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Emergency Care for Patients with Von Willebrand Disease an Instructional Manual for Medical Professionals

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Emergency Care for Patients with Von Willebrand Disease an Instructional Manual for Medical Professionals Emergency Care for Patients with von Willebrand Disease An instructional manual for Medical Professionals Authors and Editors: Susan C. Zappa RN, Lucie Lacasse RN, Rose Jacobson RN, Sherry Purcell RN and Karen Wulff RN Medical Reviewers: David Lillicrap MD, FRCPC and Marcela Torres MD von Willebrand Disease (VWD) is classified by 'type 1, 2, or 3' If the Mucous membrane type is unknown proceed as if type 1, bleeds (pg. 6) if bleeding continues consult a Administer the hematologist. recommended treatment and anti-fibrinolytics Gynecological bleeds (pg. 12) Immobilizers Ice pack p.r.n. for joint bleeds for soft tissue, muscle, joint bleeds Abdominal bleeds (pg. 10) For VWD type 3: Avoid intra- Trauma (pg. 23) muscular injections due to the Administer the recommended possibility of causing a muscle treatment bleed Minor cuts / bruises no treatment Head Injury (pg. 4) Always treat immediately with the recommended treatment Treatment and Management Guidelines for von Willebrand Disease Type of von Major life-threatening bleeds Other bleeds Willebrand (ex. - head injury, GI bleeding, severe (ex. - sutures, nosebleed, mouth bleed, dental Disease menorrhagia, etc.) extractions etc.) Type 1 or Type 2 Factor concentrate containing both Known to respond to desmopressin FVIII (8) and von Willebrand factor (DDAVP®): (eg. Humate P®, Alphanate® , wilate®): 60-80 Ristocetin cofactor units/kg IV Desmopressin 0.3 mcg/kg IV in 50 ml of Normal Saline over 30 minutes or The type 2 VWD Package insert will instruct as to rate per subcutaneously if volume can be given known as ‘pseudo volume. safely. Recommendation: a maximum dose VWD or VWF of 20 mcg. platelet type' will Note: monoclonal or recombinant only respond factor VIII (8) products do NOT have Mucosal bleeding - anti-fibrinolytics (pg. 9) to a platelet von Willebrand factor in them and transfusion - call will not stop the bleeding. For patients who do not respond to desmopres- a hematologist. sin: Give a factor concentrate containing both FVIII (8) and von Willebrand factor (eg. Humate P®, Alphanate®, wilate® ): 40-60 Ristocetin cofactor units/kg IV Package insert will instruct as to rate per volume. Note: monoclonal or recombinant factor VIII (8) products do NOT have von Wil- lebrand factor in them and will not stop the bleeding. Type 3 Factor concentrate containing both Factor concentrate containing both FVIII (8) and von Willebrand factor FVIII (8) and von Willebrand factor (eg. Humate P®, Alphanate®, wilate® ): (eg. Humate P®, Alphanate®, wilate® ): Most severe form 60-80 Ristocetin cofactor units/kg 40-60 Ristocetin cofactor units/kg of VWD. Package insert will instruct as to rate per Package insert will instruct as to rate per volume. volume. Note: monoclonal or recombinant Note: monoclonal or recombinant factor factor VIII (8) products do NOT VIII (8) products do NOT have von Wil- have von Willebrand factor in them lebrand factor in them and will not stop the and will not stop the bleeding. bleeding. If your institution does not have Humate-P®, or Alphanate®, wilate®, but does have Koate DVI® available, consult a hematologist for guidelines and instructions. Per the Medical and