What Kids Should Know

What Kids Should Know

Learning everything you can about your bleeding disorder is your ticket to independence. Right now, you might want to spend a night at a friend’s house or you may be interested in going to camp. In the future, you might want to go to college or move away from home. You can do all these and reach all these goals and many others!

Understanding your bleeding disorder and knowing how to identify, treat, and prevent bleeds, is the first step. Now, let’s get started.

This section of Next Step covers:

• Bleeding Disorder Basics
• How to Recognize a Bleed
• Why You Should Treat a Bleed Quickly
• How You Treat a Bleed
• Where Do You Receive Factor Replacement Therapy?
• Other Types of Medicine to Treat Bleeds

Bleeding Disorder Basics

Having a bleeding disorder means your blood does not clot normally. That’s because your body is missing a clotting factor. As you can probably guess by its name, clotting factors help blood to clot (called coagulation). People with bleeding disorders usually bleed longer than people who aren’t missing a clotting factor.

It’s easy to see bleeding when you get a cut or scrape. This is called an external injury. However, you can also bleed inside your body where you can’t see the blood. For example, if you get hit by a ball or sprain your ankle, you can bleed into your muscles and joints. This is called an internal injury.

To learn more about bleeding disorders, go to Types of Bleeding Disorders.

The most common treatment for a bleeding disorder is factor replacement therapy. Factor replacement therapy does exactly what it says—it replaces the missing clotting factor so blood can clot. The missing clotting factor is given by infusion, which is an injection into a vein. (In other sections of Next Step, you’ll learn more about how factor replacement therapy is given and how you can learn to give yourself an infusion!) Simple first aid and the R.I.C.E. (Rest, Ice, Compress, Elevate) treatment are helpful for certain types of injuries as well. R.I.C.E. is what athletes and sports medicine experts often recommend for sprains and strains.

How to Recognize a Bleed

When a bleed happens inside the body, it’s not always easy to tell. However, there are some subtle signs that can point to a problem. You may feel tingling, bubbling, or aching around the spot. If the bleed is in a joint—like the knee or elbow joint—the signs may be clearer. You may limp or you might not be able to bend your arm very well or your joint may be swollen or feel warm when you touch it. Of course, pain is also a sign of a bleed.

To learn more about spotting a bleed, go to Identifying Different Types of Bleeds.

Why You Should Treat a Bleed Quickly

When you feel a tingling, bubbling, or a warm or aching sensation, your body is telling you that you've been injured and need to get treatment. For this kind of internal bleed you need to infuse your factor to stop the blood from collecting in your joints and muscles. Without treatment, the blood can pool in your joints and muscles and cause a lot of pain and swelling. If you ignore this kind of bleeding or do not treat it quickly or well enough, it can damage your body and cause problems, such as arthritis.

The lesson: treat a bleed as soon as possible so you can get back to doing all the activities you love! If you’re not sure what to do, treat just to be safe.

When in doubt, treat.

How You Treat a Bleed

Here are the common treatments for a bleed:

• First Aid
  If you get a small cut or scrape, you should treat it with simple first aid: clean the wound with water, apply pressure, and cover with an adhesive bandage. If the cut is really deep, you should infuse your factor to stop the bleeding.
• R.I.C.E.: Rest, Ice, Compress, and Elevate
  If you get a joint or muscle injury, try R.I.C.E.—Rest, Ice, Compress, and Elevate an injured arm or leg. These steps are what professional athletes use to help reduce the swelling and relieve pain from a joint or muscle injury. They use it all the time and so should you! But don’t forget to infuse your factor too!

To learn more, go to R.I.C.E.

• Factor Replacement Therapy
  We’ve been talking a lot about treating bleeds by infusing clotting factor. This is also called factor replacement therapy. Basically, you’re giving your body the type of protein it needs to help your blood clot normally. Your HTC will find the perfect factor replacement therapy plan for your body.

Here’s more information about factor replacement therapy:

• Factor (also called clotting factor or factor replacement) is a dried powder form of the missing clotting factor; it is mixed with sterile water to become a liquid again. This process is called reconstitution. Factor is given by infusion into a vein (called intravenously; also known as IV). The amount of factor you need depends on several things including your weight, where the bleed is, and if it’s where you usually get a bleed (called a target joint).
• On-demand or episodic therapy. This means that factor replacement therapy is given when you have a bleed or before surgery or going to the dentist. When you sprain your ankle, twist your knee, or feel any kind of tingling or bubbling sensation, you will treat on-demand. Remember: do this early after an injury to help prevent extra pain and joint damage.
• Prophylaxis. This means that factor replacement therapy is given to help prevent bleeds from starting. Just like getting a shot to keep you from getting sick, taking factor before you bleed can help prevent bleeds. People who are on prophylactic therapy take factor on a schedule.
  The HTC or the hemophilia treatment specialist (hematologist) will decide when you should infuse (called a schedule or regimen) and how much you should infuse (called the dosage). The schedule and dosage is based on what will work best for your body and lifestyle. Taking factor on a schedule doesn’t mean you won’t have any more bleeds; but the hope is that by increasing the missing factor in your body you’ll have fewer bleeding episodes. Some people with bleeding disorders will still have bleeding episodes (called breakthrough bleeding) even when they’re on prophylactic treatment.
• Inhibitors. An inhibitor is an antibody that the body makes in response to something it thinks is foreign, like a virus or bacteria. When someone develops an inhibitor, the body’s immune system thinks the factor that was infused is not supposed to be there. So the body creates the antibody and tries to block the factor from working. When an inhibitor develops, treatment does not work as well and bleeding is more difficult to control.

Where Do You Receive Factor Replacement Therapy?

You may receive your factor replacement therapy infusions from the doctor or nurse at the Hemophilia Treatment Center (HTC); at home from your parents, family member, home health care nurse, or other caregiver; or you may be infusing yourself, called self-infusion.

Self-infusion gives you the most freedom. If you’re able to treat yourself, you’ll be able to go more places and take more trips, but it involves taking on a lot of responsibility.

Your HTC team, along with your parents, will help you decide if self-infusion is right for you.

To learn if you’re ready, go to Self-Infusion.

Other Types of Medicine to Treat Bleeds

Other treatments for hemophilia and other bleeding disorders may include treatment with a man-made hormones and medicines.

Here are the some of them:

• Desmopressin acetate (DDAVP^®). This drug is used for people who have hemophilia, as well as a bleeding disorder called von Willebrand disease (VWD). It helps the body release 2 types of clotting factors, von Willebrand factor (vWF) and factor VIII (8), from where they’re stored in the body. For people with mild, and some cases of moderate, hemophilia or VWD, DDAVP can help increase their own factor VIII (8) levels so that they do not have to use a clotting factor. DDAVP is given intravenously or it can be breathed in through the nose.
• Aminocaproic acid and tranexamic acid. These drugs help prevent clots from breaking down, so a firmer, stronger clot can form. It is often used for bleeding in the mouth (for example, after a tooth has been removed), because it blocks an enzyme in the saliva that causes clots to come apart. Aminocaproic acid can be given intravenously or taken by mouth (as a pill or a liquid). Tranexamic acid can be given by injection or by mouth (as a pill).

Treatment for bleeding disorders is a lifelong process. Parents play a major role in their children’s treatment in the younger years. During the tween years (ages 9-15), a child's growing independence becomes very important and can drastically change the routine of treatment that has so far been established. This time for exploration and personal growth also involves the child becoming more active in his or her treatment as part of this process.

While both parent and child may have anxiety during this time about treatment, communication, and education, knowing some basic steps to help your child gain independence can ease the transition for everyone and help children move confidently into this exciting stage of their lives.