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 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 / 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, , mouth bleed, dental Disease menorrhagia, etc.) extractions etc.)

Type 1 or Type 2 Factor concentrate containing both Known to respond to FVIII (8) and (DDAVP®): (eg. Humate P®, Alphanate® , wilate®): 60-80 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. 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, 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 Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays

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es - Canada Centr es - Treatment Hemophilia Tel: (519) 685-8500 ext. 53582 (519) Tel: 684 - 7200 (807) Tel: After Hours: (807) 623-7451 (416) 864-5129 Tel: (416) 685-9404 Pager: After hours: (416) 864-5431 (416) 813-5871 Tel: (416) 377-9716 Pager: After hours: (416) 813-7500 (613) 737-7600 ext. 2368 Tel: (613) 737-8252 Tel: After hours: (613) 722-7000 (705) 523-7059 Tel: (613) 549-6666 ext. 4683 Tel: 24 HR # (613) 548-3232 Tel: Tel: (819) 346-1110 ext. 14560 Tel: Bleeding Disorders Program Bleeding Disorders Science Ctr. London Health Hospital Victoria London, ON Hemophilia Program Hospital Regional Bay Thunder Science Centre Bay Thunder Hemophilia Comprehensive Centre Care Hospital St. Michael’s ON Toronto, Hemophilia Program Children Sick for Hospital ON Toronto, Clinic of Eastern Ontario Hospital Children’s ON Ottawa, Centre Care Comprehensive Regional Hemophilia & for (Adult Program) Gen. Campus Hosp. Ottawa ON Ottawa, Sudbury & North-Eastern Ontario Hemophilia Program, Hospital Sudbury Regional ON Sudbury, Southeastern Ontario Regional Program Inherited Bleeding Disorders Hospital Kingston General 3 Douglas Kingston, ON QUÉBEC Hemophilia Clinic Fleurimont CHUS - Hôpital QC Sherbrooke, Tel: (905) 521-2100 #75978 Tel: 24 hour: (905) 521-2100 ext 76443 Tel: (204) 787-2465 Tel: (204) 787-2071 #3346 Pager: Tel: (306) 655-6504 Tel: After hours: (306) 655-6424 - RN: (306) 655-1000 #10258 Pager Tel: (403) 944-4057 Tel: After hours: (403) 944-1110 (780) 407-6588 Tel: (780) 445-1683 Pager: Tel: (403) 955-7311 Tel: After hours: (403) 955-7070 Tel: (604) 875-2345 ext. 5335 (604) Tel: (604) 875-2161 Pager: After hours: (604) 875-2161 Tel: (604) 806-8855/1-877-806-8855 (604) Tel: After hours: (604) 682-2344 Hemophilia Program Hemophilia Program Sciences Corporation Hamilton Health McMaster Division Hamilton, ON ONTARIO Program MB Bleeding Disorders Sciences Centre Health MB Winnipeg, MANITOBA SASKATCHEWAN Program Bleeding Disorders Saskatchewan Hospital University Royal SK Saskatoon, Southern Alberta Rare Blood Southern & Bleeding Disorders (Adults) Foothills Medical Centre AB Calgary, Disorders Bleeding for Centre Comprehensive Hospital/ of Alberta University Hospital Stollery Children’s Edmonton, AB Southern Alberta Hemophilia Program Hospital Alberta Children’s AB Calgary, ALBERTA Pediatric Hemophilia/ Hematology Hemophilia/ Hematology Pediatric Hospital BC Children’s BC Vancouver, Hemophilia Program of BC (Adult Division) of BC (Adult Program Hemophilia Hospital St. Paul’s BC Vancouver, BRITISH COLUMBIA Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content Phoenix, AZ85016 1919 E.Thomas Rd O/P BuildingB Hemophilia Ctr Phoenix Children’s Hospital Tucson, AZ85724 1501 NorthCampbellAvenue Univ. ofAZH/SCenter Hemophilia Center–Tucson Mountain States Regional ARIZONA Eagle River, AK99577 16958 N. Eagle River Loop and Treatment Center Alaska HemophiliaAssociation ALASKA Birmingham, AL35233 1600 7thAve SACC 512 Birmingham Medical Center University ofAlabama Huntsville, AL35801 407 Governor’s DrSW, SteB Children’s Rehab. Services Birmingham, AL35201-2328 PO Drawer 2328 Children’s Rehabilitation Services Mobile, AL36604 1610 CenterSt.SuiteA Children’s Rehabilitation Services ALABAMA After hours:(602)546-0920 Phone: (602)546-0920 After hours:(520)694-6000 Phone: (520)626-7584 After hours:(907)268-1190 Phone: (907)622-4045 Pediatric afterhours:(205)939-9100 Adult afterhours:(205)934-3411 Phone: (205)939-9285 Phone: (256)518-8650 Pediatric afterhours:(205)939-9100 Adult afterhours:(205)934-3411 Phone: (205)939-5900 Pediatric afterhours:(251)405-5115 Phone: (251)432-4560 Hemophilia TreatmentCenters-USA ARKANSAS Palo Alto, CA94304 Mail Code5798 1000 Welch Road, Suite300 Div ofHematology/Oncology Stanford University Medical Center Los Angeles, CA90007 2400 S. Flower Street Hemophilia Program Orthopaedic Hospital ofLosAngeles MOB 4Duarte, CA91010 1500 E.DuarteRd Hemophilia Trt Center City ofHopeNational Medical Center San Diego, CA92123 3020 Children’s Way Children’s Hospital, SanDiego Orange, CA92868 455 SouthMainStreet Dept ofHem/Oncology Children’s Hospital ofOrange County Los Angeles, CA9002 Hem/Onc 4650SunsetBoulevard, Box #54 Children’s Hospital ofLosAngeles Madera, CA93638 9300 Valley Children’s Place Hematology/Oncology Children’s Hospital ofCentral California Oakland, CA94610-4131 747 52ndStreet Div ofHematology/Oncology Children’s Hospital Oakland CALIFORNIA Little Rock, AR72202 800 Marshall Arkansas Children’s Hospital Arkansas Centerfor BleedingDisorders Phone: (650)497-8953 After hours:(213)742-1162 Phone: (213)742-1357 Phone: (626)301-8426 Phone: (858)966-5811 Pediatric afterhours:(714)765-6677 Adult afterhours:(714)765-6677 Phone: (714)532-8459 After hours:(323)660-2450 Phone: (323)669-4141 Pediatric afterhours:(559)353-5460 Phone: (559)353-5460 Phone: (510)428-3286 After hours:(501)364-1100 Phone: (501)364-5961 San Francisco, CA94143 650 Moffitt,Box 0106 Hemophilia Program University ofCalifornia, SanFrancisco San Diego, CA92103 200 W. ArborDrive M/S0821 University ofCalifornia, SanDiego Sacramento, CA95817 Davis OneBuilding 2360 Stockton Blvd. Ste1100 Hemophilia Program University ofCalifornia at Davis COLORADO Newark, DE19718 4755 Ogletown-Stanton Rd. Christiana Hospital Hemophilia Program, L-214 Christiana Care Health Services DELAWARE Haven,New CT06510 333 CedarStreet Ped LMP4087 of Dept. Yale-New Haven HemophiliaCtr. Yale University School ofMedicine Farmington, CT06030 263 Farmington Ave. University CancerCenter Univ ofConnHealth Center UCONN HemophiliaTreatment Center CONNECTICUT Aurora, CO80045-0507 PO Box 6507MSF416 and Center Mountain States Regional Hemophilia Adult afterhours:(415)353-2421 Phone: (415)476-1280 Adult afterhours:(619)290-5539 Phone: (619)471-0335 Pediatric afterhours:(916)734-3591 Adult afterhours:(916)734-2011 Phone: (916)734-3461 Adult afterhours:(302)737-7700 Phone: (302)733-3542 Phone: (203)785-4640 Adult afterhours:(860)679-2000 Phone: (860)679-2576 Ped. 