MASAC Document #252
Replaces Doc #175
GUIDELINES FOR EMERGENCY DEPARTMENT MANAGEMENT OF INDIVIDUALS WITH HEMOPHILIA AND OTHER BLEEDING DISORDERS
The document was approved by the Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation (NHF) on August 26, 2017, and adopted by the NHF Board of Directors on September 17, 2017.
Individuals with bleeding disorders who present to an emergency department for care should receive appropriate, expeditious management. To this end, MASAC has developed the following guidelines.
1) Individuals with bleeding disorders should be triaged urgently, because delays in administering factor concentrate treatment can significantly affect morbidity and mortality in these individuals bleeding disorders
2) Consultation with the patient's hematologist or a regional hemophilia treatment center professional is strongly advised; however, this should not delay giving clotting factor replacement to the patient.
- Treatment for a suspected bleeding episode is based on clinical history. Physical exam findings may be normal in the early phases of most hemophilic bleeds. Spontaneous bleeding is common in individuals with severe disease (factor levels <1%). When in doubt, administer clotting factor replacement therapy immediately.
- Treatment decisions should be based on the suspicion of a bleeding-related problem, not the documentation of one.
- If the patient or the parent of a patient. suspects that occult bleeding is occurring, administer clotting factor replacement. Patients often are instructed to carry with them appropriate factor replacement dosing guidelines as advised by their treating hematologist.
- Clotting factor replacement therapy should be given before any diagnostics studies (X-rays, CAT scans etc.) are performed to evaluate a suspected bleeding problem, especially in the case of head trauma or suspected intracranial hemorrhage. For routine joint bleeding, no radiographic studies are indicated.
- For patients with hemophilia who have illnesses or disorders that necessitate an invasive procedure (lumbar puncture, arterial blood gas, arthrocentesis, etc.) or surgery, factor replacement therapy to 100% must be administered in the emergency department prior to the planned procedure or surgery.
- For an individual with known hemophilia, routine laboratory studies (PT, PTT, factor levels), are not indicated in the treatment of a routine bleeding episode unless requested by the patient’s hematologist. The clinical severity of a patient's hemophilia is gauged by his or her baseline clotting factor level, a value that remains fairly constant throughout that person's life.
Indications for Factor Replacement Therapy
- Suspected bleeding into a joint or muscle.
- Any significant injury to the head, neck, mouth or eyes or evidence of bleeding in these areas.
- Any new or unusual headache, particularly one following trauma.
- Severe pain or swelling at any site.
- All open wounds requiring surgical closure, wound adhesive, or steri-strips.
- History of an accident or trauma that might result in internal bleeding.
- Any invasive procedure or surgery.
- Heavy or persistent bleeding from any site.
- Gastrointestinal bleeding.
- Acute fractures, dislocations and sprains.
Hemophilia A without Inhibitor
The treatment of choice for individuals with hemophilia A (factor VIII deficiency) is recombinant factor VIII or else the patient’s product of choice. Plasma-derived concentrate is a suitable alternative in an emergency situation when recombinant Factor VIII is not available. Cryoprecipate and fresh frozen plasma are no longer recommended for treatment of individuals with hemophilia A.
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%.
Mild Hemophilia A with Non-Life or Limb Threatening Bleeding
Individuals with mild hemophilia A (factor VIII greater than 5%) who are experiencing non-life or limb threatening bleeding may respond to desmopressin if they have been shown to respond to this treatment previously. Otherwise, treatment is the same as for other individuals with hemophilia A.
Hemophilia B without Inhibitor
The treatment of choice for individuals with hemophilia B (factor IX deficiency) is recombinant factor IX or else the patient’s product of choice. Plasma-derived concentrate is a suitable alternative in an emergency situation when recombinant Factor IX is not available. Fresh frozen plasma is no longer recommended for treatment of individuals with hemophilia B. Note that cryoprecipitate does not contain Factor IX.
When bleeding is severe, the appropriate dose of factor IX is 100-120 units/kg. This should result in a factor IX level of 80-100%.
