Hemophilia in the Emergency Department – Part 2

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June 22, 2017 by dailybolusoflr

By: Casey Carr


Treatment Goals

  1. Hemarthrosis
    1. Goal factor 40%-60%
    2. Hemophilia A dose of IV Factor VIII: 20-40 units/kg
    3. Hemophilia B dose of IV Factor IX: 40-50 units/kg
  2. Soft tissue hemorrhage
    1. Goal factor 40%-60%
    2. Hemophilia A dose of IV Factor VIII: 20-40 units/kg
    3. Hemophilia B dose of IV Factor IX: 40-50 units/kg
  3. Life threatening trauma
    1. Goal factor 100%
    2. Hemophilia A dose of IV Factor VIII: 50 units/kg
    3. Hemophilia B dose of IV Factor IX: 100 units/kg
  4. Hematuria
    1. Goal factor 50%
    2. Hemophilia A dose of IV Factor VIII: 20-30 units/kg
    3. Hemophilia B dose of IV Factor IX: 50-60 units/kg
  5. Gastrointestinal bleed
    1. Goal factor 100%
    2. Hemophilia A dose of IV Factor VIII: 50 units/kg
    3. Hemophilia B dose of IV Factor IX: 100 units/kg
  6. Deep laceration
    1. Goal factor 50%
    2. Hemophilia A dose of IV Factor VIII: 25 units/kg
    3. Hemophilia B dose of IV Factor IX: 50 units/kg

The above treatment regime is first dose only; depending on injury, patients may need a prolonged duration of therapy or repeat doses.


References:

National Hemophilia Foundation Guidelines for emergency department management of individuals with hemophilia. National Hemophilia Foundation, New York (2006) [MASAC Document #175

Singleton T, Kruse-Jarres R, Leissinger C. Emergency department care for patients with hemophilia and von Willebrand disease. The Journal of Emergency Eedicine. 39(2):158-65. 2010

Franchini M, Mannucci PM. Hemophilia A in the third millennium. Blood reviews. 27(4):179-84. 2013.

Mathew P, Dinter H, Church N, Humphries TJ, Kulkarni R. Inhibitors in haemophilia A: a perspective on clotting factor products as a potential contributing factor. Haemophilia : the official journal of the World Federation of Hemophilia. 22(3):334-41. 2016

Schwartz KR, Rubinstein M. Hemophilia And Von Willebrand Disease In Children: Emergency Department Evaluation And Management. Pediatric emergency medicine practice. 12(9):1-20; quiz 20-1. 2015

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