Hemochromatosis



Hpathy Ezine, September, 2011 | Print This Post Print This Post |

Hemochromatosis is a metabolic disease of iron metabolism in which the intestine absorbs excessive iron. Detection requires measuring iron levels in the bloodstream. Treatment for Hemochromatosis is relatively simple when the disease is caught early.

Iron is essential to health. It is needed to produce hemoglobin, which transports oxygen. Six percent of Americans (15 percent of young women) need extra iron, and it is frequently advised as a supplement. Healthy people absorb about 10% of the iron in the foods they eat.

Hemochromatosis is a metabolic disease of iron metabolism in which the intestine absorbs excessive iron. Two genes exist for Hemochromatosis. If the patients has one of these genes, his or her body absorbs about 15% of the iron in food, rather than the normal 10%; if the patient has both genes, the body absorbs up to 20%. Throw in a few multivitamins and iron supplements, and significant amounts of iron can be absorbed.

The body lacks the ability to excrete iron through the kidneys as it can for most other substances. Women lose a fair amount of iron through menstrual blood loss. Small amounts of iron are lost in shed skin cells. Despite these small losses of iron, if the body continues to absorb excessive amounts of iron, an overload of iron occurs that is associated with cancers, heart disease, arthritis, chronic fatigue, diabetes, liver damage, and impotence.

Hemochromatosis is one of the most common inherited metabolic diseases. One of the every eight people carries a single gene for Hemochromatosis; one in every 250 carries both genes. Detection requires measuring iron levels in the bloodstream. If these tests are suspicious for iron overload, a blood test for genetic testing can confirm whether the genes are present. A liver biopsy can also confirm the presence of excessive iron. If a patient is diagnosed with Hemochromatosis, all blood relatives should be checked.

Treatment for Hemochromatosis is relatively simple when the disease is caught early. The iron excess must be unloaded; this is accomplished by drawing unit of blood regularly (every one to two weeks) until the patient’s iron levels are normal, then periodically (usually four times a year) to maintain a normal iron count.

Clinical Features of Hemochromatosis

The clinical features of symptomatic Hemochromatosis include liver cirrhosis that might progress into hepatocellular carcinoma, diabetes mellitus from pancreatic islet iron deposition, skin pigmentation, hypogonadotropic hypogonadism, arthritis from chondrocalcinosis and synovial hemosiderosis, cardimyopathy, and arrhythmias. Treatment with phlebotomy ameliorates all manifestations except arthropathy and insulin-dependent diabetes.

Cardiac manifestation of Hemochromatosis includes congestive and restrictive cardimyopathy and arrhythmias. Echocardiogram findings include increases in left ventricular (LV) end-diastolic and end systolic diameters and decrease in ejection fraction without increased wall thickness. Treatment with phlebotomy has been shown to reverse some of these changes. On the other hand, Hemochromatosis does not appear to increase the risk of ischemic heart disease.

Diagnosis of Hemochromatosis

Serum Fe, percent transferrin saturation, and serum ferritin levels are increased. In an otherwise healthy person, a fasting serum transferrin saturation > 50% is abnormal and suggest homozygosity for Hemochromatosis. In most untreated pts with Hemochromatosis, the serum ferritin level is also greatly increased.

If either the percent transferring saturation or the serum ferritin level is abnormal, genetic testing for Hemochromatosis should be performed. All first degree relatives of pts with Hemochromatosis should be tested for the C282Y and H63D mutations.

Liver biopsy may be required in affected individuals to evaluate possible cirrhosis or to quantify tissue iron. An algorithm for evaluating pts with possible Hemochromatosis is shown in. Death in untreated pts results from cardiac failure (30%), cirrhosis (25%), and hepatocellular carcinoma (30%); the latter may develop despite adequate Fe removal.

What is the overall prognosis of patients with Hemochromatosis?

Treated patients with Hemochromatosis who present with mild clinical disease or subclinical disease generally have a normal lifespan if iron stores are maintained at optimal levels. Patients who already have cardiac damage or serve diabetes generally will have only partial reversal of their disease state with phlebotomy therapy and will have decreased survival based on complications. Patients with hepatic cirrhosis also have decreased survival. Although it has been suggested that in some instances phlebotomy therapy may reverse this process, patients with cirrhosis, even if treated with phlebotomy therapy, are at risk for the development of hepatoma.

What is the therapy for Hemochromatosis?

The best therapy for homozygous Hemochromatosis is phlebotomy. Because such patients have marked iron overload, the removal of 1 or even 2 units of blood per week is generally well tolerated unless the patient is severely ill. At least 10 gm of iron is associated with clinical manifestations of the disease. One unit of blood contains about 250 mg of iron. It should be anticipated, therefore, that at least 40 to 80 phlebotomies would be necessary to normalize the total body iron. During phlebotomy therapy, patients are monitored with blood counts to ensure that they have maintained an adequate hemoglobin level.

Serial ferritin values follow the decrease in total body iron stores for individual patients receiving phlebotomy therapy. Once symptomatic patients have a normalized total body iron concentration, a maintenance phlebotomy regimen of two to four phlebotomies per year may be adequate to maintain body iron stores at normal levels. In patients who have Hemochromatosis due to hereditary or acquired anemic conditions associated with transfusion therapy, treatment with an iron chelator is necessary to remove iron. In such individuals it must be ensured that they receive adequate iron chelation therapy, particularly if transfusion is necessary to maintain their hemoglobin in an acceptable range.

Homeopathic treatment of Hemochromatosis - Homeopathy is one of the most popular holistic systems of medicine. The selection of remedy is based upon the theory of individualization and symptoms similarity by using holistic approach. This is the only way through which a state of complete health can be regained by removing all the sign and symptoms from which the patient is suffering. The aim of homeopathy is not only to treat hemochromatosis but to address its underlying cause and individual susceptibility. As far as therapeutic medication is concerned, several remedies are available to cure hemochromatosis that can be selected on the basis of cause, sensations and modalities of the complaints.  For individualized remedy selection and treatment, the patient should consult a qualified homeopathic doctor in person. There are following remedies which are helpful in the treatment of hemochromatosis:

Argentum Nitricum, Arsenic Iod, Calcaria Ars, China, Cuprum Met, Hepar Sulph, Hydrastis, Muriatic Acid, Phosphorous, Arsenic Album, Thuja, Sepia, Sulphur, Bryonia, Rhus Tox, Lycopodium, Nitric Acid and many other medicines.

Reference:

Michael S. Richardson; Health basics; a doctor’s plainspoken advice about how your body works and……….; 2003; 151

Eric J. Topol, Robert M. Califf: Textbook of cardiovascular medicine, Volume 355; 2006; 610

Anthony S. Fauci, Eugene Braunwald, Dennis Kasper, Stephen Hauser, Dan L Longo: Harrison’s Manual of Medicine; 2009; 975

Marie E. Wood, George K. Philips; Hematology/ Oncology secrets; 2003; 63

Ashish Sharma

Add a comment

Your comments & feedback are important to us. If you have a Hpathy.com account, please Login to comment. OR if you are not registered, Create an Hpathy.com account to avoid filling the comment form details every time.

What is 6 + 9 ?
Please leave these two fields as-is:
IMPORTANT! To be able to proceed, you need to solve the following simple math (so we know that you are a human) :-)