June 29, 2017 by dailybolusoflr
By: Casey Carr
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)
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.
Guidelines recommend factor replacement when patients are required to undergo any invasive procedure.
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%.
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