June 20, 2017 by dailybolusoflr
By: Casey Carr
Hemophilia is an X-linked recessive disorder, and is divided into hemophilia A and hemophilia B. Hemophilia A causes a deficiency in Factor VIII, and hemophilia causes a deficiency in Factor IX.
Both diseases are categorized by severity, which is represented by the level of clotting factor activity.
Severe – <1% normal clotting factor activity (35% of hemophilia A patients have severe disease, 60% of hemophilia B have severe disease)
Moderate – 1%-5% normal clotting factor activity (15% of hemophilia A have moderate disease)
Mild – 5-40% normal clotting factor activity (50% of hemophilia A have mild disease)
Patients with >40% of normal clotting factor activity are unlikely to be symptomatic
Typical acute bleeding presentations:
Hematoma and soft tissue bleeding
Many patients with known hemophilia are trained to self administer treatment when a bleeding episode begins.
Goal is to rapidly correct deficit clotting factor.
Factor level assays will NOT return while patient is in acute setting in the emergency department – in limb or life threatening presentations, assume the factor level is 0%; therefore factor dosing should be done empirically based on suspected or known injury
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