Hemochromatosis and magnetizer
It is a genetic disease that affects 1 in 200 people (300,000 in France) and which appears around the age of 35 for men and 45 for women. Permanent uncontrolled absorption of iron from the diet overloads the body, primarily the liver, pancreas, heart and bones.
The HFE gene (inherited from mother and father) which encodes a hepcidin regulator is affected by a homozygous C282Y mutation. This disease is distinguished from non-genetic iron overloads: cirrhosis, hepatitis, abdominal obesity with disorder of lipid and glucose metabolism or alcoholism.
The existence of the mutation and an above standard transferrin saturation coefficient (about 30% twice) make the diagnosis.
The severity of the attack is revealed by measuring the circulating ferritin with that stored in the organs, normally less than 300 nanograms / ml. From 1000, there is a risk of organic damage. By a poorly understood mechanism, life expectancy is higher than the rest of the population for subjects under 45 years of age with a rate between 300 and 1000 ng / ml.
If the diagnosis is made later, organ damage is frequent and fatigue is intense: the lesions are irreversible. It is therefore necessary to prevent their aggravation (cirrhosis, diabetes, joint damage or even heart disease). The treatment, other than by magnetizer, is bleeding, effective and necessarily tolerated. It lasts 15 mm weekly until the ferritin drops below 50 ng / ml, then it is spaced out while maintaining this level.
The more advanced the shape, the more difficult it is to standardize. Deferasirox, an iron chelator, is under evaluation. Treatment with a synthetic hepcidin is expected. Joint pain with or without unexplained fatigue should measure the transferrin saturation coefficient.
Iron is an essential trace element for life: it is associated with many enzymatic activities, including the transport of oxygen to the center of red blood cells: 4 g of iron from red blood cells are captured by macrophage cells in the spleen and iron from the diet, 1.5 mg per day is taken up by the intestine to balance possible blood loss. Iron is distributed by transferrin to all cells and stored when ferritin is needed.
The body does not have any means allowing it to eliminate it; its absorption regulation is ensured by hepcidin (hepatic hormone) and by the intestinal cells so that the outputs are equal to the inputs.
The therapeutic bloodletting in danger
Until 2005, bloodletting was done in health centers approved by the EFS (French Blood Establishment). As the blood did not enter the circuit of the classic donation, it was thrown away. For financial reasons, the EFS has gradually closed its centers to refocus on blood donation.
The payment for bleeding is EUR 15.75, but it costs more to perform due to the expensive process of removing waste from the blood. Consequently, over the past fifteen years, care has deteriorated significantly, both in private structures and in public bodies, with the exception of Brittany, which has a large number of patients. She organized herself accordingly. For other regions, since March 2019, people with hemochromatosis can enter the blood donation circuit at fixed EFS collection sites (iron-laden blood poses no risk to recipients). But they must be less than 70 years old, not have developed symptoms of the disease, have had their first 5 bloodletting in a health center and meet the same requirements as other blood donors. Only 30% can claim it.