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Bleeding  Disorders  What  Every  ER  RN  Should  KNOW!                        

Sherry  Purcell,  RN  Hemophilia  Nurse  Specialist/EMERGENCY  RN  

Kingston  General  Hospital    

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Canada’s Inherited Bleeding Disorder Centres

Hemophilia A, B ⏐ Von Willebrand disease

Rare factor deficiencies ⏐ Platelet function disorders

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vonWillebrand Disease (VWD) 101J * Brief History/Pathophysiology * Symptoms of VWD * Emergency Management * Case Studies * Lessons learned

Objectives

* Many emergency nurses and physicians find this disease confusing

*  “So you have von

willie……what?”

Our Focus

Dr. Eric von Willebrand

1926 - first patient described died with her 3rd menstrual period

Damaged Vessel §  Bleeding

Vasoconstriction §  Reduces

blood volume through vessel

Platelet Plug 1. Contact 2. Adhesion

VWF needed 3. Aggregation

Clotting 1. Coagulation

cascade forms fibrin

2. Platelets adhere to fibrin forming clot core

Normal Coagulation

§  DiPiro J, ed. Pharmacotherapy, 6th edition, Ch100 Coagulation Disorders, Vessel Wall and Platelets, paragraph #1. New York, New York: McGraw-Hill; 2005, pg. 1833.

* Most common, inherited bleeding disorder 1in1000 have symptomatic VWD Bowman JTH 2010; Bowman Ped Blood and Cancer 2010

* Excessive bleeding skin, mucous membranes * Inherited ♀=♂, ♀ in 1-2% of pop worldwide * ? 30,000 individuals in Canada.

* Easy bruising * Epistaxis + Dental bleeding * Prolonged bleeding from lacerations, muscle

& joint injuries * Occult bleeding.. Head + belly injuries * Menorrhagia/massive vaginal hemorrhage

VWD Symptoms

*  Several types: 1, 2, 3(most severe)

* Defect in VWD not on sex chromosome as with Hemophilia, therefore men & women are equally affected.

HOWEVER………….

VWD Types

* Women affected the most from heavy prolonged menstrual bleeding * hourly maxipad changes…off school/work * Periods lasting 10 to 14 days * chronic anemia * women at risk of postpartum bleeding plus

from ruptured ovarian cysts

However…

2008 CAPE ER PHYSICIAN-ON CHS ER ADVISORY COMMITTEE

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Factor First Cards

 Documentation of: 1. Particular clotting factor Deficiency 2. Exact dose for a major bleed (HUMATE

P or Wilate for VWD), 3. Treatment for a moderate bleed 4. Signed by their hematologist 5. Advised to carry on person always If a Hemophilia Pt(kogenate,advate,xyntha

FACTOR FIRST CARD INCLUDES :

* Replace VWD Factor by IV = the FIRST course of action for a MAJOR BLEED!!

* Then DIAGNOSTIC TESTS: Bloodwork,CT,Ultrasound, Xrays,

LATER…the same for Hemophilia PTS.

ER RNS VWD Alert

Factor Replacement

* Plasma-derived VWF/FVIII concentrate

– Humate-P and Wilate – serious bleeding, major surgery – Prophylaxis

* Recombinant VWF – in clinical trials

Possibility of Occult Bleeding

(same for all Hemophilia patients) HEAD, NECK, SPINE SPLEEN,LIVER,GI,PELVIS COMPARTMENT SYNDROME ILIOPSOAS

ER RN ALERTS

*  12 yr old girl VWD TYPE 3

*  1st PERIOD AUG 2012 1 pad q 4hrs

*  2nd PERIOD NOV 2012, 14 day duration –pad changes 2to3hrs for 3 days, then tapered

*  3rd PERIOD Nov29 1 to 2 pads hourly+large clots….home from school 3 days.