Scientific Advisory Council of the National Hemophilia Foundation: Because of the increased risk of HIV and hepatitis A, B, and C transmission, cryoprecipitate should not be used (for the treatment of von Willebrand Disease) except in an emergency situation where one of the above products is not available and delay of treatment would be life or limb threatening. Dose Chart Content Centers Hemophilia Treatment Centres - Canada Basics Intro BRITISH COLUMBIA Bleeding Disorders Program Hemophilia Clinic London Health Science Ctr. Montreal Children’s Hospital Victoria Hospital Montréal, QC Hemophilia Program of BC (Adult Division) London, ON Tel: (514) 412-4420 St. Paul’s Hospital Tel: (519) 685-8500 ext. 53582 After hours: (514) 412-4400 #23333 Vancouver, BC Tel: (604) 806-8855/1-877-806-8855 Hemophilia Clinic Head Hemophilia Program After hours: (604) 682-2344 Ste-Justine Hospital Thunder Bay Regional Hospital Montréal, QC Science Centre Tel: (514) 345-4931 #6031 Pediatric Hemophilia/ Hematology Thunder Bay Pagers: (514) 415-5573 /5584 BC Children’s Hospital Tel: (807) 684 - 7200 Vancouver, BC After hours: (514) 345-4788 Mucous After Hours: (807) 623-7451 Membr. Tel: (604) 875-2345 ext. 5335 Pager: (604) 875-2161 Quebec Centre for Inhibitors After hours: (604) 875-2161 Comprehensive Hemophilia of Coagulation Care Centre Hemophilia Clinic St. Michael’s Hospital Ste-Justine Hospital ALBERTA Toronto, ON Montréal, QC Tel: (416) 864-5129 Tel: (514) 345-2360 GI / GU Southern Alberta Hemophilia Program Pager: (416) 685-9404 Alberta Children’s Hospital After hours: (416) 864-5431 Regional Hemophilia Centre for Calgary, AB Eastern Quebec Tel: (403) 955-7311 Hôpital de l’ Enfant Jésus After hours: (403) 955-7070 Hemophilia Program Québec, QC Hospital for Sick Children Tel: (418) 649-5624 Southern Alberta Rare Blood Toronto, ON & Bleeding Disorders (Adults) Tel: (416) 813-5871 NEW BRUNSWICK Gyn. Foothills Medical Centre Pager: (416) 377-9716 Calgary, AB After hours: (416) 813-7500 Horizon Health Network Tel: (403) 944-4057 Zone 1, Monction After hours: (403) 944-1110 Hematology Clinic Hemophilia Clinic Joint / Muscle Children’s Hospital of Eastern Ontario Monction, NB Soft Tissue Comprehensive Centre for Bleeding Disorders Ottawa, ON Tel: (506) 857-5465 / 857-5467 University of Alberta Hospital/ Tel: (613) 737-7600 ext. 2368 Emergency line: (506) 874-9561 / Stollery Children’s Hospital 1-888-475-9922 Edmonton, AB Regional Comprehensive Care Centre Tel: (780) 407-6588 for Hemophilia & Hemostasis Inherited Bleeding Disorder Clinic Pager: (780) 445-1683 (Adult Program) Saint John Regional Hospital Ottawa Hosp. Gen. Campus Saint John, NB Desmopressin Tel: (506) 648-7286 SASKATCHEWAN Ottawa, ON Tel: (613) 737-8252 Pager: (506) 646-3757 After hours: (613) 722-7000 Saskatchewan Bleeding Disorders Program NOVA SCOTIA Royal University Hospital Sudbury & North-Eastern Ontario Saskatoon, SK Hemophilia Program, Pediatric Bleeding Disorder Clinic Tel: (306) 655-6504 Sudbury Regional Hospital IWK Health Centre After hours: (306) 655-6424 Sudbury, ON Halifax, NS Pager - RN: (306) 655-1000 #10258 Tel: (705) 523-7059 Factor Tel: (902) 470-8752 / 470-8819 Meds After hour emergencies: MANITOBA Southeastern Ontario Regional (902) 470-8888 Inherited Bleeding Disorders Program MB Bleeding Disorders Program Kingston General Hospital Hereditary Bleeding Disorders Health Sciences Centre Douglas 3 Program - Adult Winnipeg, MB Kingston, ON QE II Health Science Centre Tel: (204) 787-2465 X-rays Tel: (613) 549-6666 ext. 4683 Halifax, NS Labs Pager: (204) 787-2071 #3346 Tel: 24 HR # (613) 548-3232 Tel: (902) 473-5612 ONTARIO QUÉBEC NEWFOUNDLAND Hemophilia Program Bleeding Disorders Program Hemophilia Clinic Janeway Site Hamilton Health Sciences Corporation CHUS - Hôpital Fleurimont McMaster Division Eastern Health Sherbrooke, QC St. John’s, NL Hamilton, ON Tel: (819) 346-1110 ext. 14560 Tel: (905) 521-2100 #75978 Tel: (709) 777-4388 24 hour: (905) 521-2100 ext 76443 After hours Tel: (709) 777-6300 Centers Content Hemophilia Treatment Centers - USA ALABAMA ARKANSAS University of California at Davis Hemophilia Program Children’s Rehabilitation Services Arkansas Center for Bleeding Disorders 2360 Stockton Blvd. Ste 1100 Head 1610 Center St. Suite A Arkansas Children’s Hospital Davis One Building Mobile, AL 36604 800 Marshall Sacramento, CA 95817 Phone: (251) 432-4560 Little Rock, AR 72202 Phone: (916) 734-3461 Pediatric after hours: (251) 405-5115 Phone: (501) 364-5961 Adult after hours: (916) 734-2011 After hours: (501) 364-1100 Pediatric after hours: (916) 734-3591 Children’s Rehabilitation Services PO Drawer 2328 CALIFORNIA University of California, San Diego Membr. Mucous Birmingham, AL 35201-2328 200 W. Arbor Drive M/S 0821 Phone: (205) 939-5900 San Diego, CA 92103 Children’s Hospital Oakland Adult after hours: (205) 934-3411 Phone: (619) 471-0335 Div of Hematology/Oncology Pediatric after hours: (205) 939-9100 Adult after hours: (619) 290-5539 747 52nd Street Oakland, CA 94610-4131 Children’s Rehab. Services Phone: (510) 428-3286 University of California, San Francisco GI / GU 407 Governor’s Dr SW, Ste B Hemophilia Program Huntsville, AL 35801 650 Moffitt, Box 0106 Children’s Hospital of Central California Phone: (256) 518-8650 San Francisco, CA 94143 Hematology/Oncology Phone: (415) 476-1280 9300 Valley Children’s Place Adult after hours: (415) 353-2421 University of Alabama Madera, CA 93638 Birmingham Medical Center Gyn. Phone: (559) 353-5460 1600 7th Ave S ACC 512 Pediatric after hours: (559) 353-5460 COLORADO Birmingham, AL 35233 Phone: (205) 939-9285 Adult after hours: (205) 934-3411 Children’s Hospital of Los Angeles Mountain States Regional Hemophilia Pediatric after hours: (205) 939-9100 Hem/Onc 4650 Sunset Boulevard, Box #54 and Thrombosis Center Los Angeles, CA 9002 PO Box 6507 MS F416 Phone: (323) 669-4141 Aurora, CO 80045-0507 ALASKA After hours: (323) 660-2450 Phone: (303) 724-0362 Soft Tissue Joint / Muscle Adult after hrs: (303) 372-0000 Alaska Hemophilia Association Children’s Hospital of Orange County Ped. After hours: (303) 861-6740 and Treatment Center Dept of Hem/Oncology 16958 N. Eagle River Loop 455 South Main Street CONNECTICUT Eagle River, AK 99577 Orange, CA 92868 Phone: (907) 622-4045 Phone: (714) 532-8459 After hours: (907) 268-1190 Adult after hours: (714) 765-6677 UCONN Hemophilia Treatment Center Desmopressin Pediatric after hours: (714) 765-6677 Univ of Conn Health Center University Cancer Center ARIZONA 263 Farmington Ave. Children’s Hospital, San Diego Farmington, CT 06030 3020 Children’s Way Phone: (860) 679-2576 Mountain States Regional San Diego, CA 92123 Hemophilia Center – Tucson Adult after hours: (860) 679-2000 Meds Phone: (858) 966-5811 Factor Univ. of AZ H/S Center 1501 North Campbell Avenue Yale University School of Medicine Tucson, AZ 85724 City of Hope National Medical Center Yale-New Haven Hemophilia Ctr.
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