Individuals with bleeding disorders who present to an emergency room (ER) for care should receive appropriate, expeditious care management. Do not keep these patients waiting. A patient with a bleeding disorder could be experiencing a life-threatening bleed which may not be obvious at first glance.
Many patients and their caregivers are very knowledgeable about their disease – be sure to have an open dialogue with them. They may have brought factor replacement medication with them, or may have infused factor prior to arrival in the ER. If the patient has not contacted his or her hematologist/hemophilia treatment center, be sure to do so immediately. The appropriate plan of care should be coordinated with this physician.
Upon discharge from the hospital, your patient will need a log showing infusions given while in your care. Be sure you have documented all infusions administered so they have the information for their records.
The most common bleeding disorders are hemophilia A, hemophilia B, and von Willebrand Disease.
Hemophilia is a hereditary, lifelong blood disorder – often diagnosed at birth – in which the blood clots more slowly than normal. Normally, when an injury occurs that causes bleeding, our bodies respond by clotting (coagulating) the blood so the bleeding slows and eventually stops. People with hemophilia have a deficiency of a blood protein called clotting factor that causes fibrin clots to not form correctly. A deficiency of clotting factor VIII (eight) is called hemophilia A, and a deficiency of clotting factor IX (nine) is called hemophilia B. People with hemophilia do not bleed faster than other people; they just bleed longer.
Von Willebrand Disease (VWD) is a hereditary bleeding disorder characterized most often by bleeding from the skin and mucous membranes (e.g., mouth, nose, throat, gastrointestinal tract) affecting both males and females equally. This bleeding disorder is the most common inherited bleeding disorder. The most common symptoms of von Willebrand Disease are easy bruising, prolonged nosebleeds, heavy or prolonged menstrual periods (lasting more than 7 days), and prolonged bleeding following injury, surgery, dental procedures, or childbirth. Gastrointestinal bleeding can also occur.
Recommended Bleeding Disorder Treatments
Treatment of hemophilia A
Recombinant factor VIII concentrates
Plasma-derived factor VIII concentrates
- Cryoprecipitate not recommended
- Treatment of mild hemophilia A: Desmopressin nasal spray or injection (Stimate® or DDAVP® injection). If not responsive to desmopressin, factor VIII concentrate is recommended.
When bleeding is severe, the appropriate dose of factor VIII is 50 units/kg. This should result in a factor VIII level of 80%–100%.
Treatment of hemophilia B
- Recombinant factor IX concentrate
- Plasma-derived factor IX concentrates
Treatment of hemophilia A and B with inhibitors
- FEIBA, anti-inhibitor coagulant complex
- NovoSeven® RT, coagulation factor VIIa
Dosing is best determined through immediate discussion with patient’s hematologist. NovoSeven® RT is the preferred agent for patients with Factor IX and a history of inhibitors.
Treatment of von Willebrand (VWD) Disease
See medication package insert for dosing recommendations.
Treat with the synthetic intravenous desmopressin or Stimate 1.5 mg/mL nasal spray for bleedingType 2A, 2M and 2N
Type 2A, 2M and 2N
Consider treating with desmopressin (see Type 1 above) if patient previously showed a response to a desmopressin trial (per patient history). If patient has already treated with desmopressin or Stimate prior to ER visit, then a product containing von Willebrand factor (vWF) such as Alphanate®, Humate-P®, Koate®*, Vonvendi® and Wilate® should be considered.
Type 2B and type 3 – and type 1, 2A, 2M and 2N nonresponsive to desmopressin
Treat with a product that contains the vWF factor protein such as Alphanate®, Humate-P®, Koate®*, Vonvendi® and Wilate®.
For more in-depth information on treatment of a bleeding disorder,