Hemophilia A

Alternate Names : Congenital Factor VIII Deficiency

What are the treatments for the disease?

A person with hemophilia A may receive regular transfusions of factor VIII. Other transfusions may be needed in an emergency or before surgery. Medications may also be needed for treatment of the condition or complications. For example, a medication called DDAVP may increase factor VIII levels.

Factor VIII is given by slow, intravenous infusion. A person can do this at home. A person who requires frequent infusions often has a central line surgically implanted. This is a catheter that goes into one of the large veins, and provides a painless way to give the factor VIII. Serious hemorrhage may require treatment in the hospital.

Some persons have immune system problems that make hemophilia A worse. These persons may require treatment with medications to suppress the immune system.

Chronic joint deformity due to hemophilia is becoming less common. However, some individuals require operations for joint problems. The most common sites for surgery include the weight bearing bones of the hips and knees.

What are the side effects of the treatments?

All medications have side effects, including allergic reactions and stomach upset. Surgery carries a risk of bleeding, infection or allergic reaction to the anesthesia.

Before current screening procedures, a factor VIII transfusion carried a risk of HIV or hepatitis. Currently, with present screening techniques for factor VIII products, the risk of acquiring hepatitis and HIV is very low. Individuals who have only been receiving blood products in the past 10 years have a very low risk of acquiring hepatitis and HIV from factor VIII products.

What happens after treatment for the disease?

Lifelong treatment and monitoring are generally required.

How is the disease monitored?

An individual with severe disease may need to have factor VIII levels checked often. Others may require only periodic monitoring and blood tests.