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