Iron chelation therapy Iron chelation therapy: (kee-LAY-shun) A drug therapy to remove extra iron from the body. Patients with high blood iron (ferritin) levels may receive iron chelation therapy. The U.S. Food and Drug Administratin (FDA) has approved two iron chelators to treat iron overload in the U.S. - deferasirox, an oral iron chelator,… is the main treatment used when you have a condition called iron overload iron overload: A condition that occurs when too much iron accumulates in the body. Bone marrow failure disease patients who need regular red blood cell transfusions are at risk for iron overload. Organ damage can occur if iron overload is not treated. . Iron overload means you have too much iron in your body. This can be a problem for people who get lots of red blood cell red blood cell: The most numerous type of blood cell in healthy people. Red blood cells contain hemoglobin, a protein that picks up oxygen in the lungs and brings it to cells in all parts of the body. Also called erythrocyte, RBC. transfusions.
Basics
Red blood cells contain iron. Each time you get a red blood cell transfusion red blood cell transfusion: A procedure in which packed red blood cells are given to a person through an intravenous (IV) line into the bloodstream. Transfused red blood cells increase the blood count and help improve symptoms of anemia. Before transfused blood is given, donated blood is typed and crossmatched to the… you are putting more iron in your body. Your body has no good way to get rid of the extra iron. This iron can build up in your vital organs and may injure them over time. Only a doctor can decide if you have iron overload and need treatment.
What is iron overload?
Iron chelation therapy is used when you have a condition called iron overload. Iron overload means you have too much iron in your body. This can be a problem for people who get lots of red blood cell transfusions. Since red blood cells contain iron, each time you get a red blood cell transfusion you are putting more iron in your body. Your body has no good way to get rid of the extra iron. This iron can build up in your vital organs and may injure them over time.
Extra iron that is not immediately needed to make new red blood cells is normally stored in the liver, spleen, and bone marrow bone marrow: The soft, spongy tissue inside most bones. Blood cells are formed in the bone marrow. . Excess iron may accumulate in these 3 organs and in other organs that don't normally store iron, such as the pancreas, heart, joints and skin. This excess iron can injure of these organs.
Only your doctor can decide if iron overload is a problem for you. To decide, your doctor will look at how many blood transfusions blood transfusions: A blood transfusion is a safe and common procedure. Most people who have a bone marrow failure disease like aplastic anemia, MDS or PNH will receive at least one blood transfusion. When you receive a blood transfusion, parts of blood from a donor are put into your bloodstream. This can help some… you have had and do blood tests to check how much iron is in your blood and body. The blood tests might include serum ferritin ferritin: (FER-i-tin) A protein inside of cells that stores iron for later use by your body. Sometimes ferritin is released into the blood. The ferritin level in the blood is called serum ferritin. level, iron concentration, and transferrin transferrin: A protein that carries iron through the blood and to the organs where it is stored. saturation. It is possible your doctor may do other tests like a liver biopsy or an MRI (Magnetic Resonance Imaging) to understand if iron is building up in organs.
Early on, iron overload can cause no symptoms, or it can cause non-specific symptoms that are also seen in other conditions. Some of these symptoms include:
- tiredness or weakness
- loss of sex drive
- weight loss
- abdominal pain
- joint aches or pain
Young people with iron overload might not grow or go through puberty normally.
Women who have iron overload might stop getting their periods.
With severe iron overload, you may experience:
- gray-colored or bronze-colored skin
- shortness of breath
- arthritis
- liver disease, including cirrhosis or liver cancer
- enlarged spleen that may cause abdominal pain or difficulty eating a normal-sized meal
- diabetes
- shrunken testicles
- heart problems, including both heart failure and heart rhythm problems
High levels of iron can be detected through two simple blood tests. These tests tell doctors how much iron is stored in your body.
To get a more exact measure of iron overload, there are some other tests your doctor may want to perform:
How quickly does iron overload happen?
This is different for each person. It is difficult to predict the rate at which iron will accumulate in a given patient. For some people, it can take many transfusions over many years for the buildup of iron to cause problems. But, for others it can happen very quickly after as few as 10 to 15 transfusions (20 to 30 units of red blood cells). This is why it is important to talk to your doctor about any symptoms you are having and get your iron levels tested regularly.
Does iron overload make you sick right away?
Iron overload affects each person differently. And there is no definitive set of symptoms that tells you whether or not you have iron overload. Some of the symptoms are like those of many other diseases. These include tiredness, weakness, abdominal pain, low sex drive, and joint pain. This can make it difficult for a doctor to recognize and diagnose.
