so they can be at the top of their game and in control of their condition.
We want you to get out there and do your thing.
Watch our video that explains the build-up of iron in the human body and how iron interacts with cells and systems in our body. It aims to help patients understand the importance of daily adherence to their chelation therapy.
Some drugs, including iron chelators, can cause a serious complication known as severe neutropenia or agranulocytosis, which is a sudden and severe drop in neutrophils, an important type of white blood cell.
Because neutrophils help to fight infection, a low neutrophil count may place you at risk for developing a serious infection that can lead to death.
Tell your parents and get emergency medical care right away if you have any of the symptoms of infection listed on the right.
All drugs carry some risks. While most side effects are minor, some can be serious and in a few cases even life-threatening. Before any drug can be licensed, authorities must balance its benefits against its risks, and the health agency in your country will only approve a drug after determining that the benefits outweigh the risks for most people, most of the time. As an Iron Warrior, you need to know what the risks are with your chelation treatment, and you must immediately tell your doctor if you suspect a medical problem might be related to your treatment.
Some chelators may be associated with an increased risk of infection. Talk to your doctor about what you can do to decrease the risk of infection.
In addition, patients with thalassaemia may have had their spleen removed for medical reasons. This increases their risk of getting ill from certain dangerous bacterial germs, including the bacteria pneumococcus, meningococcus, and haemophilus influenzae (which is not related to the flu virus, despite the similar-sounding name).
Good news – There are vaccines available to protect you against most of these germs. Thalassaemia patients of any age who have had their spleen removed must make sure that all their vaccinations are up to date.
Bad news – Some forms of pneumococcus are NOT covered by the vaccines available.
Best advice – In patients without a spleen, any fever over 101.5 °F (38.5 °C) should be considered an emergency. Different doctors have slightly different approaches for this problem, but no high fever can be safely ignored if the spleen is absent.
These germs Listeria, Vibrio and Klebsiella grow faster in the presence of Iron.
Iron Warriors should avoid:
Raw milk and cheese because they carry Listeria
Raw seafood, which can carry Vibrio. (Cooked seafood should be safe)
Raw beef (such as Steak Tartare) which can carry toxoplasmosis.
If you are on chelation treatment and ever have an unexplained fever or feel extremely ill, it is very important to tell your physician if you have eaten any of these foods.
Unluckily, the chelators that act to get iron out of your body may also act to get it INTO these germs, so Iron Warriors may need to temporarily stop their chelation therapy at the start of an illness until they get a diagnosis from the doctor.
The kidneys act to purify the blood. They have many jobs, including maintaining the right blood acidity (pH), maintaining stable levels of electrolytes like sodium, potassium, chloride, and bicarbonate, getting rid of waste products through the urine, and reabsorbing blood components that need to stay in the body, including sugar (glucose) and proteins. Kidney problems can lead to disturbances of blood electrolytes, sometimes associated with vomiting. Several drugs, including certain chelators, can cause a serious complication known as Renal Fanconi Syndrome. If you are not urinating frequently, contact your doctor.
It is essential that all Iron Warriors know as much about thalassaemia as possible. Don’t ever hesitate to ask your doctor questions, and make sure that you get answers that you can understand and that empower you to control your own health. Many questions you have will be specific to your care and health status. Some questions may be answered here on our Iron Warriors Essential List of Q and A’s. If you have any urgent concerns, speak immediately to your doctor (and family members) so they can help you get the speedy answers or medical care that you may need.
It is important to keep your iron levels low to avoid health complications in the future. You can keep an eye on your iron levels by getting them checked regularly. Your iron levels will help your doctor decide what the best chelation plan is for you.
The simplest method is a blood test that measures ferritin, the major iron storage protein in the body. High levels of serum ferritin indicate that you have too much iron in your body. Keeping track of trends in serum ferritin is important and gives your doctor information that helps in making decisions about your chelation regime. However, the results can be variable, and they do not provide direct information about how iron is affecting your organs, especially your heart function.
The best method for measuring iron levels in different organs is through Magnetic Resonance Imaging (MRI). It is used to measure how much iron is in the heart and liver and is being explored as a way of measuring iron in the pancreas and pituitary glands as well. It is recommended that children with thalassemia start having MRI screening at 10 years of age or as soon they can undergo a scan without being sedated.
