Bleeding Disorders What Every ER RN Should KNOW! Sherry Purcell, RN Hemophilia Nurse Specialist/EMERGENCY RN Kingston General Hospital

Transcription

1 Bleeding Disorders What Every ER RN Should KNOW! Sherry Purcell, RN Hemophilia Nurse Specialist/EMERGENCY RN Kingston General Hospital 1

2 Canada s Inherited Bleeding Disorder Centres 26 Hemophilia A, B Von Willebrand disease Rare factor deficiencies Platelet function disorders

3 Objectives vonwillebrand Disease (VWD) 101J Brief History/Pathophysiology Symptoms of VWD Emergency Management Case Studies Lessons learned

4 Our Focus Many emergency nurses and physicians find this disease confusing So you have von willie what?

5 Dr. Eric von Willebrand first patient described died with her 3 rd menstrual period

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7 Normal Coagulation Damaged Vessel Bleeding Vasoconstricti on Reduces blood volume through vessel Platelet Plug 1. Contact 2. Adhesion VWF needed 3. Aggregation DiPiro J, ed. Pharmacotherapy, 6th edition, Ch100 Coagulation Disorders, Vessel Wall and Platelets, paragraph #1. New York, New York: McGraw-Hill; 2005, pg Clotting 1. Coagulation cascade forms fibrin 2. Platelets adhere to fibrin forming clot core

8 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.

9 VWD Symptoms Easy bruising Epistaxis + Dental bleeding Prolonged bleeding from lacerations, muscle & joint injuries Occult bleeding.. Head + belly injuries Menorrhagia/massive vaginal hemorrhage

10 VWD Types Several types: 1, 2, 3(most severe) Defect in VWD not on sex chromosome as with Hemophilia, therefore men & women are equally affected. HOWEVER.

11 However 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

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13 2008 CAPE ER PHYSICIAN-ON CHS ER ADVISORY COMMITTEE

14 Factor First Cards 14

15 FACTOR FIRST CARD INCLUDES : 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

16 ER RNS VWD Alert 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.

17 Factor Replacement Plasma-derived VWF/FVIII concentrate Humate-P and Wilate serious bleeding, major surgery Prophylaxis

18 ER RN ALERTS Possibility of Occult Bleeding (same for all Hemophilia patients) HEAD, NECK, SPINE SPLEEN,LIVER,GI,PELVIS COMPARTMENT SYNDROME ILIOPSOAS

19 CASE # 1 12 yr old girl VWD TYPE 3 1 st PERIOD AUG pad q 4hrs 2 nd PERIOD NOV 2012, 14 day duration pad changes 2to3hrs for 3 days, then tapered 3 rd PERIOD Nov29 1 to 2 pads hourly+large clots.home from school 3 days.

20 CASE # 1 DEC 5 th 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%

21 CASE # 1 IV N/S HUMATE P 2400RiCof u. IV (piggy-backed in mini bag) Bloodwork, (cbc,ferritin,type&hold)

22 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

23 MORE TO THIS STORY 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

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25 LESSONS LEARNED TAKE THIS DISEASE SERIOUSLY RECOLLECTION OF DEATH OF FINNISH GIRL AT AGE AT AGE 14(3 rd Period) IMMEDIATE + APPROPRIATE TREATMENT BRINGS QUICK RESOLUTION OF BLEEDING!

26 CASE # 2 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

27 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

28 CASE # 2 5 days in Peds ICU then to floor Discharged home Aug 27 th Non wt-bearing leg cast Shoulder immobilizer

29 LESSONS LEARNED 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.

30 CASE #3 Dr. Michelle Sholzberg Hematologist, St Michael s Hospital Comprehensive Care Centre 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

31 CT Abdomen

32 CASE # 3 SENT HOME.no Humate given MARCH 7 th 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.

33 LESSONS LEARNED Believe the Patient Give FACTOR FIRST If bleed is suspected: CALL Bleeding Disorder Clinic staff DON T be reassured by a stable HGB

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35 NOW WHEN THE PARAMEDIC LINE RINGS IN YOUR ER HOPE NO REACTIONS LIKE THIS

36 WE ARE READY TO GO!

37 Thank you!