Provide factor replacement to children with hemophilia who are at risk for bleeding after traumaregardless of their clinical signs and symptoms
Provide factor replacement to children with hemophilia who are at risk for bleeding after traumaregardless of their clinical signs and symptoms: Excerpt from Avoiding Common Pediatric Errors
Author:
Emily Riehm Meier, MD
What to Do - Make a Decision
HemophiliaAandBarebleedingdisordersresultingfromdecreasedlevelsof
factor VIII and IX, respectively, and follow an X-linked inheritance pattern.
Hemarthroses and intramuscular bleeds are the hallmarks of hemophilia.
Even so, petechiae and mucosal bleeding can occur. Intracranial hemorrhage
is the leading cause of death due to bleeding in hemophiliac patients, so
special care must be taken with patients presenting with a history of head
trauma or headache.
Normal levels of factor VIII and IX activity are 50% to 200%. Patients
with <1% factor activity are considered severe hemophiliacs, those with
1% to 5% activity are considered moderate, and those with >5% activity
are considered mild. Several types of factor replacement are available and
used to treat patients with both hemophilia A and B. The vast majority of
patients have a home supply of factor; it is of utmost importance to use
of the brand of factor used at home whenever possible in an emergency
situation. Hemophiliacs should be instructed to bring a home supply of
factor with them if they have experienced trauma and need to seek care
in an emergency department. If such a patient presents with a history of
trauma and/or pain, factor should be infused immediately; even preceding
diagnostic evaluation. This caveat is especially important if the pain and/or
siteoftraumaisintracranialorintra-abdominal.Thesetwositesareofspecial
concern because of the risk of increased intracranial pressure with a head
bleedandtheriskof largeamountofbloodlossintheabdominalcavitybefore
symptoms arise. Bleeding is the cause of pain in most hemophiliac patients
and needs to be treated in a timely fashion to prevent long-term sequelae.
The dose of factor replacement is different for hemophilia A and B.
The general rule for hemophilia A is 1 unit of factor replacement per kilogram of body weight to increase the factor VIII level by 2%, whereas for
hemophilia B, 1 unit of factor replacement per kilogram increases the factor
IX level by 1%. Joint, muscle, and other minor bleeding require 40% to 50%
correction (meaning that type A hemophiliacs receive 25 U/kg of factor,
whereas hemophilia B patients receive 50 U/kg); while head, ophthalmologic, or intra-abdominal trauma demand 100% correction (50 U/kg for
hemophilia A and 100 U/kg for hemophilia B patients). Because the half-
life of factor is fairly short (8–12 hours for factor VIII and 12–24 hours for
factor IX), repeat infusion of replacement factor may be warranted, depending on the clinical situation.
Suggested Readings
Furie B, Limentani SA, Rosenfield CG. A practical guide to the evaluation and treatment of
hemophilia. Blood. 1994;84:3–9.
Roberts HR, Eberst ME. Current management of hemophilia B. Hematol Oncol Clin North Am.
1993;7:1269–1280.
>
Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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