Definition
Typically hereditary, hemochromatosis identifies a pathology in which iron accumulates excessively in the tissues of the organism: we are talking about a rather serious disease, since iron, depositing itself in the various organs, can cause serious damage, especially in the liver. cardiac, pancreatic and articular. Since it is often a genetically transmitted disease, haemocormatosis cannot be cured definitively, although some drugs and some dietary-behavioral rules can reduce the symptoms.
Causes
It is estimated that the hereditary variant of hemochromatosis is the most frequent: the alteration of a gene involved in the regulation of the amount of iron absorbed with food is, in this case, the triggering cause. In other cases, the exaggerated accumulation of iron can be favored by pathologies, such as: sideroblastic anemia, alcoholic liver disease, abuse of vitamin C and iron with the diet, thalassemia.
- Risk factors: male, over 50 years of age
Normal dose of iron absorbed by a healthy individual: 1-2 g / day
Absorbed dose of iron in case of hemochromatosis: 4-6 g / day → iron deposits in the body reach up to 20-30 grams
Symptoms
In the past, hemochromatosis was defined "bronzial diabetes", in reference to the bronze color assumed by the skin of an individual affected by this disease: the chromatic alteration of the skin is one of the very first symptoms with which hemochromatosis begins; other characteristic signs are added: joint pain, hepatomegaly, hypogonadism, drowsiness, fatigue.
- Complications: liver cancer, cirrhosis, diabetes mellitus, fibrosis
The information on Hemochromatosis - Hemochromatosis Treatment Drugs is not intended to replace the direct relationship between health professional and patient. Always consult your doctor and / or specialist before taking Hemochromatosis - Hemochromatosis Treatment Drugs.
Medicines
The "hemochromatosis" must be treated in time, even if, unfortunately, the disease is not always diagnosed early; the onset of symptoms, as well as the intensity with which they occur, is rather gradual and is heavily influenced by the amount of excess iron absorbed by the body. According to these words, it is understandable how the removal of the surplus of iron - before the hemochromatosis degenerates - is indispensable to say the least to escape the complications of the disease.
The treatment of choice, even in the genetic form of hemochromatosis, is therefore the removal of excess iron: this procedure, called phlebotomy, consists in the regular extraction of blood (similar to blood donation), in order to restore the normal level of plasma iron. It is not possible to report an approximate amount of blood taken: this element depends on the age of the patient, the concentration of iron in the blood and the general health of the patient.
In the case of confirmed hemochromatosis, the patient must also be instructed on the correct dietary approach: therefore, all foods in which iron is present in abundance, such as red meat, offal and crustaceans, should be avoided. foods rich in fiber (wholemeal bread, wholemeal pasta, vegetables, etc.) should be consumed in abundant quantities in the context of hemochromatosis, since phytates and fiber decrease intestinal absorption of iron. The consumption of alcohol is prohibited. : compliance with this dietary rule is very important to curb damage to the liver.
As for drugs, the administration of chelating substances is the most suitable: chelating substances favor the expulsion of iron from the body, through faeces or urination. Chelation therapy is often associated with concomitant administration of ascorbic acid.
The following are the classes of drugs most used in the therapy against hemochromatosis, and some examples of pharmacological specialties; it is up to the doctor to choose the most suitable active ingredient and dosage for the patient, based on the severity of the disease, the state of health of the patient and his response to treatment:
Long-term chelation therapy for the treatment of hemochromatosis
- Desferrioxamine (eg Desferal): very important chelating drug capable of complexing iron and favoring its expulsion towards the outside through urination. The drug is available as a powder and solvent for solution for injection; in most cases, Desferal therapy is started following 10-20 blood transfusions. The dosage should be carefully established by the doctor on the basis of the severity of the hemochromatosis, as well as the concentration of iron in the blood. It is also recommended to monitor the amount of iron excreted in the urine (after the dose of the drug) during 24 hours. hours after drug administration. As an approximate value, it is possible to report that the average daily drug dose is between 20 and 60 mg / kg; the drug is administered by subcutaneous infusion of 8-12 hours, 3-7 times weekly Desferrioxamine is the drug of choice used in therapy for the treatment of hemochromatosis.
- Deferasirox (eg Exjade): this is another chelating drug to be taken orally, not marketed in our country. The drug, available in tablets to be dissolved in water or juice, is indicated for children over the age of 6 and for adults suffering from hemochromatosis, forced to numerous blood transfusions. The drug is usually prescribed when Desferrioxamine is not indicated for that patient. Indicatively, start therapy with an estimated drug dose of around 10-30 mg / kg, once a day (based on the concentration of ferritin in the blood and the amount of blood used during the transfusion); it is recommended to take the drug on an empty stomach, at least 30 minutes before a meal, at about the same time every day. The maintenance dose must be perfected in each individual case; generally, adjust the dosage by 5-10 mg / kg every 3-6 months. Do not exceed 30 mg / kg per day.
- Deferiprone (eg Ferriprox): as an alternative to Desferrioxamine (in patients with hemochromatosis who cannot tolerate standard chelation therapy), it is recommended to take Deferiprone, a fairly recent oral chelating drug, indicated for the accumulation of iron (especially for patients with major thalassemia). The drug is available in 500 mg tablets or as an oral solution of 100 mg / ml. Indicatively, the dosage suggests taking 75-100 mg / kg of the drug per day, in three equally divided doses. the treatment of hemochromatiosis, do not exceed the dose of 100 mg / kg / day.
Integrative therapy with vitamin C, to be associated with chelation therapy
- Vitamin C (eg. Redoxon, Cebion, Cimille, Univit, C Tard, Agruvit, Univit, Duo C): an "integration of ascorbic acid is important in patients undergoing chelation therapy for iron. It is considered very likely that the excess iron causes, consequently, a vitamin C deficiency (up to degenerate into scurvy): the explanation for this lies in the hypothesis that iron oxidizes vitamin C. Vitamin C supplementation in association with chelation therapy for the treatment of hemochromatosis it is essential to increase and favor the excretion of excess iron. It is therefore recommended to undertake supplementary therapy with vitamin C, to be started 28 days after the first administration of the chelating drug. Indicatively, administer 50 mg of ascorbic acid in children under the age of 10; double the dose for older children. For adults undergoing chelation therapy for the treatment of hemochromatosis, up to 200 mg per day of ascorbic acid can be taken.
