Hemophilia A and B are bleeding disorders resulting from decreased levels of factor VIII and IX, respectively, and follow an X-linked inheritance pattern. Hemarthroses and intramuscular bleeds are the hallmarks of hemophilia. Even so, petechiae and mucosal bleeding can occur. Intracranial hemorrhage is the leading cause of death due to bleeding in hemophiliac patients, so special care must be taken with patients presenting with a history of head trauma or headache.

Normal levels of factor VIII and IX activity are 50% to 200%. Patients with >1% factor activity are considered severe hemophiliacs, those with 1% to 5% activity are considered moderate, and those with >5% activity are considered mild. Several types of factor replacement are available and used to treat patients with both hemophilia A and B. The vast majority of patients have a home supply of factor; it is of utmost importance to use of the brand of factor used at home whenever possible in an emergency situation. Hemophiliacs should be instructed to bring a home supply of factor with them if they have experienced trauma and need to seek care in an emergency department. If such a patient presents with a history of trauma and/or pain, factor should be infused immediately; even preceding diagnostic evaluation. This caveat is especially important if the pain and/or site of trauma is intracranial or intra-abdominal. These two sites are of special concern because of the risk of increased intracranial pressure with a head bleed and the risk of large amount of blood loss in the abdominal cavity before symptoms arise. Bleeding is the cause of pain in most hemophiliac patients and needs to be treated in a timely fashion to prevent long-term sequelae.