RADIOLOGY TESTS Radiology tests are used to detect changes that may indicate a future problem, diagnose a complication, or monitor a response to a treatment plan. The following are examples of radiology tests: • X ray—takes pictures of the inside of the body to diagnose problems and is the most commonly used radiology test. A wrist X ray is often done to monitor bone growth. • Magnetic resonance imaging (MRI)—uses a magnetic field and radio waves to produce exact images of what soft tissues, bones, and organs look like. For the test, your child will lie on a table that will then move into a tubelike machine, which surrounds your child with a magnetic field. The test is painless, but the machine makes a loud banging noise that may be scary for some children. Children must be able to lie completely still during the scan, and some may require sedation to help them lie still. • T2* MRI—a special type of noninvasive MRI that measures iron content in the heart. • FerriScan MRI—a special type of MRI that measures the iron content in the liver. • Bone density test—a test that uses X rays to determine how much calcium and other bone minerals are present in areas of the bone such as the hip, forearm, or wrist. The density of the bone is compared to the average values based on sex, age, and size and helps determine if your child has strong bones or osteoporosis, a condition that causes weak, fragile bones that are likely to break.
OTHER TESTS • Electrocardiogram (ECG)—measures and records the electrical activity of the heart and diagnoses a range of heart problems. • Echocardiography (ECHO)—uses sound waves or ultrasound to look at the structure of the heart and how the blood flows through it. • 24-hour Holter monitor—diagnoses arrhythmias (irregular heartbeats). It is a noninvasive test that is usually used for children older than age 12. • Liver biopsy—removes a small piece of the liver and examines it for iron overload, scarring, and other problems.
n HOW IS THALASSEMIA TREATED?
BLOOD TRANSFUSIONS Your healthcare team will determine when and how often your child will
need a red blood cell transfusion. The healthy red blood cells that are being transfused have the hemoglobin needed to carry oxygen that is essential to the body. The goals of transfusions are to minimize bone marrow expansion, avoid hypersplenism, and promote normal growth and development. Transfusions are given through a vein during a 2–4-hour period. Patients with thalassemia major will depend on blood transfusions, which are usually given every 2–4 weeks. Patients with thalassemia intermedia or hemoglobin H disease may need blood transfusions only during times of stress, such as growth spurts, illness, surgery, or pregnancy. IRON CHELATORS An iron chelator is a medication that attaches to extra iron in the body and removes it. Currently, there are three chelators widely available: Deferoxamine (Desferal ® ), which is given by needle under the skin or into a vein, and Deferasirox (Exjade ® and Jadenu ® ) and Deferiprone (Ferriprox ® ), which are given by mouth. Your child’s healthcare provider will decide which chelator is the best option.
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