MASAC Document #257 Replaces Doc #252
Patients & Caregivers: IMPORTANT! See Dr. Marion Koerper’s comments at end of document
The document was approved by the Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation (NHF) on December 2, 2019, and adopted by the NHF Board of Directors on December 5, 2019.
Patients 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.
Triage
1) Individuals with bleeding disorders should be triaged urgently as 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.
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 50 kg.
5) 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.
6) 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%.
7) 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).
8) 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)
9) Tourniquets should not be applied tightly to extremities because they may cause bleeding.
10) 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.
11) 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 transport.
Copyright 2019 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.
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.
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Dr Koerper’ comments
In December 2019 MASAC issued this revised version of their recommendations for management of individuals with bleeding disorders who are being seen in an Emergency Room. We recommend that you make a copy for your physician and a copy to take to the ER to give to the treating MD in the ER.
Bear in mind that most ER doctors are unfamiliar with the treatment of individuals with bleeding disorders, and most ER Pharmacies do not stock most clotting factor concentrates unless they are affiliated with a Hemophilia Treatment Center (HTC).
In order to avoid treatment problems in an ER, besides, taking this document, we make the following recommendations;
Enroll in your closest HTC. The CDC website has a list of all US HTCs, so you can find the one that is closest to you.
Once enrolled, talk to the hematologist about your diagnosis and what treatment option is best for you.
From the HTC nurse, learn how to give your treatment product, either intravenously, subcutaneously or intranasally, depending on the product.
Enroll in the HTC Home Delivery Program and order 2-4 doses of the agreed upon treatment product to be delivered to you to keep on hand in case of emergency.
Obtain from the HTC Hematologist a treatment letter that states your name, birthday, diagnosis, preferred treatment product, and dose. The letter should also include a 24-hour phone number that you and the ER doctor can use to reach the hematologist on call to confirm this information and discuss your current problem.
Call the 24-hour on call hematologist if you are unsure whether to go to the ER and which ER to go to.
If you are advised by the hematology staff to go to the ER, take a copy of these MASAC recommendations, and the letter from your hematologist. Also, take at least 4 doses of your treatment product to be used in the ER if they do not have any doses on hand.
Be sure that a dose of the treatment product is given before any procedure, such as lumbar puncture (LP), IM injection, head scan, or any surgery.
If you are not comfortable with your care, call your 24-hour hematology number.