In some patients with hemophilia, the immune system produces an antibody that inhibits the action of replacement blood products and prevents clot formation. This antibody is known as an inhibitor. The presence of an inhibitor makes the treatment of bleeding episodes more difficult. An inhibitor destroys the clotting factor before it has a chance to stop the bleeding. The reason inhibitors develop is uncertain; however, they occur more frequently in people with severe forms of hemophilia, particularly factor VIII deficiency, because of their need for more frequent infusions. Inhibitors tend to develop within the first one to three years of treatment, typically between the 50th and 100th exposure days.
For more information, please visit our inhibitors section by clicking here.
One of the major complications of hemophilia is joint damage or “hemophilic arthropathy” that can occur when there is bleeding into joints. This is the most common clinical complication of hemophilia. Bleeding into knees, elbows, ankles, shoulders, and hips can lead to chronic swelling and later joint deformity. Many people with severe hemophilia can suffer from painful, debilitating joint bleeds and associated mobility issues that severely impede their quality of life.
In the late 1970s-and 80s people with hemophilia were treated with blood products derived from thousands of donors. When the U.S. blood supply became contaminated by HIV, the products used as treatment for thousands of people with bleeding disorders also became contaminated. More than 50% of the hemophilia population became infected with HIV prior to 1985. The tremendous impact of HIV/AIDS on the hemophilia community is still with us. So many families have lost loved ones. This devastation has placed an emotional, health, ethical and financial burden on affected families and the entire community as well.
The tragedy of the HIV/AIDS crisis gave rise to heightened vigilance surrounding the safety of the nation’s blood supply and blood products. HIV transmission by factor concentrates in the United States has not occurred since 1986 due to viral inactivation methods used in manufacturing blood products. While new screening methods and product processing procedures are now in place, continued improvements and steadfast oversight are needed to ensure that this tragedy is not repeated.
Hepatitis viruses were also transmitted in blood products used by persons with bleeding disorders. Today’s blood products are much safer than those of the past. As of 1997, there have been no reports of hepatitis C transmission through clotting factor treated with newer processes.
There are six main hepatitis viruses, which cause problems ranging from mild chronic infections to liver failure. Almost 95% of all hepatitis cases are hepatitis A, B, or C. Some hepatitis viruses can be asymptomatic for many years and may never become chronic. Others can progress to liver cancers, end stage liver disease, and other life threatening conditions.
Symptoms may include fatigue, nausea, vomiting, joint aches, liver tenderness and enlargement, and weight loss. For more information about hepatitis, call HANDI for a copy of the publication “Understanding Hepatitis”.
Transmission of hepatitis A remains a risk for people with bleeding disorders who use plasma-derived products. This is because hepatitis A virus can resist the viral inactivation methods used to manufacture plasma products. Hepatitis A is preventable. MASAC recommends all patients with bleeding disorders receive a hepatitis A and B vaccination. Currently, there is no vaccination for hepatitis C.