Emergency Care BC

End of Shift Podcast: Bloody Well Right - Part 1

Episode Summary

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 acquired bleeding disorders: Factor (VIII and IX) deficiencies, von Willebrand disorder and some platelet disorders.

Episode Notes

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