Sep 29 2023 47 mins 4
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 < 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 Resource:
- Treatment of Persons with Inherited Bleeding Disorders (PwIBD) in the Emergency Room
by Dr. Shannon Jackson, Michelle Bech