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IL 60612 Chicago, University Northwestern for Center Northwestern Bleeding Disorders Clair Suite 850 St 676 N. IL 60611 Chicago, Center Medical University Rush Section of Pediatric Hematology/Oncology Suite 718 PBI Harrison Street 1725 W. IL 60612-3833 Chicago, Hospital Children’s Stroger of Cook County Hem/Oncology Dept of Ped St., Rm. 7206 Wood 700 S. IL 60612 Chicago, Hemophilia Treatment Centers - USA Centers Treatment Hemophilia Phone: (208) 381-2782 After hours: (208) 327-8007 Phone: (808) 983-8551 After hours: (808) 524-2575 Phone: (404) 785-3240 Phone: (404) 785-3240 After hours: (404) 785-3240 Phone: (404) 727-1608 778-5000 Adult after hours: (404) after hours: (404) 778-5000 Pediatric Phone: (770) 518-8272 Phone: (706) 721-0870 After hours: (706) 721-2505 Phone: (706) 721-3626 IDAHO Hemophilia Center Idaho Regional Inst. Tumor Mountain States Hem/Onc Peds. 100 East Idaho Street ID 83712-6297 Boise, HAWAII Hemophilia and Thrombosis Center of Hawaii Med Center for Kapi’olani and Children Women 1319 Punahou Street Honolulu, HI 96826 Children’s Healthcare of Healthcare Children’s Rite Scottish Atlanta at Ctr & Blood Aflac Cancer Service Disorders Rd, Ste 400 Mark 5455 Meridian 30342 Atlanta, GA Hemophilia Emory University Office Program NE Dr. 2015 Uppergate 30322 Atlanta, GA Inc. Hemophilia of Georgia, Ste 170 Road, 8800 Roswell 30350 Atlanta, GA - Adult of Georgia College Medical Dept of Adult Hem/Onc 1120 15th St BAA-5407 30912-3125 GA Augusta, of Georgia College Medical Hem. Program Pediatric Hem/Onc Dept of Pediatric BG-2013 Street, 1446 Harper 30912-3730 GA Augusta, Phone: (912) 350-7285 after hours: (912) 658-3017 Pediatric Phone: (813) 972-7582 Phone: (904) 390-3789 (904) 390-3600 after hours: Pediatric Phone: (305) 585-5635 (305) 585-5400 after hours: Pediatric Phone: (727) 767-4176 (727) 562-6862 after hours: Pediatric Phone: (202) 687-0117 687-7243 Adult after hours: (202) Phone: (202) 884-3622 after hours: (202) 884-5000 Pediatric Backus Children’s Hospital Children’s Backus Avenue 4700 Waters 23089 Box P.O. 31403-3089 GA Savannah, GEORGIA University of South Florida - Adult of South Florida University Hospital James A. Haley V.A. Hematology-111-R Blvd Downs 13000 Bruce B. FL 33612 Tampa, Nemours Children’s Clinic Children’s Nemours Hem/Onc of Ped. Division Way 807 Children’s FL 32207 Jacksonville, Miami Comprehensive Hemophilia Miami Comprehensive Center - Pediatrics of Miami University Dept. of (R-131) Pediatrics Miami, FL 33101 FLORIDA Hospital All Children’s Hem/Onc Associates Ped 880 6th St So Ste 140 FL 33701 St. Petersburg, Georgetown University Hospital University Georgetown Cancer Center, Lombardi of Hem/Onc Division NW Road, 3800 Reservoir DC 20007 Washington, DISTRICT OF COLUMBIA OF COLUMBIA DISTRICT Center Medical National Children’s Dept of Hem/Oncology NW Avenue, 111 Michigan DC 20010 Washington, Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content Iowa City, IA52242 Ped 2507JCP of Dept Iowa Reg. HemophiliaCenter University ofIowa Hospitals &Clinics IOWA Scarborough, ME04074 100 USRoute 1,Unit104 Maine Hemophilia&Thrombosis Center Maine Medical Center MAINE Orleans,New LA 70112 1430 Tulane Av Box TB-31 Tulane Univ. School ofMed. and ClottingDisorders Louisiana Ctrfor Bleeding LOUISIANA Lexington, KY40536-0284 740 SouthLimestoneStreet J457 Kentucky Clinic Hemophilia Treatment Center University ofKentucky Louisville, KY40492 200 E.ChestnutStreet Norton Kosair Children’s Medical Center Louisville, KY40202 529 SouthJackson Rm229 Hemophilia Treatment Center Brown CancerCenter KENTUCKY After hours:(319)356-1616 Phone: (319)356-4277 Pediatric afterhours:(207)885-7565 Adult afterhours:(207)885-7683 Phone: (207)885-7683 After hours:(504)988-5433 Phone: (504)988-5433 After hours:(859)323-5321 Phone: (800)333-7359 After hours:(502)629-7750 Phone: (502)629-7750 Pediatric afterhours:(502)595-4673 Adult afterhours:(502)562-4053 Phone: (502)595-4582 Hemophilia TreatmentCenters-USA MARYLAND Grand Rapids, MI49503 100 Michigan Street, N.E., MC#85 Disorders Program DeVos Childrens Coagulation DeVos Children’s Hospital Detroit, MI48201 3901 Beaubien Blvd. Hemostasis &Thrombosis Ctr Children’s Hospital ofMichigan Ann Arbor, MI48104 210 EastHuron, SuiteC2 Cascade HemophiliaConsortium MICHIGAN Worcester, MA01605 119 BelmontStreet UMass Memorial Hospital New England HemophiliaCenter Boston, MA02115 Fegan 717.2 Children’s Hospital Boston HemophiliaCenter Boston, MA02115 75 Francis Street BWH MidCampus-3 Brigham &Women’s Hospital Brigham andWomen’s Boston HemophiliaCenter MASSACHUSETTS Baltimore, MD21205 720 Rutland Avenue 1125 Ross Johns Hopkins University Medical Center Pediatric after hours:(616)391-1774 Phone: (616)391-2033 Pediatric afterhours:(313)745-5111 Phone: (313)745-5690 Phone: (734)996-3300 After hours:(800)955-8252 Phone: (800)955-8252 Pediatric afterhours:(617)355-6101 Adult afterhours:(617)732-5656 Phone: (617)355-8246 Phone: (617)732-5844 Pediatric afterhours:(410)232-9037 Adult afterhours:(410)955-6070 Phone: (304)614-0834 Kalamazoo, MI49007 601 John St,SuiteE.300 Ped Hematology/Oncology West Michigan Pediatric at Bronson Ann Arbor, MI48109-0235 1500 EastMedicalCenterDrive F2480 Mott and Coagulation Disorders University ofMichigan Hemophilia Traverse City, MI49684 1105 SixthStreet Munson Medical Center East Lansing, MI48823 2900 HannahBlvd Room 202 and ClottingDisorders Center for BleedingDisorders Mich. State University Detroit, MI48202-2689 K-13 Hematology /Oncology 2799 West Grand Boulevard Thrombosis Treatment Center Adult Hemophiliaand Henry Ford Hospital Kalamazoo, MI49007 200 NorthPark Street Michigan CancerCenter Hemophilia ClinicofWest Flint, MI48503-5993 One Hurley Plaza Hurley MedicalCenter Hemophilia Treatment Center Eastern Michigan Detroit, MI48201 4100 John R4HudsonWebber Disorders andThrombosis Comprehensive Centerfor Bleeding DMC Karmanos CancerInstitute Phone: (269)341-6350 After hours:(734)936-6267 Phone: (734)936-6393 Pediatric afterhours:(800)468-6766 Adult afterhours:(231)935-5000 