Hemophilia in the Emergency Department – Part 3

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June 29, 2017 by dailybolusoflr

By: Casey Carr


Special considerations:

Inhibitors

Patients with congenital hemophilia may develop IgG antibodies (referred to as inhibitors) to factor VIII and factor IX replacement. This is most common in patients with severe hemophilia A, in which it can occur in up to 30% of patients. It is still not fully understood why this occurs. Many patients will know their inhibitor status, but others may not, and the presence of an inhibitor should be suspected if bleeding is not controlled with several infusions of factor concentrate. These patients require urgent consultation with hematology. The strategy for correcting deficit clotting factors in the presence of an inhibitor utilizes “by-passing” agents, named for the ability to bypass specific clotting factors in the coagulation cascade. There are two bypassing agents available to these patients: activated recombinant factor VIIa and factor eight inhibitor bypassing activity (FEIBA)

Elderly

Hemophilia treatment has improved over the past twenty years, with a concomitant increase in life expectancy. As such, patients with hemophilia are being faced with increasing co-morbidities . The incidence of hypertension, dyslipidemia, and diabetes is higher in patients with hemophilia. Of note, treatment of cardiovascular events is complex and requires urgent collaboration with hematology.

Procedures

Guidelines recommend factor replacement when patients are required to undergo any invasive procedure.


Pearls:

Factor concentrates cannot be administered with other fluids; place additional venous access sites with this in mind.

Intramuscular injections should be avoided if possible

Iliopsoas hemorrhage is a soft tissue bleed that requires a high index of suspicion, as it can cause severe motor limitations and house large volumes of blood. Patients will commonly present with groin and hip pain, femoral paresthesias, and hip contractures. The goal factor level is 80%.


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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