Published: September 29, 2023
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.
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 < 1% of normal factor
- 10 % are MODERATE, with 1 – 5 % of normal factor
- Rest are classed MILD, with < 40 % normal factor
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 Resources:
End of Shift Podcast Hosts
The End of Shift Podcast is hosted by ECBC members Eric Angus and Joe Haegert.
Eric Angus is an emergency physician and trauma team leader at Lions Gate Hospital. He is married with 15-year-old twins. His non-medical interests include origami, meditation, mountain biking, skiing, rock climbing, just generally being outside, and drinking wine. He has a diploma in mountain medicine and volunteers for ski patrol and the North Shore Rescue team. He is an ATLS instructor. He dabbles in stoicism and Buddhist philosophies.
Joe Haegert practices emergency and trauma medicine at the Royal Columbian Hospital in New Westminster, British Columbia. He is a talented teacher, engaging speaker, and devoted clinician. He lives in South Surrey with his wife Sandy and managed to raise three children without much incident. Known for his unflagging enthusiasm, Joe enjoys all aspects of the outdoors and recently has taken to turning wooden burls into all manner of bowls and tables.
Disclaimer: The views and opinions expressed in this podcast are those of the authors and do not necessarily reflect the official policy or position of ECBC.
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