NHF GUIDELINES Bundle

1. Individuals with bleeding disorders should be triaged urgently since delays in administering appropriate therapy, such as infusion of factor concentrate, can significantly affect morbidity and mortality. 2. Consultation with the patient's primary provider of bleeding disorder care, in most cases a hematologist, is strongly advised. If this provider is unavailable, consultation with a bleeding disorders provider from the closest hemophilia treatment center is recommended. Administration of clotting factor replacement to the patient should not be delayed waiting for a consultation. Triage Assessment 1. Treatment for a suspected bleeding episode is based on clinical history. Physical exam findings may be normal in the early phases of most bleeding episodes associated with an underlying bleeding disorder. Spontaneous bleeding is common in those with severe disease (baseline factor levels <1%). When in doubt, administer clotting factor replacement therapy immediately. 2. Treatment decisions should be based on the suspicion suspicion of a bleeding-related problem, not the documentation of one. 3. 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. Diagnostic Studies 1. Clotting factor replacement therapy should be given before any diagnostic studies (X-rays, CT 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. 2. 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 or bypass therapy to 100% must be administered in the emergency department prior to the planned procedure or surgery. In this situation, consultation with a hematologist is strongly recommended. 3. For an individual with known hemophilia, routine laboratory studies (PT, aPTT, factor levels), are not indicated in the treatment of a routine bleeding episode unless requested by the patient's hematologist. Treatment should not be delayed waiting for test results, which may take several hours. In some cases, screening assays such as the aPTT and factor activity assays will not be accurate depending on the product the patient is taking and/or the reagents used for the assays at the local laboratory. 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.

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