Phone: (231)935-7227 Phone: (517)353-9385 Adult afterhours:(313)916-2600 Phone: (313)916-3790 Pediatric afterhours:(269)341-6350 Adult afterhours:(269)373-7488 Phone: (269)373-7479 Pediatric afterhours:(810)257-9000 Adult afterhours:(810)762-8200 Phone: (800)257-9432 Adult afterhours:(313)745-5111 Phone: (313)576-8707 Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays Phone: (973) 877-5340 After hours: (973) 877-5340 Phone: (732) 235-6542 After hrs: (732) 828-3000 on call Ask for Hem. Fellow Phone: (716) 896-2470 Phone: (716) 878-7446 after hours: (716) 896-2470 Pediatric Phone: (718) 470-7380 After hours: (718) 343-6776 Phone: (585) 922-5700 After hours: (585) 399-1717 Phone: (505) 272-6420 Adult after hours: (505) 272-2111 (505) 272-4461 after hours: Pediatric St. Michael’s Medical Center Medical St. Michael’s Comp. Nadeene Brunini Center Care Hemophilia Ave. 111 Central NJ 07102 Newark, Johnson Wood UMDNJ-Robert School Medical Regional Jersey New Hemophilia Program Place, Johnson Wood One Robert CN-19 #378C, Room NJ 08903 New Brunswick, NEW YORK Hemophilia Center of - Adult York New Western Center Erie County Medical 462 Grider Street Suite 20 1st floor, NY 14215 Buffalo, Hemophilia Center of - Ped. York New Western of Buffalo Hospital Children’s St. 219 Bryant NY 14222 Buffalo, Center Medical Long Island Jewish Center Hemophilia Treatment 358 Room Institute, Oncology 270-05 76th Avenue NY 11040 Park, New Hyde Mary M. Gooley Inc. Hemophilia Center, Suite 425 Avenue, 1415 Portland NY 14621 Rochester, NEW MEXICO Hemophilia Center R. Montoya Ted of N M Dept. of Pediatrics Univ MSC10 5590 of1 University New Mexico 87121 NM Albuquerque, Hemophilia Treatment Centers - USA Centers Treatment Hemophilia Phone: (603) 650-5522 After hours: (603) 650-5000 Phone: (856) 435-7502 after hours: (215) 590-1000 Pediatric Phone: (973) 926-6511 Adult after hours: (973) 926-7230 after hours: (973) 926-7161 Pediatric Phone: (702) 732-0971 Adult after hours: (702) 732-2011 (702) 732-0971 after hours: Pediatric Phone: (303) 724-0362 Adult after hrs: (303) 372-0000 After hours: (303) 861-6740 Ped. Phone: (402) 559-4227 NEW HAMPSHIRE Dartmouth-Hitchcock Hemophilia Center One Medical Center Drive Lebanon, NH 03756 NEW JERSEY of Philadelphia Hospital Children’s Center Speciality Hem/Onc Section of Jersey New Building 1014 Oak Road, 1012 Laurel NJ 08043 Voorhees, Center Medical Beth Israel Newark Hemophilia Comprehensive Center Treatment Osborne Terrace at Avenue 201 Lyons NJ 07112 Newark, NEVADA NEVADA Hemophilia Treatment Center of Las Vegas Center for Children’s Cancer & Blood Diseases Pkwy #202 Maryland 3059 S. NV 89109 Las Vegas, Mountain States Regional Hemophilia Regional Mountain States Center and Thrombosis 6507 MS F416 PO Box CO 80045-0507 Aurora, NEBRASKA Regional Nebraska Center Hemophilia Treatment Center Medical of Nebraska University Medical Center 987680 Nebraska Omaha, NE 68198-7680 MONTANA Phone: (314) 577-5332 after hours: (314) 577-5600 Pediatric Phone: (816) 234-3508 Adult after hours: (816) 404-4000 after hours: (816) 234-3000 Pediatric Phone: (573) 882-9355 After hours: (573) 882-4141 Office: (314) 577-6168 (314) 268-5643 Fax: Phone: (601) 984-2710 After hours: (601) 984-1000 Phone: (612) 626-6455 273-3000 Adult after hours: (612) (612) 813-5940 after hours: Pediatric Phone: (800) 344-7726 After hours: (507) 284-2511 The John Bouhasin Center for for Bouhasin Center John The with Bleeding Disorders Children Hospital Glennon Children’s Cardinal Dept of Pediatrics Saint Louis Univ. Blvd. 1465 South Grand MO 63104 St. Louis, Kansas City Regional Hemophilia Center City Regional Kansas Hospital Mercy Children’s The 2401 Gillham Road MO 64108 Kansas City, Hemophilia Treatment Center Hemophilia Treatment One Hospital Dr 7W12 MO 65212 Columbia, MISSOURI Center for Saint Louis University Disorders Bleeding and Thrombotic Adult Program Center, Hemophilia Treatment Pavilion Hospital, West St. Louis University Hem/Onc Floor, 3rd Avenue, 3655 Vista MO 63110 St. Louis, University of Mississippi of Mississippi University Center Medical Hematology/Oncology Ped Dr Wilson 350 W Woodrow 39213 MS Jackson, MISSISSIPPI University of Minnesota University Fairview Center, Medical Center Hemophilia and Thrombosis MMC 713 St., SE 420 Delaware MN 55455 Minneapolis, MINNESOTA MINNESOTA Comprehensive Mayo Center Hemophilia 106 St. SW Hilton 200 First 55905 MN Rochester, Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content New York,New NY10021 525 E.68thSt,Room P-695 Diagnostic andTreatment Center Regional Comprehensive Hemophilia Weill Medical College ofCornellUniversity Johnson City, NY13790 33-57 HarrisonStreet UHS Wilson Hospital UHSH BloodDisorder Center Albany, NY12208 ScotlandAve47 New A52 at Medical Albany College von Willebrand Trmt Ctr. The Regional Comp. Hemophilia& Syracuse, NY13210 750 E.AdamsSt,Rm5400 Cancer &BloodDisorder c/o Centerfor Children with Pediatric Program SUNY Upstate Medical University Syracuse, NY13210 750 E.AdamsStreet c/o Regional OncCenter Adult Program SUNY Upstate Medical University Box York, 1078New NY10029 19 East98thStreet, Suite9D Hemophilia Treatment Center Regional Comprehensive Mount SinaiSchool ofMedicine Greenville, NC27858-4354 PCMH 288West Brody School ofMedicine East Carolina University NORTH CAROLINA Pediatric afterhours:(212)746-3400 Adult afterhours:(212)746-2927 Phone: (212)746-3418 After hours:(607)763-6000 Phone: (607)763-6436 Pediatric afterhours:(518)262-5513 Adult afterhours:(518)786-7723 800-773-7080 Phone: (518)262-5827or Pediatric afterhours:(315)701-1790 Phone: (315)464-5294 Adult afterhours:(315)464-8200 Phone: (315)464-8200 After hours:(212)876-8701 Phone: (212)876-8701 Phone: (252)744-4676 Hemophilia TreatmentCenters-USA Dayton, OH45404-1815 One Children’s Plaza Treatment Center West Central OhioHemophilia Dayton Children’s Medical Center Columbus, OH43205 700 Children’s Drive Columbus HemophiliaTreatment Center Columbus Children’s Hospital Cincinnati, OH45229 3333 BurnetAvenue Hemophilia Treatment Center Medical Center Cincinnati Children’s Hospital Akron, OH44308-1062 One Perkins Square Hemophilia Treatment Center Medical CenterofAkron Children’s Hospital OHIO Fargo, ND58122 820 Fourth Street North Treatment Center Hemophilia &Thrombosis DBA Roger MarisCancerCenter MeritCare Hospital NORTH DAKOTA Winston-Salem, NC27157-1081 