CASE # 1

*  DEC 5th father called clinic office *  Advised to give Tranexamic acid 1gm po *  Peds Hematology notified *  Girl brought to COPC for assessment (very pale, dizzy,

passing fist sized clots

*  B.P. 97/65 P. 118 O2Sat 100%

CASE # 1

* IV N/S * HUMATE P 2400RiCof u. IV (piggy-backed

in mini bag) * Bloodwork, (cbc,ferritin,type&hold)

CASE # 1

* HGB-87 FERRITIN =8 (n.30 to 400) * 1400hrs posturals: BP 101/63 lying P 100 BP 105/65 standing, Pulse 150 Peds Hematology notified …decided to discharge girl, + to continue Tranexamic acid tabs 1gm TID & have Mom retreat with Humate at 11pm

CASE # 1

* Within 1 hour, call from Mom…daughter bled through 2 sets of clothes at home. * Peds Hematologist notified…to KGH ER *  IV + HUMATE given..PEDS Service Notified

Bloodwork Hgb now 61) *  T&C (2 units given + IV Premarin * Bleeding resolved- 2 days..home on Yaz BCP * Follow up at our Women’s Clinic

MORE TO THIS STORY

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TAKE THIS DISEASE SERIOUSLY

RECOLLECTION OF DEATH OF FINNISH GIRL AT AGE AT AGE 14(3rd Period)

IMMEDIATE + APPROPRIATE TREATMENT

BRINGS QUICK RESOLUTION OF BLEEDING !

LESSONS LEARNED

* 10 yr old girl (VWD Type 1) * Tubing accident Aug/12,Mom injured * Transferred from local hospital to our KGH

trauma centre…NO FACTOR FIRST card !! * ER physicians ordered Cryo + platelets…

until Peds Hematologist changed order to Humate…given IV immediately

CASE # 2

* INJURIES…pneumothorax, tibial # tripod facial #, clavicle + rib #s * Sedated for chest tube * Then for CT scanning ,more Xrays * Humate P ordered q 24 hrs * To PEDS ICU

CASE # 2

* 5 days in Peds ICU then to floor * Discharged home Aug 27th * Non wt-bearing leg cast *  Shoulder immobilizer

CASE # 2

* ER PHYSICIANS/Trauma Team * REQUIRE MORE EDUCATION re CURRENT

TREATMENTS FOR VWD…Cryo no longer used in Canada as a first line.(remote communities only) *  Involve Hematology upon arrival to ER if possible *  Importance of pts wearing medic alert…………

Factor First card may not always be with them.

LESSONS LEARNED

* TYPE 3 VWD female age 36, with documented low VWD factor levels, given Humate P in 2010 for Right hemorrhagic ovarian cyst. * March 5, 2012 presented to the ER with RLQ pain

requesting Humate P. * ER Dr. assessed.. .bloodwork Hgb 130g/L, * Referred to GYN service..ordered a CT

CASE #3 Dr. Michelle Sholzberg Hematologist, St Michael’s Hospital

Comprehensive Care Centre

CT Abdomen

*  SENT HOME ….no Humate given * MARCH 7th back to ER ++abdominal pain nausea,

vomiting * Hemodynamically stable * ULTRASOUND ---9x7x5.6cm R. Hemorrhagic Ovarian

Cyst *  SENT HOME WITH ANALGESIA *  11pm returned to the ER. DR Sholzberg contacted…

Humate P IV x 3days + TAA po * RAPID IMPROVEMENT.. No Blood Products.

CASE # 3

Believe  the  Patient  

Give  FACTOR  FIRST  If  bleed  is  suspected:  

CALL  Bleeding  Disorder  Clinic  staff  DON’T  be  reassured  by  a  stable  HGB  

LESSONS LEARNED

*           

NOW  WHEN  THE  PARAMEDIC  LINE  RINGS  IN  YOUR  ER    

HOPE  NO  REACTIONS  LIKE  THIS  

WE ARE READY TO GO!

Thank you!