Sickle Cell Disease Patient & Family Handbook

Iron Overload The body needs iron to make hemoglobin. Breakdown of red blood cells (hemolysis) results in the release of iron into the body so that it may be used to make new red blood cells. There is an increased rate of hemolysis in individuals living with sickle cell disease. Blood transfusions also can contribute additional iron to the body. Your child’s hematology team will monitor the results of a laboratory test (called a ferritin test, which is also the name of the protein containing the iron) to determine if iron levels are becoming too high. The body has no way of getting rid of iron naturally. If the ferritin level is very high, medications called iron chelators may be used to remove excess iron from the body to prevent organ damage. n WHEN WILL MY CHILD NEED TO BE IN THE HOSPITAL? From time to time, children diagnosed with sickle cell disease will need to spend time in the hospital. The most common reasons for hospitalization are fever and pain that cannot be relieved by medications at home. Other reasons include aplastic crisis, splenic sequestration, and respiratory symptoms. Some children with sickle cell disease may need surgery. Because of chronic anemia and the potential for red blood cells to become sickle shaped, these children are at greater risk for complications from surgery and anesthesia. These complications include infection, acute chest syndrome, and stroke. Before surgery can take place, children often will need to receive fluid by vein and possibly a blood transfusion. Following surgery, they will need to be observed closely for complications. Make sure your child’s hematology team is aware of any planned surgeries. This includes any time your child’s dentist plans to use sedation or anesthesia.

n WHAT TESTS AND PROCEDURES WILL MY CHILD NEED? Blood Tests

Often, it will be necessary for your child to have blood tests to monitor their sickle cell disease, look for complications, and check responses to treatment. The following are some common blood tests: • Hemoglobin electrophoresis measures the types of hemoglobin present in the blood to determine if your child has sickle cell disease and, if so, which type. • A complete blood count (CBC) determines the number of

white blood cells (infection-fighting cells) and platelets (clot-forming cells) and the level of hemoglobin in your child’s body. A reticulocyte count, which measures the rate at which the body is producing new red blood cells, often is done with a CBC. • A chemistry panel checks that your child’s body has the correct amount of important substances such as electrolytes, protein, and sugar. It also checks the liver and kidney function and nutritional status.

10

Powered by