Hemophilia and inhibitors

Transcription

1 Hemophilia and inhibitors

2 What is hemophilia? You might know what Hemophilia is, but let s start from the beginning to better understand! When you get hurt and bleed, your body turns on a mechanism called blood clotting to stop the bleeding. In this process different clotting factors work together to form the blood clot. If you have Hemophilia you don t have enough of certain clotting factors. This means that your blood won t clot the way it should and it will be harder for you to stop the bleeds. The two most common types of hemophilia depending on the missing factor: hemophilia A and hemophilia B.

3 What causes hemophilia A or B? Hemophilia A and B are genetic disorders carried on the X chromosome This means that when you were born, it was passed from your mother to you through her genes. As you probably know, girls have two X chromosomes (XX) and boys have one chromosome X and one chromosome Y (XY). Boys get an X chromosome from their mother and a Y chromosome from their father. Hemophilia symptoms As your body is not clotting properly you can have 2 types of bleeds: internal or external. Internal bleeds normally happen on joints: knees, hips, ankles and wrists. They might swell (get big) and hurt a lot and you may have a hard time moving around. This means that if your mother has the gene for hemophilia, you can have hemophilia. Treatment of hemophilia A and B XY XX XY If you have hemophilia A, it means your body doesn t have enough of clotting factor VIII (factor 8) and if you have hemophilia B, your body doesn t have enough factor IX (factor 9). Hemophilia can be mild, moderate or severe based on the amount of the clotting factor missing in blood. Treatment consists on getting injections of the factor that you are missing (FVIII or FIX) to help your body form the clot and stop the bleeding.

4 An inhibitor? Some of these white cells produce antibodies that stick to the invaders and help to destroy them What, s that? 1 So, for example, when you get a cold, your body produces antibodies to destroy invaders and protect you. If you have an inhibitor, you need to understand what that means First of all, you need to learn about certain cells inside your body, called white cells. Your body produces white cells which are your body s soldiers. Their job is to fight invaders. invaders 2 Sometimes your body gets confused and starts thinking that the factor you infuse is an invader. When this happens, the body makes antibodies against the factor and attacks it. These antibodies are called inhibitors. white cell 3 Inhibitors can prevent the factor from working and make it harder to stop bleeds. This means that you might not be able to use your regular factor because the inhibitor will destroy it. Inhibitors are more frequent in Hemophilia A than in Hemophilia B

5 Who gets inhibitors in hemophilia A? How do doctors know you have inhibitors? Anybody with hemophilia A can get inhibitors at any age Although they usually start when you are a baby or young like you are now, inhibitors can also show up when you are older. There are many reasons why you might get inhibitors, including race and type of FVIII used to treat hemophilia. No one really knows why some people with hemophilia get inhibitors and other don t. It s nothing you have done wrong, it just happens sometimes. When bleeding does not stop right after infusing your factor, it might be because there is an inhibitor that is blocking the effect. Your doctor will need some blood to test and see if there are inhibitors in your blood. This test will show just how strong the inhibitor might be so that your doctor is better able to adjust your treatment.

6 How are patients with inhibitors treated? There are 2 main ways your doctor may treat your inhibitors 1 One way is to get rid of your 2 inhibitors with a treatment called immune tolerance induction (ITI). With ITI, you will have to infuse large amounts of factor, sometimes over several months. You may need a special IV or port. The goal of this treatment is to help your body to get used to the factor. When this happens, your body will stop making inhibitors and inhibitors will slowly go away. Once the inhibitors are gone, you can continue with your usual treatment. Everyone who tries to get rid of inhibitors knows it can be hard. The second option is to use another type of factor treatment called bypassing agents (BPA). These products will help stop the bleedings by activating another part of the clotting process. However, BPA won t get rid of the inhibitors and they will stay in your body. However, the good news is that many kids who go through ITI really get rid of inhibitors!

7 What You Can Do! If you do your part, you will feel better on most days you will be able to join your family and play with your friends as well as play certain sports. Being a kid with hemophilia is much like life for any other kid. It does mean, though, that you need to do some things differently. When you have inhibitors it can be tough and there will be some days when you just feel bad. Just remember to be brave and keep going. Doing what your parents and doctors say will help you stay healthy.

8 This space is for you! Write down whatever you want to tell or ask your doctor during the next visit. Remember to bring this leaflet with you! Your name:

9 This educational leaflet has been developed by Grifols. Consult your Health Care Professional for further information. BG/FVI/0517/0028a Date of Preparation March Grifols S.A. All full rights reserved worldwide Grifols UK Ltd. Byron House, Cambridge Business Park Cambridge, CB4 0WZ United Kingdom Tel