These types of scans require special software and training, but any hospital that has an MRI machine should be able to obtain these. If these scans are not available at your hospital, talk to your doctor about the nearest place where you can have them done. You should also ask your doctor to encourage your hospital to make them available, if possible.
Using more than one chelator drug is referred to as combination therapy. Your doctor may recommend that you take two drugs on the same day or that you alternate them in a particular sequence. Since the side effects and risks of drugs may increase when they are combined, you may need more frequent safety monitoring.
Follow the chelation treatment that was prescribed for you, and don’t skip any doses unless you have a fever or your doctor has given you a temporary break from it. Skipping treatments means that your long-term health will be at risk.
The Iron Warriors know that sticking to chelation is a big challenge, but we are all here to support your very best efforts to meet these challenges and thrive.
Yes, several independent groups have published Standards of Care. The most recently published guidelines were issued in 2016 by the United Kingdom Thalassaemia Society. You may reference them at this link – http://standards.ukts.org
The United Kingdom Thalassaemia Society 2016 guidelines includes an excellent overview in Chapter 15 on how to handle a variety of acute clinical presentations. We encourage you to
read this information and share it with all of your medical providers. You may reference this information at http://standards.ukts.org
Dr Antonio Piga
Director of Pediatrics,
San Luigi Gonzaga University Hospital,
You may have heard the saying “Know thyself”. Knowing what makes you happy and fulfilled is how you can determine if you are emotionally healthy. Sometimes you may need help in figuring out what things bring you happiness, but the answer really lies within you, and it may change month by month or week by week or even day by day.
The most important thing is that you try and make it a habit to reflect on how you feel about things. If you can’t think what makes you happy and strong and set on enjoying each day, you should talk to your health care team. They may have suggestions on how you can make adjustments to your daily life that will increase your comfort and improve your ability to accomplish your goals and enjoy your own personal journey.
Sometimes, your friends might be curious about Thalassaemia and have questions about it but are not sure how to bring up the topic.
Here are some questions and answers that you can use to start a conversation with them that might make it easier to talk about your experiences.
Thalassaemia is a disorder in which red blood cells don’t work properly. The other blood cells (white cells, platelets) are fine in people with Thalassaemia. Red blood cells have the job of carrying oxygen to all other cells in the body. Thalassaemia is inherited, meaning that it is caused by genes that were passed on by parents. It can’t be spread to other people like a cold or the flu.
Yes, there are different types, each of which is caused by a specific genetic defect that causes each type, and treatment may vary depending on the type.
Since thalassaemia is an inherited disorder, people who carry a gene for it are at risk of passing it on to their children. If expectant parents know that they are carriers, testing can be done before birth to find out if the baby will have thalassaemia. With the more serious forms of thalassaemia, the disorder often becomes apparent in the first year of life.
Enjoy each other’s company and plan fun and healthy adventures together. Be a good listener. Be understanding. At times, (maybe before a transfusion), your friend may be more tired than usual and not ready for a big night out on the town. Support each other in avoiding alcohol and cigarettes. Alcohol acts to make the damaging effects of iron worse while smoking affects bone growth and is associated with osteoporosis (loss of bone mass, which makes bones fragile and more likely to fracture). Getting together for sports or going for a walk can help maintain strong bones.
Background image by Klubovy via iStock
Nutritional deficiencies may be common in people with thalassemia.Make sure you have adequate dietary intake of calcium, vitamin D, folate, trace minerals (copper, zinc, and selenium), and antioxidant vitamins (E and C).
Calcium is key – A diet with inadequate calcium will result in a decrease in the amount of calcium stored in the bones, which can then become weak and will fracture easily.
Avoid soda pop – its high sugar and additive ingredients work against your ability to build strong bones.
Some foods, such as orange juice, may increase iron absorption. Tea, dairy products, and coffee may decrease iron absorption. Chelating well and not skipping your medication is more important than worrying about iron levels found in food.
What is nutrient density and why does it matter? Nutrient density is the selection of food choices that are high in nutritional value. It is important that everyone chooses foods that they like but that they limit the number of low nutritional value foods they consume. Focus on choosing a variety of foods that are packed with vitamins, minerals, fiber, and other nutrients as well as proteins, fat, and carbohydrates.
Image by Toa Heftiba