n WHAT IS HEMOPHILIA? The blood clotting system is made up of proteins that work together to prevent too much bleeding or too much clotting. There are a number of proteins (factors) that must work together to form a fibrin clot, which keeps blood from leaking out of the vessel. Hemophilia is an inherited bleeding disorder. Hemophilia occurs when one of the factors needed to form a clot is missing or does not work well. Blood may flow out of the body, for instance, from a cut or scrape or it may leak into tissue around the vessel, causing internal bleeding. Any type of trauma, such as falling and bumping the knee, can cause a bruise (hematoma). Bleeding into tissue can cause pain, swelling, and slow healing. A person with hemophilia does not bleed faster than someone without it, they bleed longer.
n WHAT ARE THE TYPES OF HEMOPHILIA? There are two types of hemophilia. Hemophilia A , also called classic hemophilia , is a shortage of Factor VIII (8). Hemophilia A is the most common form of hemophilia, occurring in 80% of people with hemophilia. Hemophilia B is a shortage of Factor IX (9) and is sometimes called Christmas disease after the first person diagnosed with this disorder. Hemophilia B occurs in 20% of people with hemophilia.
The normal range of Factor VIII and Factor IX activity is 50%–200%. There are different levels of factor activity in hemophilia. In severe hemophilia, the factor level is less than 1% or almost cannot be measured. Moderate hemophilia is a factor level of 1%–5%. Mild hemophilia is a factor level of 6%–49%. People with severe hemophilia may experience
spontaneous bleeding, or bleeding that starts with no obvious injury. The bleeding pattern in moderate hemophilia is a little harder to predict, but people with moderate hemophilia certainly are more likely to have prolonged bleeding with minor trauma and can develop joint disease with recurrent bleeding into the same joint. People with mild hemophilia are more likely to bleed as a result of trauma or surgery. They rarely have bleeding without injury.
n WHAT ARE SOME OF THE SYMPTOMS OF HEMOPHILIA?
Newborn Bleeding If there is a possibility that the baby may have hemophilia (because the mom is a suspected or known carrier), we recommend discussing the method of delivery with the obstetrician. The use of fetal scalp monitoring, forceps, or vacuum suction to assist with delivery should be avoided. If vaginal deliery is not in progress, transition to a C-section delivery is recommended. In addition, a scheduled C-section delivery
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