NHF GUIDELINES Bundle

➤ Factor replacement must be administered intravenously by IV push over 1-2 minutes or per label instructions. ➤ 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." ➤ When treating an individual with mild hemophilia A who is responsive to DDAVP, the dose and prior responsiveness are usually known. The dose of DDAVP is 0.3 micrograms/kg subcutaneously or intravenously in 30 mL normal saline over 15-30 minutes. It may also be administered as a 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 venipunctures. 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 a patient with hemophilia is unknown, the factor level should be assumed to be 0%. ➤ Intramuscular injections, including immunizations, should be avoided whenever possible. If they must be given, factor replacement therapy must precede the injection. (It can be timed following a prophylactic dose of clotting factor concentrate. This is not required when vaccination is given 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, 23 g 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 should be avoided in individuals with hemophilia unless there is a cardiac indication, and then only under close observation for bleeding, e.g. monitoring hemoglobin levels. Acetaminophen and/or oral opioids 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 a patient 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 before transport.

• Cover