The following text comes from www.HemophiliaEmergencyCare.com. To determine specific factor dosages for a given patient, go to the web site and click on 'Get Factor Dosage Orders'.

Hemophilia Basics

Definition, Effects, Incidence

Hemophilia is a genetic disorder characterized by a deficiency or absence of one of the clotting proteins in plasma. This prevents the formation of a firm, fibrin clot and results in a soft, unstable clot. Persons with hemophilia do not bleed faster than others; rather the bleeding is continuous. Significant blood loss can occur if treatment is delayed.

Hemophilia mostly affects males due to the X-linked inheritance pattern. The incidence worldwide is estimated to occur in 1:7,500 live male births; all races and ethnic groups are affected.

Deficiencies of factor VIII (8) [Hemophilia A or Classic Hemophilia] and factor IX (9) [Hemophilia B or Christmas Disease] are the most common and referred to as hemophilia. Factor VIII (8) deficiency is four times more common than factor IX (9) deficiency but the clinical presentations and inheritance patterns are the same.

Serious bleeding sites

The six major sites of serious bleeding which threaten life, limb, or function are:
   - intracranial
   - spinal cord
   - throat
   - intra-abdominal
   - limb compartments
   - ocular

All of the above require immediate assessment and intervention, and are characterized by:
   - bleeding into an enclosed space
   - compression of vital tissues
   - potential loss of life, limb, or function

Severity

The amount of bleeding expected in an individual with hemophilia depends upon the severity of the deficiency. Normal plasma levels of factor VIII (8) and IX (9) range from 50-150%.

Those with less than 1% factor VIII (8) and IX (9) are considered to have severe hemophilia. Frequent bleeding episodes are common, particularly into joints. Bleeding can occur spontaneously or from trauma.

Persons with factor levels of 1-5% are considered to have moderate hemophilia. These persons may experience bleeding after minor trauma but should not bleed spontaneously. After repeated bleeding into the same joint, persons with moderate hemophilia may experience spontaneous bleeding in that joint.

Persons with more than 5% factor activity are considered to have mild hemophilia and bleed only after significant trauma or with surgery. Some carrier girls and women (called symptomatic carriers) can have lower than normal plasma levels of factor VIII (8) or IX (9) and thus may exhibit symptoms of mild hemophilia.

Treatment

Treatment for bleeding involves replacing the deficient factor as the first course of action. This requires intravenous infusion of commercial factor concentrates. Specific doses, additional drugs and medical interventions depend upon the site and severity of bleeding. Once factor replacement therapy has been infused, diagnostic procedures and examinations can begin.

Working with patients

Parents and persons with hemophilia are knowledgeable about the management of the disorder and their input should be sought and heeded. Most hemophilia families are medically sophisticated and should not be dismissed as novices. Interview the family about whether factor concentrate has been administered prior to arriving at the ER; if so, determine when and at what dose. Additional factor may be required, depending upon the time lag and severity of the bleed. Establish who the treating hematologist or hemophilia treatment center is and contact them for other than routine bleeding.