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Definition: Iron chelation therapy is the removal of excess iron from the body with special drugs. Chelate is from the Greek word “claw”.
Patients who have anemia (low hemoglobin) and iron overload at the same time cannot tolerate phlebotomy (blood donation). These patients need iron chelation therapy to remove the iron.
Anemia with iron overload is prominent in several conditions such as sickle cell disease, thalassemia major, myelodysplasic syndromes (MDS), enzyme disorders, iron transport or storage disorders, and some forms of cancers. Patients with these conditions require repeated blood transfusions to survive.
Each unit of blood used in transfusion contains about 250 milligrams of iron. The body cannot excrete iron, except in tiny amounts-about one milligram per day, which is sloughed off in skin or perspiration. Therefore the excess iron is trapped in the tissues of vital organs, such as the anterior pituitary, heart, liver, pancreas and joints. When the iron reaches toxic levels, damage can result in diseases such as diabetes, cirrhosis, osteoarthritis, heart attack, and hormone imbalances. Hypothyroidism, hypogonadism, infertility, impotence and sterility can result from these hormone imbalances. The patient can experience symptoms of chronic fatigue, mood swings, loss of sex drive, confusion, and memory loss. If not addressed, excess iron can result in complete organ failure and death.
It is important to remove the unnecessary iron before damage can occur. Iron reduction is accomplished with chelation therapy, which is the removal of iron pharmacologically with an iron-chelating agent such as desferrioxamine, brand name Desferal or deferasirox, brand name Exjade. Both of these products are manufactured by Norvartis Pharmaceuticals and are currently the only two iron chelator drugs approved for use in the US. These drugs are specifically formulated to bind with iron so that the iron can be excreted in urine.
Other types of chelation
The type of chelation therapy used to de-iron patients should not be confused with EDTA (ethylenediaminetetra-acetic acid), a method used by some alternative medicine practitioners. EDTA is a broad-spectrum chelator, meaning that it binds with and removes a wide number of minerals, including iron, but it is not specific to iron.
Deferrioxamine, brand name Desferal is not absorbed in the intestinal tract; therefore, this drug must be administered intravenously at an infusion center. Or the drug can be given subcutaneously, using a portable battery-operated infusion pump. Generally, the pump is worn at night, where slow infusion of the iron chelating agent is administered over a period of about eight hours, for the duration of four to six nightly infusions per week. Patients are given a step-by-step demonstration of how to sterilize the skin, insert the needle and operate the pump.
Before Desferal is administered by either method, a test dose is given to be certain that there are no immediate reactions to the drug. Desferal is administered slowly at first, beginning with 1.0 gram, three to four times per week with monitoring of iron excretion in a cumulative 24-hour urine sample. If effective, the dose can then be adjusted upwards, one gram at a time, up to four times per week, until the patient reaches a tolerable level. The dose should not to exceed 50 milligrams/kg weight, or about 3 grams per day. Periodic examination of the patient is necessary until positive response to treatment is confirmed. Patients might be given an additional two grams of Desferal intravenously for each unit of blood transfused. Desferal is injected separately from blood transfusions.
Desferal has been approved for use in the USA since the late 1970’s. Deferiprone also called L1, brand name Ferriprox is different from Desferal in that Ferriprox can be taken orally. In August of 1999, Apotex Inc., the Canadian Pharmaceutical Manufacturer of Ferriprox was granted approval in Europe by the European Agency for the Evaluation of Medicinal Products to use the oral chelator for treatment of iron overload in patients with thalassemia when desferrioxamine therapy is contraindicated or in those who develop serious toxicity with desferrioxamine therapy. Ferriprox is not approved for use in the USA.
The primary role of iron-chelation therapy is to prevent premature death from heart attack due to myocardial iron overload. Statistically 50% of patients with thalassemia major die of heart attack before the age of 35, primarily due to iron-related heart failure.
Side effects of IV iron chelation:
The urine can become orange colored, which is a harmless side effect. Immediate symptoms of adverse reaction to IV iron chelation therapy might include: visual disturbances, blurred vision, rash or hives, itching, vomiting, diarrhea, stomach or leg cramps, fever, rapid heart beat, hypotension (low blood pressure), dizziness, anaphylactic shock, and pain or swelling at site of intravenous entry. Long term problems might include kidney or liver damage, loss of hearing or cataracts.
Patients should report such symptoms immediately to their physician who can adjust dosage. Further, physicians might examine patient’s visual status with slit-lamp fundoscopy (examination of the eye) and hearing status with audiometry or hearing test. Liver enzymes (ALT, AST, GGT and ALP), a kidney function test such as BUN, serum ferritin and transferrin iron saturation percentage might also be measured by the attending physician.
Limitations of Desferal
Nursing mothers will need to talk with their physicians about iron chelation drugs. It is not known how much of the drug gets into breast milk; thus, a mother who is receiving Desferal treatment might consider low iron soy formula substitutes.
When to begin iron chelation therapy
Initiating chelation therapy depends upon several factors: the patient’s overall health, hematologic values, especially hemoglobin, hematocrit and the tissue iron levels. Tissue iron is determined by measuring serum ferritin, and fasting serum iron and TIBC (total iron-binding capacity). Transferrin-iron saturation percentage (TS%) can be calculate with these last two tests (serum iron divided by TIBC multiplies by 100%). The results from these tests can be used to monitor iron build up and to address the excess iron as soon as possible. Some physicians will begin iron chelation therapy when serum ferritin is between 1,000-1,500ng/mL. Serum ferritin that is above 2,500 ng/mL before beginning iron chelation therapy might result in organ damage.
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Life is sorrow, overcome it.