Medical CenterBoulevard Department ofPediatrics The Bowman Gray Campus Baptist Medical Center School ofMedicine Wake Forest University Chapel Hill,NC27599-7016 CB #7016 W1022 OldClinicBuilding School ofMedicine Univ. ofN. Carolina at Chapel Hill Pediatric afterhours:(937)641-3000 Adult afterhours:(937)641-3000 Phone: (937)641-5877 Pediatric afterhours:(614)722-3250 Phone: (614)722-3240 Pediatric afterhours:(513)636-4200 Phone: (513)636-4269 Phone: (330)543-8732 Pediatric afterhours:(701)234-6000 Phone: (701)234-7544 Pediatric afterhours:(336)716-4324 Adult afterhours:(336)713-5440 Phone: (336)716-4324 After hours:(919)966-4736 Phone: (919)966-4736 Cincinnati, OH45267-0562 Mail Location 562 231 AlbertSabin Way Hemophilia Treatment Center Medical Center University ofCincinnati Cleveland, OH44106 11100 Euclid Avenue Ped HemMailStop6054 Univ Hospitals Health System UHHS Cleveland Columbus, OH43210 320 W. 10thAvenue M414 StarlingLoving Hall Hemophilia Treatment Center Ohio State University Medical Center Toledo, OH43606 2150 W. Central Avenue Children’s Medical Center The Toledo Hospital Treatment Center Northwest OhioHemophilia Youngstown, OH44501 500 GypsyLane, 1stFloor Regional Referral Center Youngstown HemophiliaCenter Forum Health Portland, OR97239-2901 707 SWGainesRd OR Health &ScienceUniv. Oregon HemophiliaTreatment Center OREGON Oklahoma City, OK73104 940 N.E. 13thSt,Rm.3B3308 Oklahoma Centerfor BleedingDisorders OKLAHOMA Adult afterhours:(513)584-7661 Phone: (513)584-7639 Pediatric afterhours:(216)844-3345 Adult afterhours:(216)844-8220 Phone: (216)844-3345 Adult afterhours:(614)293-8000 Phone: (614)293-8183 Pediatric afterhours:(419)291-8520 Adult afterhours:(419)473-3200 Phone: (419)291-2210 Phone: (330)884-4176 After hours:(503)494-9000 Phone: (503)494-8716 Pediatric afterhours:(405)271-5437 Adult afterhours:(405)271-4222 Phone: (405)271-3661 Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays Phone: (801) 588-3477 Adult after hours: (801) 581-2121 after hours: (801) 588-2000 Pediatric Phone: (409) 772-1165 Phone: (713) 500-8360 After hours: (713) 704-4284 Or (713) 536-1152 Phone: (214) 648-1939 (214) 590-8000 After hours-Parkland: (214) 648-7070 After hours-UT Southwest: Phone: (214) 456-2379 (214) 456-7000 after hours: Pediatric Phone: (210) 704-2187 Phone: (832) 822-4242 After hours: (832) 824-2099 UTAH Mountain States Hemophilia Center Regional Center Medical Primary Children’s Medical Drive 100 N. UT 84113 City, Salt Lake Galveston Hemophilia Program Galveston of TX Med. Branch University Adult Hematology/Oncology Sealy Annex Rm 4.160 John TX 77555-0565 Galveston, Hemophilia and Gulf States Center 400 Suite 6655 Travis, Houston, TX 77030 Comprehensive North Texas Hemophilia Center Adult Program School Medical of TX Southwestern Univ 5323 Harry Hines Blvd. NC8.126 Room 75390-8852 TX Dallas, Comprehensive North Texas Program Hemophilia Center-Pediatric Center Medical Children’s 1935 Motor Street 75235 TX Dallas, Hemophilia & South Texas Center Trt. Thrombophilia Hospital Children’s Christus Santa Rosa 8th Floor Rosa, Santa 333 N. TX 78207 San Antonio, Hemophilia Children’s Texas Center and Thrombophilia 1420 Ste. 6701 Fannin Houston, TX 77030 Hemophilia Treatment Centers - USA Centers Treatment Hemophilia Phone: (682) 885-4007 after hours: (682) 885-4000 Pediatric Phone: (865) 544-9170 After hours: (865) 544-9170 Phone: (901) 448-6454 Adult after hours: (901) 448-6454 (901) 448-6454 after hours: Pediatric Phone: (901) 448-6454 After hours: (901) 448-6454 Phone: (615) 936-1765 Adult after hours: (615) 936-1803 after hours: (615) 936-1765 Pediatric Phone: (803) 434-3533 After hours: (803) 434-3533 Phone: (605) 333-7171 After hours: (605) 333-7188 TEXAS Program Bleeding Disorders Worth Fort Center Medical Cook Children’s Suite 220 Avenue, 901 Seventh TX 76104 Ft. Worth, TENNESSEE Comprehensive East Tennessee Hemophilia Center Center Medical of Tennessee University 4 North West 1924 Alcoa Highway TN 37920-6999 Knoxville, Hospital Research St. Jude Lauderdale 332 N. TN 38101-0318 Memphis, - Memphis of Tennessee University Hemophilia Clinic of Memphis of Tennessee University Suite 300 920 Madison Ave TN 38103-3446 Memphis, Center Medical University Vanderbilt Clinic Hemostasis-Thrombosis 397 PRB Ave 2220 Pierce TN 37232-6310 Nashville, Palmetto Health Richland Health Palmetto Center of South Carolina Hemophilia Rd. Medical Park 7 Richland Suite 203 SC 29203-6872 Columbia, SOUTH DAKOTA Center South Dakota Blood Disorders for Children’s Sioux Valley Clinic Specialty 18th Street 1305 West 57117-5039 SD Sioux Falls, SOUTH CAROLINA SOUTH CAROLINA Phone: (401) 444-8250 After hours: (401) 350-9707 Phone: (215) 662-9960 Adult after hours: (215) 662-4000 Phone: (610) 402-0640 Adult after hours: (610) 402-7880 Phone: (412) 209-7280 Adult after hours: (412) 209-7040 Phone: (717) 531-7468 Adult after hours: (717) 531-8521 Phone: (215) 590-4493 after hours: (215) 590-1000 Pediatric Phone: (215) 955-8435 955-8874 Adult after hours: (215) Rhode Island Hospital Hemophilia Center of Rhode Island Clinic George RI 02903 Providence, RHODE ISLAND Penn Comprehensive Hemophilia Program Comprehensive Penn of Pennsylvania Univ. Med. Ctr - Presbyterian 39th St. MAB Ste 106 51 N. 19104 Philadelphia, PA Lehigh Valley Hospital Lehigh Valley Center Hemophilia Treatment Suite 103 Blvd. 1240 South Cedar Crest 18105-1556 Allentown, PA Hemophilia Center Pennsylvania of Western of3636 Boulevard the Allies 15213 PA Pittsburgh, Hemophilia Center Pennsylvania of Central Med. Center Hershey S. Milton The H046 850, PO Box Drive, 500 University 17033 PA Hershey, Children’s Hospital of Philadelphia Hospital Children’s Hemophilia Program Blvd 34th St. & Civic Center Building Wood 4th Floor 19104 Philadelphia, PA PENNSYLVANIA PENNSYLVANIA Center Hemophilia Foundation Cardeza Hospital University Jefferson Thomas Suite 4225 Building, Gibbon 11th Street 111 S. 19107 Philadelphia, PA Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content Richmond, VA 23298-0461 4th Fl, Rm442,Southwing 1200 E.Broad Street West Hospital Virginia Commonwealth University Charlottesville, VA 22908 Jordan Hall,Room 2353 Hem/Onc Box 800747 Adult HemophiliaProgram University ofVirginia Hospital Charlottesville, VA 22908 Box 800386,Pediatric Hematology University ofVirginia Hospital Norfolk, VA 23507 601 