it harder to diagnose it earlier. At first, they range form fatigue, weakness, abdominal pain and weight loss because free iron generates the production of free radicals, damaging lipids and DNA! However, as time passes by and iron accumulates in the body >> symptoms become serious: loss of libido, arthritis, amenorrhea, sexual impotence, dyspnea to reach to liver cirrhosis, liver cancer, heart failure, diabetes, etc.!

Phlebotomy is the primary choice of treatment. Excess iron is removed through drawing off of a unit of blood, using the same technique as a blood donation, but with a much higher frequency. This treatment is effective because phlebotomies remove red blood cells that contain iron thus a signal is sent for the stored iron in the tissues and organs to be pulled out to make new red blood cells. It could be done once or twice per week. Now, phlebotomies have been shown to increase the quality of life but lots of phlebotomies can be damaging on the long run. Therefore, watching your diet is crucial!

Dietary Interventions
Limit your Vitamin C!
Watching the iron in your diet can decrease the number of phlebotomies needed and therefore aid in increasing the quality of life in people with HH.
  • Reduce the consumption of red meat because it is high in heme iron, the kind of iron that is easily absorbed.
  • Avoid foods that are high in animal fat because they can bind to free iron and therefore increase free radicals.
  • Limit vitamin C supplements that help the absorption of non-heme iron (iron found in plants, grains and small amount in meat). Limiting meats can be obvious but vitamin C's effect is often forgotten!
  • Consume plenty of fruits and vegetables because they contain lots of fibers and antioxidants which inhibit free radicals. Some of the green veggies also contain oxalates that can bind to iron and decrease its absorption in the intestines.Limit alcohol consumption because it can damage the liver as well as it increases the absorption of iron. 
  • Limit sugary foods and beverages because they enhance iron absorption as well. 
  • Increase nuts, grains, rice and legumes because they are high in fiber, which can decrease iron absorption.
  • People with HH can benefit from having tea and coffee because they contain lots of tannins that can bind to iron, decreasing its absorption! However, people with HH and liver damage should refrain from increasing their tannins consumption. 
  • Avoid raw shellfish, one because they have lots of iron and two because there's a high chance they may contain bacteria (Vibrio vulnificus) that can be fatal to people with high levels of iron.
Moreover, these couple of books can help in decreasing the amount of iron consumed in your diet: The Hemochromatosis Cookbook and Cooking with Less Iron.

However, there's a rumor going on that Rudolph's red nose maybe just a symptom of HH, so hey, his nose is so great that it illuminates the team's path to lead Santa's sleigh.  
HH doesn't limit you.

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