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Have you ever heard a song from so long ago with so many memories tied to it that it made you cry? And didn’t you with that you could go back into time when everything seemed so much simpler and carefree? Those are songs that are the soundtrack of our lives… the ones that bring back childhood memories, best friends, first love, first heartbreak… the memories.
WARNING: THIS CONTENT IS ONLY FOR ADULTS. CHILDREN READ IT UNDER ADULT SUPERVISION.
Chorionic villus sampling (CVS) is a test carried out during pregnancy to detect specific abnormalities in an unborn baby. A sample of cells is taken from the placenta (the organ that links the mother’s blood supply with her unborn baby’s) and tested for genetic defects.
CVS is offered in pregnancies where there is a high risk of the baby having a serious inherited condition. This could be because:
Read more about why CVS is used, including information on other conditions it can help to diagnose early.
CVS is carried out between weeks 10 and 13 of pregnancy and is not recommended earlier than this. The risk of CVS causing complications, such as miscarriage or birth defects in the baby, is higher if it is carried out before week 10 of the pregnancy.
Read more about the possible complications of CVS.
CVS is an alternative to amniocentesis, where a sample of the mother’s amniotic fluid is taken for testing. CVS can be carried out earlier than amniocentesis, which is usually carried out between weeks 15 and 20 of pregnancy.
Results from amniocentesis can take two to three weeks to come through. This may mean that your pregnancy is at a more advanced stage, around 20 weeks or more, before you can consider the results.
If you are at risk of passing a genetic condition onto your child, your GP or midwife will be able to discuss the tests with you, explain why they might be necessary, and help you and your partner make your decision.
In some cases, you may be referred to a genetic counsellor (a healthcare professional trained in genetics). They will discuss your risk of passing on certain genetic conditions and can offer you advice about what to do when you get the results of CVS.
During CVS, a sample of cells, called chorionic villi cells, will be taken from the pregnant woman’s placenta using either:
Read more about these methods and how CVS is performed.
The test takes about 5 minutes, although the whole consultation will take about 30 minutes. CVS has been described as uncomfortable rather than painful, and there may be some cramps afterwards which are similar to menstrual cramps.
The first result should be available within a few days, and this will tell you whether a major chromosome problem has been discovered.
The full results, including for smaller, rarer conditions, can take two to three weeks to come back. If the test is looking for a specific disorder, the results may take up to a month
At an early stage of pregnancy, the embryo divides into two parts:
The part of the embryo that forms the placenta starts out as finger-like sections that are called chorionic villi. These burrow into the wall of the womb to get close to the mother’s blood vessels.
The chorionic villi are formed by the division of the fertilised egg, which means they have exactly the same DNA as the embryo, including any possible genetic abnormality. Any defect in the chorionic villi will also be present in the foetus
The latest article in the Clinical Therapeutics review series, “Iron-Chelating Therapy for Transfusional Iron Overload,”comes from Dr. Gary Brittenham at the Columbia University Medical Center.
Because humans lack any effective means to excrete excess iron, repeated transfusions over time inexorably produce the clinical problem of iron overload. In patients with thalassemia who undergo transfusion from infancy, iron-induced liver disease and endocrine disorders develop during childhood and are almost inevitably followed in adolescence by death from iron-induced cardiomyopathy unless preemptive chelation is introduced.
• How does iron accumulation lead to organ dysfunction?
Non-transferrin-bound iron is a heterogeneous assortment of iron complexes that appear to be the major mediators of extrahepatic tissue damage in transfusional iron overload. Non-transferrin-bound plasma iron enters specific cells, particularly hepatocytes, cardiomyocytes, anterior pituitary cells, and pancreatic beta-cells. In these cells, iron accumulation leads to the generation of reactive oxygen species, resulting in cellular dysfunction, apoptosis, and necrosis.
• How are the available iron chelators administered?
Deferoxamine is administered subcutaneously or intravenously, usually with a portable pump, for 8 to 10 hours each day, 5 to 7 days per week. Subcutaneous administration is preferred except in patients with severe cardiac iron deposition, for whom continuous intravenous deferoxamine therapy is recommended. The synthetic chelator deferasirox is well absorbed from the gastrointestinal tract and thus can be administered orally. A third iron chelator, the synthetic oral agent deferiprone is not approved for use in the United States or Canada.
Table 1. Iron-Chelating Agents in Clinical Use.
Q: When should iron-chelating therapy be initiated?
A: Ideally, iron-chelating therapy should be initiated prophylactically, before clinically significant iron accumulation has occurred. Treatment should begin when patients have received 10 to 20 red-cell transfusions. Patients who have already undergone repeated transfusion without sufficient chelation can also be successfully treated, but they may require more intensive regimens.
Q: What is the clinical benefit of iron-chelating therapy?
A: Only one small, randomized trial has compared chelation plus deferoxamine with no therapy; this trial enrolled 20 children with (beta)-thalassemia. After a mean of 5.8 years of treatment with intramuscular deferoxamine, the liver iron concentration was 25.9 mg per gram of liver tissue (dry weight) in the deferoxamine group and 42.2 mg per gram in the control group. At 14 years, one death had occurred in the deferoxamine group and six deaths had occurred in the control group.
Breast cancer is diagnosed with self- and physician-examination of the breasts, mammography, ultrasound testing, and biopsy. There are many types of breast cancer that differ in their capability of spreading to other body tissues (metastasis). Treatment of breast cancer depends on the type and location of the breast cancer, as well as the age and health of the patient. The American Cancer Society recommends that a woman should have a baseline mammogram between the ages of 35 and 40 years. Between 40 and 50 years of age mammograms are recommended every other year. After age 50 years, yearly mammograms are recommended.
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