Children’s Lane ofHamptonRoads Bleeding Disorders Center King’s Daughters Children’s Hospital ofthe VIRGINIA Burlington, VT05402 StPOBox108 Cherry 70 Vermont Regional HemophiliaCenter VERMONT Adult afterhours:(804)828-0951 Phone: (804)827-3306 Pediatric afterhours:(434)924-0211 Adult afterhours:(434)924-0000 Phone: (434)243-5809 Pediatric afterhours:(434)924-0211 Adult afterhours:(434)924-0000 Phone: (434)924-8499 Pediatric afterhours:(757)668-7243 Phone: (757)668-7243 Phone: (802)865-1326 Internet Resources Hemophilia TreatmentCenters-USA www.wfh.org World Federation of Hemophilia www.hemophilia.org 1-800-42-HANDI National HemophiliaFoundation www.hemophilia.ca Canadian HemophiliaSociety LaCrosse, WI54601 1836 SouthAvenue Gundersen Clinic Milwaukee, WI53201-2178 PO Box 2178 Southeastern Wisconsin The BloodCenterof Bleeding Disorders Comprehensive Centerfor WISCONSIN Morgantown, WV26506 PO Box 9162 Mary Babb Randolph CancerCenter Robert C.Byrd Health SciencesCenter West Virginia University Medical Center Charleston, WV25304 3200 MacConkle Avenue, SE c/o CancerCare Center- Charleston Area Medical Center WEST VIRGINIA Seattle, WA 98104-1256 921 Terry Avenue Hemophilia Program Puget SoundBloodCenter&Program WASHINGTON Pediatric afterhours:(800)362-7567 Adult afterhours:(800)362-9567 Phone: (608)782-7300 After hours:(414)257-2424 Phone: (414)257-2424 Adult afterhours:(877)427-2894 Adult afterhours:(877)541-9446 After hours:(206)292-6525 Phone: (206)292-6507 www.ProjectRedFlag.org Project Red Flag www.HemophiliaEmergencyCare.com Patients withHemophilia Emergency Care for www.cdc.gov CDC Aurora, CO80045-0507 PO Box 6507MSF416 and Thrombosis Center Mountain States Regional Hemophilia WYOMING San Juan,PR00936 Pediatrics Box 5067 of Department School ofMedicine University ofPuertoRico Hemophilia Treatment Center Puerto Rico Hagatna, GU96932 PO Box 2816 Health &Social Services Department ofPublic Hemophilia Care Program Guam Comprehensive U.S. TERRITORIES Madison, WI53705 2704 MarshallCourt for BleedingDisorders UWHC Comprehensive Program Green Bay, WI54311 Ave.1794 E.Allouez Hemophilia Outreach Centre Ped. Afterhours:(303)861-6740 Adult afterhrs:(303)372-0000 Phone: (303)724-0362 Phone: (787)777-3535Ext.7013/4 Phone: (671)735-7168 After hours:(608)262-0486 Phone: (608)890-9495 After hours:(920)965-0606 Phone: (920)965-0606 Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays 1 Table of Contents Table 2 4 6 10 12 14 16 18 19 20 22 23 ) ® and Octostim ® , Stimate Emergency Care for Patients for Care Emergency ® with von Willebrand Disease Willebrand with von (DDAVP Disease Basics Willebrand / von Introduction Head Injury Bleeding Mucous Membrane Bleeding GI/Urinary Tract Bleeding Gynecological Bleeding / Soft Tissue /Muscle Joint Acetate Desmopressin & Administration Medications Factor Other Medications / X-rays / Labs Procedures Invasive / Emergencies Trauma / References Bibliography Copyright © 2006, Updated January 2011 Susan C. Zappa, RN, CPN, CPON, Cook Children's Medical Center; Cook Children's CPON, CPN, RN, Zappa, January 2011 Susan C. © 2006, Updated Copyright Sherry L. Health Sciences Centre; Jacobson, RN , Winnipeg, Hospital; Rose Ottawa The BScN, RN, Lucie Lacasse, of School University Tulane RN, Wulff, Hospital; Karen Kingston General Medicine. RN, Purcell, or further information For only. purposes for educational to photocopy granted Permission All rights reserved. (682) 885-2587. [email protected] Medical Center, Cook Children's at contact Susan Zappa inquiries, Phone: (671) 735-7168 Phone: (787) 777-3535 Ext. 7013/4 Phone: (303) 724-0362 Adult after hrs: (303) 372-0000 (303) 861-6740 After hours: Ped. Phone: (920) 965-0606 Phone: (920) (920) 965-0606 After hours: Phone: (608) 890-9495 After hours: (608) 262-0486 U.S. TERRITORIES TERRITORIES U.S. Guam Comprehensive Program Hemophilia Care Department of Public Services & Social Health 2816 PO Box GU 96932 Hagatna, Puerto Rico Center Hemophilia Treatment Rico of Puerto University of Medicine School Department of 5067 Box Pediatrics San Juan, PR 00936 WYOMING Hemophilia Regional Mountain States Center and Thrombosis MS F416 6507 PO Box CO 80045-0507 Aurora, Hemophilia Outreach Centre Outreach Hemophilia 1794 E. Allouez Ave. WI 54311 Bay, Green Program UWHC Comprehensive Bleeding Disorders for 2704 Marshall Court Madison, WI 53705 Trauma Labs Joint / Muscle Mucous CentersIntro Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. ContentBasics 2 The prevalence isashigh1to2percent inthegeneral population. Prevalence bothinplatelet-plugdefect formation andinfibrinformation. FVIII,which factor onvon is dependent Willebrand asits naturally factor occurringplasmacarrierandstabilizer,of leadstoa von factor, Willebrand Even in deficiencyordefect thoughtheprimary von deficiency thesecondary Willebrand Disease isthat of thereby protecting itfrom inactivation andclearance. vascular injury, particularly through larger multimers. Italsoforms anon-covalent complexwithcoagulation VIIIinplasma, factor von Willebrand isessentialforplatelet-plug factor formation asanadhesionprotein that diverts circulating tothesitesof Effects ofvonWillebrand Disease Willebrand factor, alarge multimeric . Itisnon-sexlinked. Therefore, inbothmenandwomen. itcanoccurequally von von Willebrand bleeding Diseaseisanautosomally-inherited disorder causedby thequantitative of deficiencyordysfunction Definition • thismanualare to: thisdisorder anditstreatment. The goals of understanding of This manualcontributes tovon Willebrand Disease(VWD)care by enhancingtheemergency personnel’s department Purpose diagnosis andtreatment. guidelines, recommendations andsuggestions only. The attending physician hasthefinal responsibility for appropriate This manualisaguideformedicalpersonnelwho withVWDtreatment. maybelessfamiliar The contentconsistsof To The AttendingMedicalStaff ing complications. It issuggested that thepatient’s treatment centerorhematologist beconsultedfor final management of bleed- themanualas areference. provided ontheinsidecover of VWD.tions andtreatment. The Treatment treatment varies to the typeandseverity of andmanagement information is points forquick review. bleeding presentations, theirpossible The textontheright page complica- givesdetailof further interest. VWD. The illustration onthe leftpage Turn providesby toanarea information systemsandcomplications of of This VWDemergencies. manualprovides astandardized format The forevaluation contentissegmented andtreatment of Use •promote aconsultative dialogue withtheemergency (ED),treatment department center, andpatient/family •provide areference fortheemergency centerstaff Introduction &vonWillebrandDiseaseBasics creto fthehemostatic abnormality correction of promote understanding of Early triage andtreatment reduce morbidity.

