Hemophilia is primarily treated by replacing the absent or abnormal clotting factors to prevent severe blood loss and complications from bleeding. Clotting factors are replaced by injecting (infusing) a clotting factor replacement into the veins. Infusions of clotting factors help blood to clot normally and prevent damage to muscle, joints, and organs.

There are different types of blood clotting factor replacement.

Fresh frozen plasma is the liquid part of blood (plasma) taken from a donor and frozen for later use.

• Frozen plasma has all types of clotting factors, so it can be used to treat many different bleeding problems. It costs less than other kinds of clotting factor.
• A lot of plasma is needed to provide enough clotting factor to prevent bleeds. It must be frozen. It's usually used in a hospital and is not easily kept at home.

Cryoprecipitate is a blood product that has many clotting factors, so it can also be used to treat many kinds of bleeding problems.

• It is concentrated, so you need less of it to stop a bleed than you would of frozen plasma. But you need more of it than you do of other types of factor concentrates, including the kind made in a lab, to raise clotting factors to a safe level.
• It's not likely to spread a virus, because it comes from only one donor. It costs less than other types of concentrated clotting factor.
• It must be frozen, so it is usually used in a hospital and is not easily kept at home.

Factor concentrates are used to prevent or treat bleeds that happen outside a hospital.

• If you give yourself (infuse) factor concentrate on a regular basis, you can prevent some bleeds from happening. And if you infuse soon after a bleed begins, you can stop the bleed before it gets bad.
• You can carry factor concentrates with you. They are easily stored and can be infused at home.
• Donated factor concentrate comes from blood from many donors and has a high level of clotting factor. Donors are carefully screened. Donated blood is purified to kill most viruses.
• Recombinant clotting factors are made in a lab. They are made with recombinant DNA technology. They are concentrated into a powder form that is then mixed with sterile water and injected. They don't come from donated blood.

What To Expect After Treatment

The infusion of clotting factor stops bleeding within hours, although the exact amount of time varies. Heavy bleeding takes longer to control than light bleeding. If the proper amount of replacement factors is given, bleeding will stop normally.

Why It Is Done

Clotting factors can treat bleeding episodes or prevent bleeding before surgeries and activities that may cause bleeding.

Clotting factor replacement can be done in one of two ways:

• On demand, to prevent or control a bleeding problem that has happened or is about to happen
• On a regularly scheduled basis, to prevent bleeding problems

For help on the decision to have regularly scheduled or on-demand clotting factor replacement, see:

Hemophilia: Should I Have Regularly Scheduled or On-Demand Clotting Factor Replacement?

How Well It Works

With on-demand treatment, you can give yourself a shot to quickly control bleeding. You can give yourself a shot before you begin activities where there is a high risk of bleeding. To stop internal bleeding from causing long-term damage to joints, you must replace clotting factors promptly.

With regularly scheduled treatment, you likely will have fewer bleeds if you get clotting factor on a regular schedule. This is especially important if you have severe hemophilia.

Risks

Virus infection from donated clotting factor. The risk of getting a virus from donated clotting factor is very low. If you use clotting factor that is made in a lab, you have almost no risk of getting an infection. Clotting factor that is made in a lab is also called recombinant clotting factor.

In the United States, donated blood products are carefully tested. If there is a chance that donated blood has been infected with a virus, such as HIV, hepatitis B, or hepatitis C, it is not used.

Donated blood also goes through purification processes that destroy viruses that are not found with tests.

Some viruses are harder to detect even with careful screening. People who receive many units of donated blood factor concentrate over their lives have a slightly increased risk of getting one of these viruses. For example, hepatitis A and parvovirus (the virus that causes fifth disease) are harder to detect. They can sometimes be spread by donated blood.

Inhibitors. Some people develop antibodies to the injected clotting factor. These antibodies are called inhibitors. If you develop inhibitors, the usual forms of clotting factors may not effectively prevent or stop bleeding. Children are more likely to develop inhibitors than adults.

For more information about inhibitors, see Hemophilia: Treatment for People With Inhibitors.

What To Think About

With regularly scheduled treatment, clotting factor must be injected into a vein every few days. It may be hard to stay on schedule with the injections.

Adults and parents of children who have hemophilia can learn to inject replacement clotting factors. Children may also be taught to infuse themselves with clotting factors by age 10. Younger children and those who have developed antibodies (inhibitors) may not be able to infuse themselves.

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