As a kid with a bleeding disorder, there are so many things to learn. “Hemophilia,” “inhibitor” and “infusion” are just some of the big words you might have heard your parents or doctors use. Knowing what some of these words are can help you when you talk to your teachers, friends or hemophilia treatment center staff about your bleeding disorder. If you have any questions about a word you don’t understand, ask your parents or doctor to explain it. Here are a few important words to get you started:
Hemophilia: This is a bleeding disorder in which your blood doesn’t clump, or “clot,” together the right way. Hemophilia is genetic, which means it comes from your parents and family. It happens when your body is missing a clotting protein called factor. Without factor, your body can’t repair itself as simply from an injury.
von Willebrand (will-a-brand) disease: If you have this bleeding disorder, it means you’re missing or don’t have enough of a clotting factor protein known as von Willebrand factor. There are different kinds of von Willebrand disease. Type I (1) is the mildest and most common, and type III (3) is severe.
Butterfly needle: This looks like a butterfly because it has two “wings” on either side of the needle. It is very small, so it’s good for kids like you with kid-size veins. When doctors use the butterfly needle, they grab both sides of the wings and use a flexible tube to insert it. The best thing about the butterfly needle is that it doesn’t have to go too deep into your skin to work. It is more exact than a larger needle, so you don’t have to get stuck so many times!
Clot: When you take a bath, you know it’s important to plug the drain so water doesn’t go down the pipe. Think of a clot as a drain plug. In a clot, blood clumps together to stop the bleeding and protect a cut or injury from getting infected. If you have a bleeding disorder, your blood doesn’t clot right away. That means you might bleed longer than you should after an injury.
Factor: If you have a bleeding disorder, you might already know what factor is. It actually means two things: First, it’s the medicine, or clotting factor, you infuse to control your bleeding disorder. Second, it’s a protein. People with bleeding disorders don’t have enough of the protein factor they need. That’s why they must inject clotting factor, which helps your body when it needs to form a clot and stop bleeding. There are a few different types of clotting factor that you infuse. Each has a Roman numeral. If you have hemophilia A, you infuse factor VIII (or 8) into your blood. If you have hemophilia B, you infuse factor IX (9). Kids with von Willebrand disease don’t always have to infuse factor. Sometimes, using a nasal spray that has factor is enough to stop the bleeding.
Inhibitor: Think of an inhibitor as a brick wall in your body, stopping the factor that you infuse from doing its job. Your body has antibodies, tiny cells that fight off things your body senses are not normal. It’s your own special defense system! Sometimes, these antibodies can go too far and slow down the way your body uses factor.
Infusion: Look at your wrist. Do you see the tiny veins? These tubelike structures carry blood where it’s needed in your body. People with bleeding disorders have to infuse their medicine. That means you put medicine right into those veins, getting it into your body faster than taking a pill. That’s because blood travels very fast through your veins. Think of the inside of your body as a road map with thousands of “roads,” or veins, running through it. Infusing medicine into one of those veins means it goes throughout the whole system or roadway in seconds!
Port: A doctor places this small plastic box, the size of a golf ball, under your skin. It usually goes in your upper chest, right above your heart. Ports are good for some kids with inhibitors or severe hemophilia who need to infuse often. It makes it easier to put factor into your body—and you don’t have to use as many needles! It’s important to keep your port very clean, so it doesn’t get infected.
Prophylaxis (pro-fa-lax-sis): This long word can be tough to say! But its meaning is pretty simple: It’s something you do to stop bleeds before they start. You know that brushing your teeth every day helps stop tooth decay and gum disease. Getting vaccines from your doctor keeps you from getting sick. All of these are healthy ways of stopping something that could make you feel worse. They all can be called “prophylaxis.” Many kids with bleeding disorders infuse with factor regularly. That’s because they’re trying to stop their bodies from bleeding before they get hurt. So now that you know what it means, every time you infuse—think prophylaxis!
Do you have more questions about these or other words? Be sure to ask your parents or doctors. They are there to help!