the complexities of

von Willebrand Disease with an emphasis on rapid treatment for

Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays 3 - factor Willebrand von factor Willebrand von normal

. Specific doses, additional drugs and medical additional . Specific doses, ® and wilate ® It is in these rare cases of type 3 (1 in 1 million people) that type 3 (1 in 1 million people) that cases of It is in these rare

Introduction & von Willebrand Disease Basics Disease Willebrand & von Introduction , Alphanate ® of production a decreased from results cases, of abnormalities qualitative by cases is characterized or compound in homozygous trait as an autosomal recessive cases and is transmitted • intracranial • spinal cord • oropharynx bleeding • vaginal • gastrointestinal • ocular • intra-abdominal heterozygous persons. This severe form of the disease is characterized by a very low or undetectable von Willebrand factor in of form Willebrand severe von This or undetectable persons. low a very by the disease is characterized heterozygous factor VIII activity. usually detectable plasma with a low, and factor VIII; typically transmitted as an autosomal dominant trait in the heterozygous state. in the heterozygous and factor as an autosomal dominant trait typically VIII; transmitted autosomal dominant. Inheritance is generally and 2N. 2M and is further 2A, 2B, into subtypes divided hemophilia. similar to those cases of severe and severe, frequent more symptoms are Patients living with VWD or their parents are often knowledgeable about the management of the management about often knowledgeable and their input are living with VWD or their parents their disorder Patients the prior to arriving at has been administered anyshould be sought and heeded. Interview medication the family whether about of and severity dependent on the time lag may be required, ED; if treatment Additional dose. and what the when determine so, as needed. and contact them for assistance and follow-up center, or treatment hematologist the treating Determine bleed. Family interventions depend upon the type ofinterventions of VWD and the site and severity of to the inside cover Please refer bleeding. the manual procedures diagnostic emergency has been given, Once treatment treatment. on the recommended detailed information for more can begin. The major sites of serious bleeding which threaten life, limb, or function major sites of are: The limb, life, threaten which serious bleeding Serious bleeding sites Serious bleeding mainstay ofThe of is the replacement treatment at the time of proce- protein the deficient/defective invasive bleeding or before or an infusion of or intravenous) intranasal, (subcutaneous, desmopressin may require This von performed. commercial are dures as Humate-P such factor/FVIII concentrate Willebrand gical bleeding can also become life or limb-threatening in these patients. in these patients. can also become life or limb-threatening gical bleeding Treatment The hallmark of von Willebrand Disease is mucosal bleeding. Mucous membrane bleeds such as bleeding from the nose, mouth, the nose, from as bleeding such of bleeds hallmark Mucous membrane The bleeding. Disease is mucosal Willebrand von the most common. If are bleeding and vaginal genitourinary bleeds tract, membrane these mucous left untreated, gastrointestinal and/or post-sur trauma untreated from resulting Serious bleeding emergencies. can become acute and sometimes life-threatening Bleeding episodes Acquired von Willebrand Disease: This is an acquired syndrome that resembles von Willebrand Disease in its clinical manifestation manifestation Disease in its clinical Willebrand von resembles that syndrome is an acquired Disease: This Willebrand von Acquired and autoim- as lymphoproliferative conditions such with clinical instances in association It occurs in rare patterns. and laboratory heart disease. tumor and valvular Wilm’s cancer, essential , hypothyroidism, diseases, mune • of accounts for 60 to 80 percent 1: which Type • of accounts for 10 to 30 percent 2: which Type • of 1 to 5 percent 3 : Accounts for Type von Willebrand Disease is classified into three main phenotypes and each have subtypes based on the quantity and quality of based subtypes have main phenotypes and each three into Disease is classified von Willebrand the von factor (VWF): Willebrand The type of VWD determines the treatment - see inside of the front cover for treatment options. von Willebrand Disease Willebrand von options. treatment for cover of the front - see inside the treatment of VWD determines type The a hematologist. contact bleeding continues as if type 1. If proceed 3’. If the type is unknown, by ‘type 1,2 or is classified Types of von Willebrand Disease of von Willebrand Types Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 4 Head Injury * Recommended treatment table inside front cover potential forallheadinjuries. (ICH)isa Discharge Instructions such asCTscan. perform diagnosticstudies treatment first Administer recommended 24 -48hourperiod). (instead oftheusualinstructions for be givenforatwoweek period Head injuryinstructionsshould patient’s hematologist. ICH tothetreatmentcenteror Report anysignsorsymptomsofan treatment recommendations patient’s hematologistforfollow-up Call thetreatmentcenteror may bedischarged. ICH isdiagnosed,thepatient contacted immediately.Ifno and thehematologist patient shouldbeadmitted If anICHisdiagnosed,the * , andthen * . Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays Head Injury Head has been given. Notify the has been given. * Recommended treatment table inside front cover 5 cover inside front table treatment Recommended * before any diagnostic tests. A hematologist should be contacted. A hematologist tests. any diagnostic before * Diagnostic imaging Diagnostic treatment CT scan to rule out ICH after the appropriate Obtain an emergency Instructions If instruct the family for signs and symptoms of home, to monitor the patient discharged is the patient center to Consult the treatment to the hematologist. any and report abnormalities deterioration neurologic if department. treatment for follow-up the emergency arrange home from is discharged the patient Possible admission Possible if should be admitted to the hospital for observation patient The to blow he/she has suffered a severe severity, with increasing headache Symptoms can include symptoms. any neurologic the head or exhibits stiff deficits, focal neurologic in level vision problems, changes or neck, seizures, vomiting, of irritability, . head bleeds risk of repeated increased at with a past history of ICH are Patients consciousness. patient’s hematologist or treatment center as soon as possible. center as soon as possible. or treatment hematologist patient’s Treatment appropriate the with promptly be treated should with or without , trauma, All significant head treatment Intracranial hemorrhage (ICH) is a potential risk for individuals with von Willebrand Disease, and is most and Disease, Willebrand von individuals with a potential risk for (ICH) is hemorrhage Intracranial risk of The severe with the more is increased injury. with commonly associated hemorrhage intracranial can occur. impairment neurologic or severe death types of and treatment, recognition early Without VWD. be evident. symptoms may not always neurologic Early Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 6 Mucous MembraneBleeding * A DentalorE.N.T.consultmaybeneeded. Recommended treatment table inside front cover Discharge Instructions hematologist thenextday. with theirtreatmentcenteror Patients shouldfollow-up needed. Diet Modificationstable onpg.9as of amodifieddiet.Consultthe fibrinolytics, andtheimportance control epistaxis,theuseofanti- Instruct thepatientonhowto treatment or tonguelaceration)mayrequire Mouth bleeds(gum,tooth,frenulum (if available)orfibringlue(Tisseal local measuressuchastopicalthrombin fibrinolytics. Ifthebleedingisminor, prolonged mucosalbleeding. Assess foranemiaiftherehasbeen 9 . used. Refertotheanti-fibrinolyticsonpg. conjunction withanti-fibrinolyticscanbe pg. 8. “Controlling Epistaxis”tableon to othermeasures.Refer Nose bleedsmayrespond * andtheuseofanti- ® ) in Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays . * is required prior to is required * Mucous Membrane Bleeding Membrane Mucous to increase the von Willebrand Willebrand the von to increase * Recommended treatment table inside front cover 7 cover inside front table treatment Recommended * , further observation, X-rays and admission may be required depending upon and admission may be required X-rays , further observation, * After the recommended treatment After the recommended Retropharyngeal the specific circumstance. The patient may not know the reason for the symptom or bleeding. It may have been caused by trauma, infec- trauma, been caused by It may have symptom or bleeding. for the may not know the reason patient The treatment If prompt is suspected, may be spontaneous. tion, or the bleed blockage airway or speaking swallowing difficulty • have distress experiencing respiratory • are area mouth or throat in the swelling severe • have or nose a known injury sustained to the mouth, tongue, • have bleeding and/or prolonged experiencing profuse • are Mucous membrane bleeding may require medical care in the emergency department. Treatment may be required may be required department. Treatment emergency in the medical care may require bleeding membrane Mucous who: for patients factor will be necessary prior to extraction. A frenulum or tongue laceration will require treatment will require or tongue laceration factor A frenulum will be necessary prior to extraction. Bleeding may occur with extracted, erupting or exfoliating teeth. It is more common with extracted and exfo- common with extracted teeth. It is more erupting or exfoliating Bleeding may occur with extracted, the tooth. Treatment teeth. A dental consult may be needed to extract liating Bleeding in the mouth can be hard to control. A frenulum or tongue laceration may respond to topical to topical thrombin respond may laceration or tongue A frenulum to control. Bleeding in the mouth can be hard - If treat A single need further treatment. or other similar agents. will probably the patient continues, the bleed Re- in re-bleeding. often results enzymes saliva lysis from clot but stop the bleeding, ment may temporarily A is most commonly to maintain hemostasis. seen on days 3-5. An anti-fibrinolytic may be indicated bleeding pg. 9). (see Diet Modifications at the same time as treatment modified diet should be started Oral Cavity Oral Uncontrolled epistaxis may require treatment in conjunction with anti-fibrolytics. Be sure the patient knows the patient Be sure in conjunction with anti-fibrolytics. treatment epistaxis may require Uncontrolled 8) (see pg. and stop the bleeding how to control Epistaxis Remember prompt treatment will greatly reduce the bleeding, often preventing serious complica- often preventing the bleeding, reduce will greatly treatment prompt Remember If place. takes - the accumu bleeding space, the more is in a closed waits, the bleed the patient longer tions. The of obstruction and pain. lation airway tissue damage, will cause surrounding blood any invasive procedures. procedures. any invasive Trauma Labs Joint / Muscle Mucous Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Membr. Content Mucous MembraneBleeding 8 vessel. 10. AnEarNoseThroat(ENT)consultmaybe requiredforpossiblecauterizationofa 7. 6. 5. 4. 3. 2. 8. 9. also helpful. crusts whichmightcrackandrestartbleeding. Adequatehumidificationinthehomeis the narestokeepmembranessoftandmoist, andpreventtheformationofhard During activebleeding,NoseBleedQR needed. Recommended treatment*and/oranti-fibrinolyticagents(seenextpage)maybe If thebleedingcontinues,reapplypressureforanotherfiveminutes. soft clots. Release thepressuretoseeifbleedinghasstopped,gentlyblowoutandremoveany minutes. Apply firmconstantpressuretotheentiresideofnosethatisbleedingfor15 the throat. Tilt theheadforwardsoanybloodwillcomeoutnaresandnotdownbackof two dropsofoxymetazoline(eg.NeoSynephrine During activebleeding,orwhenthebleedinghasstopped,youmaysprayapply may stop. manufacturer's directions.Thepowderwillsolidifytheblood,formacrustandbleeding preparation canbeutilized.Thepowderneedstomixedwithblood,asperthe Instruct thepatienttousemucosalmembrane moisturizer(eg.Vaseline to thesidethatwasbleeding.Thesecanbeused athomePRNforepistaxis. 