End of Shift Podcast: Bloody Well Right - Part 1

The older ED crowd will remember this classic Supertramp track from the 1974 album, Crime of the Century. It's our springboard to talk about bleeding disorders. Dr Shannon Jackson is a clinical hematologist and the medical director of the Provincial Adult Bleeding Disorder program based out of St. Paul’s Hospital in Vancouver. The program deals primarily with inherited bleeding disorders: Factor (VIII and IX) deficiencies, von Willebrand disorder, platelet disorders and undefined bleeding disorders.  This is episode 1 of 2 with Dr. Shannon Jackson.   Factor VIII or IX deficiency: VIII deficient: hemophilia A, 1 in 10,000 people and IX deficient: hemophilia B, 1 in 50,000 people X-linked recessive disorders, males have phenotypic disease, females can be obligate carriers from their fathers – but one third of emerging cases are NEW MUTATIONS 30% patients are SEVERE, with 10 % are MODERATE, with 1 – 5 % of normal factor Rest are classed MILD, with Von Willebrand disorder: Autosomal inheritance on chromosome 12, 1 in 100 people Type 1: low antigen amount, so low activity – 70 % cases Type 2: defective antigen, so poor activity – 15% cases Type 3: no vWF   FEARED bleeds are intracranial, GIB, iliopsoas, vaginal, medial forearm, anterior calf Patients are usually very well informed - perhaps more than you. They carry Factor First cards, identifying their disease, appropriate doses of factor for treatment and thankfully – the contact information for the on-call hematologist.   Steps in the ED? Reverse order! Order factor first (or ddAVP if fVIII unavailable) Then imaging, Then history. Tranexamic acid also a useful adjunct – UNLESS gross hematuria (can trigger renal and ureteric concretions)   Related Clinical Resource: Treatment of Persons with Inherited Bleeding Disorders (PwIBD) in the Emergency Room by Dr. Shannon Jackson, Michelle Bech