Hemophilia, an inherited disease, affects around 18,000 people in the United States, mostly males, according to the National Heart Lung and Blood Institute (NHLBI). Hemophilia A, the most common type of hemophilia, affects 85 percent of hemophiliacs. A lack of the clotting factor VIII causes hemophilia A; factor VIII works with platelets, blood cell fragments, to help blood clot. Lack of factor VIII doesn't increase the speed of bleeding, but increases the length of time a person bleeds. Hemophiliacs bleed after injury, but also bleed spontaneously. Symptoms center on bleeding tendencies.
Bruising
People with Hemophilia A bleed for longer times from even minor injuries. A small bump against a piece of furniture that would not affect a person without the disease leaves a bruise on a hemophiliac. Most children with hemophilia A don't show any symptoms of the disease until they start to move around on their own. Children with hemophilia will have an unusual number of bruises compared to other children. People with mild hemophilia may only be symptomatic after having surgery or a major injury; however, 70 percent of people with hemophilia A have a severe form of the disease, the NHLBI reports. Severe hemophiliacs have a 1 percent or less of the normal amount of clotting factor in their blood, the University of Colorado at Denver Health Sciences Center says.
Joint and Muscle Symptoms
Hemarthrosis, or bleeding into a large joint, occurs frequently in people with hemophilia A, especially in the active childhood years. Continued bleeding, which most often affects the ankle, elbow, hip, knee and shoulder, according to UCHSC, can destroy the bone and cartilage of the joint over time, leading to permanent deformity. Similar damage occurs within the muscles, most often the arms, legs and groin. Immobilizing joints when a bleed first occurs and surgery to repair damaged joints are often necessary, Medical University of South Carolina (MUSC) states. Symptoms of a bleed into the joints or muscles are pain, swelling, tightness, deformity and inability to use the joint or muscle.
Administration of factor VIII concentrates, which can be derived from human blood or made in the laboratory, are more expensive than previous treatments of whole blood or plasma, but are unlikely to transmit viruses such as Hepatitis C and HIV, according to the NHLBI.
Brain Bleed
Certain types of bleeding from hemophilia A are more likely to be life threatening than others. Bleeding into the brain, which can occur spontaneously or after an injury, is the most common cause of death in children with hemophilia, MUSC warns. Even a small bump on the head can start bleeding that can lead to brain damage, with retardation, blindness or death if aggressive treatment isn't begun immediately. Change in level of consciousness, stupor, apathy, headache, difficulty speaking or loss of coordination can all indicate a bleed into the brain, the Milton S. Hershey Medical Center reports. Bleeding into the stomach or throat can also be life threatening, UCHSC states.
