Bleeding Disorders - Dalhousie University

Bleeding Disorders
AARON SIBLEY MD FRCPC, DABEM
ASSISTANT PROFESSOR, DALHOUSIE UNIVERSITY
EMERGENCY PHYSICIAN, QEH CHARLOTTETOWN,
PEI
4 1 ST D A L H O U S I E E M E R G E N C Y M E D I C I N E
REFRESHER
FEB 19-21, 2015
Disclosures
 None
Coagulation Cascade
Outline
 Management of presentations of known Hemophilia
A, B, vWD
 Not covered:
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Diagnosis of unknown bleeding disorders
Acquired bleeding disorders e.g. NOACS, DOACs??
Objectives
 To think like an emergency physician when
approaching pts with a known bleeding disorder
 To discuss both transfusion and non-transfusion
treatment modalities in pts with bleeding disorders
Bleeding Disorders: Essential Information
 Type of deficiency e.g. FVIII, FIX, vWF
 Degree of factor deficiency
 Presence of inhibitors
 HIV/Hep status
FactorFirst Card
Case #1
 12 year old male involved in high-speed rollover
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MVC
Brought to ED via EMS, OPA, BVM, Board/Collar
GCS E2, V2, M4 (8), Contusion R forehead
HR 130, BP 70/40, unable to obtain sats
Mother arrives, indicates son has hemophilia A
Background
 Hemophilia: an inherited, X-linked, recessive,
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disorder resulting in deficiencies of factor VIII (A),
or IX (B)
Incidence 1/10,000 males (A), 1/25,000 (B)
Joint and muscle bleeds are hallmarks
80% of cases are A
~50% of cases have a ‘severe’ deficiency of factor
Pts may have developed inhibitors to factors
Management Priorities:
 What is the most important step in managing this
patient?
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Factor VIII replacement (Factor-CABs)
Will require repeat dosing
 What is the desired factor level in life threatening
bleeding?
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100%
 How is factor dosed/administered?
 FVIII concentrate 1 Unit/kg IV increases factor level by 2%
 FIX concentrate 1Unit/kg IV increases factor level by 1%
Case #1 Continued
 The mother indicates to you that the child has FVIII
inhibitors:
 What are your management options?
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Higher doses of FVIII
Bypass agents e.g. rFVIIa (Niastase), aPCC (FEIBA), ?PCC
(Octaplex)
Contact your hemophilia treatment centre
 What if you don’t have any factor on site?
 FFP (factor 3-5%/unit)
 Cryoprecipitate (100 units factor VIII/bag)
Case #1
 Do massive transfusion protocols and Tranexamic
Acid (TXA) apply to this population?
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Transfusion protocols should be followed as per usual (1:1:1)1
TXA may be used as an adjunct (1g over 10 mins, 1 gram over 8
hrs)2
 What other methods may be used to control
bleeding?
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Direct pressure, Proper tourniquet application
Pelvic binder
Fracture stabilization
Surgical control
1) Holcomb JB et al. Transfusion of plasma, platelets, and red blood cells in a 1:1:1 vs a 1:1:2 ratio and mortality in patients with severe trauma:
The PROPPR randomized clinical trial. JAMA 2015 ; 313:471.
2)The Crash-2 Collaboratiors, The importance of early treatment with tranexamic acid in bleeding trauma patients: an exploratory analysis of the
CRASH-2 randomised controlled trial. Lancet 2011;377:1096-1101
Hemophilia Trauma Pearls
 Even minor head injuries without significant signs of
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trauma should be managed aggressively with factor
Neck injuries can rapidly cause airway compromise
No central lines, no NG tubes until factor given
(intra-osseous devices are not contraindicated)
Hx of trauma may be remote (8 hrs to days)
Think out side the box e.g. helicopter picks up factor
on way to pt
Case #2
 50 year old male, known severe hemophilia B
 Struck left elbow on corner of counter
 Self administered 20 units/kg
 Presents to ED 6 hours later in severe pain, redness,
swelling at joint
Case #2
 What is the most important step in managing this
patient?

Early factor replacement (full factor dose in advanced bleed)
 What are some useful adjuncts to factor therapy?
 Rest- position of comfort, splint/crutches
 Ice
 Compression (gentle)
 Elevate
Case #2 Continued
 How should I control pain?
 Aggressive titration of opioids (no NSAIDs)
 Does he need an x-ray?
 No, unless there is high clinical suspicion of fracture
 Is there a role for joint aspiration?
 No, unless:
Septic arthritis suspected
 Recalcitrant pain
 Neurovascular compromise
 No improvement in 24hr with bleeding, tense, painful joint
 Inhibitors ruled out
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Mild – Moderate Bleeding Essentials
 Pts will often know when bleeding in joints occurs
despite lack of clinical evidence
 Consider alternate dx if joint isn’t improving 24hrs
after factor e.g. osteomyelitis
 Be suspicious of compartment syndrome
 No IM injections
Hemophilia Management Summary
 Early and appropriate factor replacement without
delay is paramount (but don’t forget good routine
care!)
 Factor First Card can help guide factor replacement
and management
 Early consultation with hemophilia centre is
recommended for all but minor cases
 Know the needs of your community, i.e. how many
hemophiliacs are there? What is their severity? Do
they have inhibitors?
Case #3
 25 year old female presents with epistaxis
 Local pressure for 15 minutes has not stopped the
bleed
 She has a history of type 1/mild vWD , responsive to
Desmopressin (DDAVP)
Von Willebrand Disease
 Most common congenital bleeding disorder
 Mucosal and heavy menstrual bleeding common
 vWF is a cofactor for plt adhesion, carrier of fVIII
 Three major groups:
 Type 1: partial quantitative disease (80% pts)
 Type 2: qualitative disease (15% pts)
 Type 3: severe deficiency (<10 % pts)
Case #3
 What is the role of ‘usual care’ in this pt?
 Cautery and packing are still important
 Is DDAVP useful for all pts with vWD?
 No, only for pts with type 1 who have shown they respond
 All others should receive vWF and FVIII containing
concentrate or Cryoprecipitate
 Dose prior to placing and removing any packing
 What is the role of TXA?
 Can soak cotton packing (or Merocel) with TXA 500mg/5ml
 Oral TXA 1500mg tid 5 days
vWD Summary
 Usual care is still important
 Determine responsiveness to DDAVP
 Tranexamic acid may be used for mucosal bleeds,
menorrhagia
 Those who are not responsive to DDAVP or type 2, 3
require factor replacement
Questions?
 Resources:
 World Federation of Hemophilia guidelines 2012 www.whf.org
Acknowledgements
 Dr Jennifer Fesser (Hematopathologist PEI)
 Dr Nazir Abdel Samad (Hematologist, NB)
 Dorine Beliveau RN (NB)
 Dr Sue Robinson (Hematologist, NS)
 Sue Van Oosten RN (NS)