Finding out that you or your child has hemophilia A can be difficult, to say the least. However, understanding what kinds of treatments are available for this bleeding disorder is one way to feel better about what’s ahead. Keep reading to find out more.
No cure exists for hemophilia A, and those with the disorder require life-long treatment. They will likely be treated at a specialized hemophilia treatment center (HTC). Treatment most often involves replacing the missing clotting factor (factor VIII) via infusions. This is often called replacement therapy.
Factor VIII replacement can be obtained from blood donations, but is now usually created artificially in a lab. This is called recombinant factor VIII. There are several recombinant factor VIII replacements available for treating hemophilia A. Examples include:
- Helixate FS
- Kogenate FS
Preparations of factor VIII made from human blood include the following:
- Hemofil M
Side effects of factor VIII replacement therapy can include:
- shortness of breath
- stinging or burning at the injection site
On-demand vs. prophylactic treatment
How often you receive a replacement therapy for hemophilia A depends on the severity of your disorder. If you have a mild form of hemophilia A, you may only need to be treated with replacement therapies right after a bleeding episode. This is called episodic or on-demand treatment.
In contrast, if you have severe hemophilia A, you may need to receive periodic infusions of factor VIII to help prevent bleeding episodes and complications. This is called prophylactic therapy. You can be trained to inject the drug directly at home through an implantable port. A port is a device that is surgically placed under your skin and connects straight to a blood vessel. This is done so you won’t need to locate a vein for every injection.
Some people with mild hemophilia A may be treated with a synthetic hormone called desmopressin (DDAVP). Desmopressin works by raising the levels of factor VIII in the blood. It can be given intravenously (into the vein) or by a nasal spray.
Potential side effects of desmopressin include the following:
- stomach pain
Other medications and treatments
Your doctor may recommend other treatments to control hemophilia A.
Antifibrinolytics are drugs that help prevent the breakdown of clots formed during a bleeding episode. They are often used to treat more mild forms of hemophilia A. Examples include aminocaproic acid (Amicar) and tranexamic acid (Lysteda).
Fibrin sealants are applied directly to wounds to help with clotting and healing. They’re particularly valuable when undergoing dental work.
Your doctor may recommend physical therapy to help with the symptoms of joint damage.
Replacement therapy that comes from human blood are routinely screened for viruses like hepatitis and HIV. However, there is still a very small risk of contracting a disease from any blood product. If you have hemophilia, it’s important to be up to date on available vaccinations, including vaccines for hepatitis A and B.
About 30 percent of people treated for severe hemophilia A develop inhibitors (also called antibodies) against the replacement clotting factors. This means that their body mistakes the replacement clotting factors as foreign invaders and tries to attack them. For these people, controlling a bleeding episode can be very difficult. Drugs called bypassing agents can be used to work around the inhibitors.
Bypassing agents include:
- rFVIIa (NovoSeven)
- anti-inhibitor coagulant complex (FEIBA)
A type of treatment called gene therapy is currently under investigation for hemophilia. In gene therapy, the defective gene responsible for causing hemophilia is replaced with a normal gene. In theory, this approach would cure hemophilia, but so far the therapies that have been developed only provided short-term relief in humans.
Treating hemophilia goes beyond taking medications. You can take steps to help prevent a bleeding episode and stay healthy. These include:
- exercising regularly to help build up muscles and protecting joints — swimming or walking being good options
- avoiding contact sports like football, rugby, hockey, or wrestling
- avoiding certain drugs including those that can aggravate bleeding, like aspirin (Bufferin) and ibuprofen (Advil), and that prevent clotting, including heparin, warfarin (Coumadin), and clopidogrel (Plavix)
- practicing good dental hygiene to avoid the need for dental work and tooth extractions
- wearing pads, helmets, and safety belts when participating in activities with a risk of falling
Deciding which treatment is right for you
Hemophilia is not a simple disease. If you have hemophilia A, you should be in contact with a local HTC to receive the best medical care. The doctors and nurses at an HTC know the most about your disorder. Your specialized team of hematologists, lab professionals, nurses, dental hygienists, physical therapists, social workers, and other professionals can help decide which treatment course is best for you.