- If a patient with hemophilia or the parent of a patient with hemophilia brings clotting factor with them to the emergency department, allow them to utilize it. They should be permitted to reconstitute the product and administer it whenever possible. Individuals with bleeding disorders are encouraged to have an emergency dose of factor concentrate or DDAVP in their home and to take it with them when they travel. In those situations where a patient does not bring their own clotting factor concentrate, emergency departments must be prepared to provide clotting factor replacement. Emergency departments must have ready access to factor replacement products so that they are available within 1 hour of the patient's arrival.
In order to expedite care, emergency physicians should order unreconstituted factor from their pharmacy or blood bank and reconstitute the product in the emergency department.
- Factor replacement must be administered intravenously by IV push over 1-2 minutes.
- The factor dose should be ordered as “up to the closest vial contents." The full content of each reconstituted vial should be infused, since a moderate excess of factor concentrate will not create a hypercoagulable state but will prolong the therapeutic level of the product administered; thus, it is prudent to “round up.”
4) For individuals with inhibitors (antibodies to factor VIII or IX), treatment decisions may be more complicated. The care of inhibitor patients should be urgently discussed with the patient's hematologist. If an individual with an inhibitor presents in a life- or limb-threatening scenario, the safest immediate action is to prescribe recombinant factor VIIa (rFVIIa) at a dose of 90 mcg/kg or activated prothrombin complex concentrates (FEIBA) at 75-100 units/kg.* The patient or family can also provide information on response to these therapeutic bypassing agents.
* Note: In factor IX patients with a history of inhibitors and anaphylaxis, do not give factor IX-containing products unless the bleeding is life-threatening.
- When treating an individual with mild hemophilia A who is responsive to desmopressin, the dose and prior responsiveness are usually known. The dose of desmopressin is 0.3mcg/kg subcutaneously or else intravenously in 30 ml normal saline over 30 minutes. It may also be administered as an ultra- concentrated nasal spray "Stimate" at a dose of\ 1 spray in one nostril for individuals <50 kg and 1 spray in each nostril for individuals >50 kg.
- The most experienced IV therapist or phlebotomist should perform any venipuncture. Traumatic venipunctures and repeated needle sticks cause painful hematomas that may limit further IV access.
- In any suspected bleeding emergency in which the clotting factor level of an individual with hemophilia is unknown, the factor level should be assumed to be 0%.
- Intramuscular injections should be avoided. If they must be given, factor replacement therapy must precede the injection. Parenteral agents should be given intravenously or subcutaneously. Tetanus immunizations may be administered subcutaneously.
- In situations in which patients are hemodynamically stable and are not requiring volume replacement, the smallest gauge needle should be utilized for obtaining IV access (25g butterfly needles in young infants, 23g butterfly needles in older children and adults).
- Tourniquets should not be applied tightly to extremities because they may cause bleeding.
- Aspirin and aspirin-containing products are contraindicated in individuals with hemophilia. Acetaminophen and/or codeine may be used for analgesia. Non-steroidal anti-inflammatory (NSAID) drugs may be carefully administered to select patients, such as individuals with chronic arthritic pain who are not actively bleeding or being treated for a recent bleeding problem.
- If an individual with hemophilia is bleeding and requires transportation to another facility for definitive care, all efforts should be made to replace the deficient clotting factor before transport.
This material is provided for your general information only. NHF does not give medical advice or engage in the practice of medicine. NHF under no circumstances recommends particular treatment for specific individuals and in all cases recommends that you consult your physician or local treatment center before pursuing any course of treatment.
Copyright 2017 National Hemophilia Foundation. To facilitate the dissemination of these medical recommendations, reproduction of any material in this publication in whole or in part will be permitted provided: 1) a specific reference to the MASAC recommendation number and title is included and 2) the reproduction is not intended for use in connection with the marketing, sale or promotion of any product or service. NHF reserves the right to make the final determination of compliance with this policy. For questions or to obtain a copy of the most recent recommendations, please contact the NHF Director of Communications at 1-800-42-HANDI or visit the NHF website at www.hemophilia.org.