If left untreated, iron overload can cause organ damage in some people. If this happens, you can become sick very quickly.
When should I worry about iron overload?
In general, you should start being screened for iron overload at the time of your diagnosis. After you have received about 20 units of blood, you should be tested again.
It is important to keep track of how many units of blood you receive each time you get a blood transfusion blood transfusion: A procedure in which whole blood or one of its components is given to a person through an intravenous (IV) line into the bloodstream. A red blood cell transfusion or a platelet transfuson can help some patients with low blood counts. . You may get 2 or more units each time you get a blood transfusion. You might need to get tested for iron overload after only 10 blood transfusion episodes.
If you don't know how many units you get during your blood transfusions, ask your doctor or nurse to help you find out.
Can anything make iron overload worse?
Patients with iron overload should not take iron supplements or multivitamins with iron.
Alcohol use can increase iron overload in the liver and can also damage the liver. Also, some viral infections such as Hepatitis C can cause the liver to be damaged by iron more quickly and seriously.
Check with your doctor to see if you should make any changes to your diet. Because the typical iron absorption from our diets is low (1 to 4 milligrams of iron per day) compared to the amount of iron in a single unit of blood cells (200 to 250 mg per day), some doctors will counsel you to eat a generally healthy diet and not to worry. Other doctors may recommend a diet that is low in iron-rich foods, avoiding such items as red meat, fish fish: See fluorescence in situ hybridization. , iron fortified cereals and eggs.
What is Iron Chelation Therapy?
Drugs called iron chelators remove extra iron from your body. There are two iron chelators approved by the U.S. Food and Drug Administration (FDA) for use in the U.S.
- Deferoxamine (Desferal®) is usually administered by subcutaneous (under the skin) infusion using a small portable pump about the size of a CD player. The pump is worn for 8-12 hours a day, usually at night while sleeping. Many patients find taking deferoxamine deferoxamine: Deferoxamine is an iron-binding agent that belongs to a class of drugs known as heavy metal antagonists. It works by helping the kidneys and gallbladder get rid of the extra iron. difficult because of the need to carry around a pump.
- Deferasirox is a newer iron chelator that now comes in two forms (see below). Deferasirox Deferasirox: It comes in a tablet form which is dissolved in juice or water and taken by mouth. Iron build-up can occur in patients who get a lot of red blood cell transfusions. Deferasirox binds with the iron in the body and is removed by the kidneys. Bone marrow failure disease patients are at risk for… is not recommended for people with high-risk MDS.
- Exjade® is a tablet form that must be dissolved in juice or water and taken (by mouth) once a day. Most patients tolerate it very well.
- Jadenu® is new tablet formulation of deferasirox approved for use in 2015. It is taken on an empty stomach or with a light meal once a day with water or other liquids.
One additional iron chelator is currently being used in Europe, Asia, and Canada, but is not yet approved by the FDA for use in the United States:
- Deferiprone or L1 (Ferriprox™) comes in a pill form and is taken 3 times a day. It is generally well tolerated by patients, but it can cause a drop in white blood cell white blood cell: Cells in the body that fight disease and infection by attacking and killing germs. There are several types of white blood cells including neutrophils, eosinophils, basophils, lymphocytes and monocytes. Each type of cell fights a different kind of germ. Also called WBC, leukocyte. counts, so patients need to have their blood checked weekly while taking this drug (white blood cells protect you against infections).
How well does it work?
Many studies have demonstrated that iron chelators are very effective at reducing iron overload caused by lots of blood transfusions.
What are common side effects?
Some common side effects of iron chelators include:
- Nausea and dizziness
- Diarrhea
- Rash
- Vomiting
- Vision and hearing problems
More serious effects such as kidney or liver injury can occur. Once the body gets used to the drug, side effects usually go away. Your doctor should monitor your liver and kidneys for potentially serious side effects while you are taking deferasirox.
When taking either deferoxamine or deferasirox, you should:
- Have your vision and hearing tested prior to starting therapy, with re-testing every 6-12 months. Both deferoxamine and deferasirox can cause damage to the eyes and ears.
- Avoid taking Vitamin C unless it is prescribed by your doctor. Under your doctor's specific orders, Vitamin C can be added at a later time to iron chelation therapy and may improve results for some patients. Vitamin C should only be taken in a moderate dose, such as 100 mg daily.