1. Instruct thepatient: Controlling Epistaxis clots thatwillinterferewithhemostasis. To gentlyblowhis/hernosetoremovemucusandunstable ® powderanover-thecounter ® , Dristan ® orAfrin ® ) nasalspray/drops ® , Secaris ® ) in Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays and ® Mucous Membrane Bleeding Membrane Mucous Recommended treatment table inside front cover 9 cover inside front table treatment Recommended * Diet Modifications Directions for the patient: ) has been utilized for treatment, ® . In some cases they may be prescribed . In some cases * , or Stimate ® are both anti-fibrinolytic agents. Either may be prescribed for may be prescribed agents. Either anti-fibrinolytic are both ® , Octostim ® may also be used to help control mucous membrane bleeding. may also be used to help control ® Contraindicated if hematuria present Contraindicated if hematuria Contraindicated if hematuria present Contraindicated if hematuria * independently. days 3-4 g every 6 hours for 3 -10 Adult: oral dose 500 mg or 1000 mg per tab Supplied: Tablet: mg per ml Syrup: 250 in 20 ml vial Injectable: 250 mg /ml available Adult dose: oral dose 1000 mg-1500 mg tid for 3 - 10 days Adult dose: oral dose 1000 mg-1500 acid per tab Supplied: Tablet: 500 mg tranexamic Cyklokapron * Anti-Fibrinolytics Amicar nasal or oral bleeding. be indicated in may Anti-fibrinolytics mucous membrane bleeding to promote clot stabilization in conjunction with stabilization in conjunction with bleeding to promote clot mucous membrane treatment the recommended and Gelfoam Amicar - epsilon aminocaproic acid Recommended dosage: aminocaproic acid Amicar - epsilon Cyklokapron - Recommended dosage: Cyklokapron - tranexamic acid Injectable: 100 mg/ml available in 5 and 10 ml ampules Injectable: 100 mg/ml ® Child: oral dose 50-100 mg/kg (not to exceed 4 g) every 6 hours for 3 - 10 days (not to exceed 4 g) every 6 hours oral dose 50-100 mg/kg Child: Child: oral dose 25 mg/kg every 6- 8 hours for 3 - 10 days Child: oral dose 25

healed. Suggested foods: flavored gelatin, non-carbonated drinks, sherbet, healed. Suggested foods: flavored gelatin, non-carbonated or pureed foods, lukewarm soups (no cream soups), baby foods, blenderized pasta. pressure Avoid using a straw, chewing gum, and do not smoke. Negative the bleeding site. from the sucking action can dislodge the clot and aggravate tacos, etc. Foods to avoid include hard foods like chips, nuts, popcorn, If Desmopressin (DDAVP the patient has fluid restrictions for 24 hours.

Follow-up care per the treatment center or patient’s hematologist. Topical agents such as topical center or patient’s hematologist. Topical agents such as topical Follow-up care per the treatment Thrombin These medications must be given as ordered to keep blood levels constant. They are not readily as ordered to keep blood levels constant. They are not readily These medications must be given for (they must be ordered). If possible, dispense the amount available through local pharmacies to allow time for the local pharmacy to order. Other options 2-3 days from the hospital pharmacy disorders treatment center or (U.S.) home care companies. are the family’s home supply, bleeding

1. Diet should be restricted to soft, cool, or lukewarm foods until the area is fully 1. Diet should be restricted to soft, cool, or lukewarm foods 2. 3. 4.

Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 10 Gastrointestinal /UrinaryTractBleeding Discharge Instructions * gastrointestinal problems. intracranial hemorrhageaswell Nausea andvomitingmayindicate Recommended treatment table inside front cover - noheavylifting - rest - increasefluids hematologist product*asper -Management:Treatment -painonextension -flexedhip Iliopsoas bleeding hematemesis hematuria,melena, asfever,pain,orincreased - reportanysymptoms such -avoidanti-fibrinolytics thepatient’shematologist -consultthetreatmentcenteror -forcefluids -bedrestfor24hours Hematuria patient’shematologist treatmentcenter orthe -follow-upwiththe Treat immediately Abdominal pain -vomitingblood -melena -flankpain hematologist for: * asper Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays should be initiated. Once this is done, this is done, Once should be initiated. * Recommended treatment table inside front cover 11 cover inside front table treatment Recommended * Gastrointestinal / Urinary Tract Bleeding Tract / Urinary Gastrointestinal Scrotal bleeding may occur after trauma, especially in toddlers. Treatment will be required and follow-up with the and follow-up will be required Treatment especially in toddlers. may occur after trauma, bleeding Scrotal be arranged. center should or treatment hematologist . Contact the hematologist. with hematuria contraindicated are Anti-fibrinolytics Hematuria is often frightening to the patient but not a serious event. Instruct the patient to remain at bed rest and bed rest at to remain Instruct the patient not a serious event. but is often frightening to the patient Hematuria - treat require may hematuria Protracted the next 24 hours. over hour oz or 500 ml every fluids to 16 to increase ment. Genitourinary bleeding For nausea and vomiting without an obvious cause, consider that these may be symptoms of these may be symptoms consider that cause, without an obvious nausea and vomiting For bleeding. intracranial and consider CT scan signs and symptoms, and other neurologic changes, status mental head injury, about Inquire of the head. Symptoms of abdominal muscle bleeding (rectus, pectorals, latissimus, obliques) are a palpable mass, rigidity, and rigidity, mass, a palpable are obliques) Symptoms of latissimus, pectorals, (rectus, bleeding muscle abdominal unnoticed for days with a steadily and go may be present cavity in the abdominal bleeding pain. Concurrent falls of the hemoglobin Rupture when should be considered spleen, or pancreas hemoglobin. dropping the liver, trauma. following dramatically Symptoms - can precipi the groin A history or stretching of crossbar falling on a bicycle lifting, weight objects, lifting heavy types of These bleeding. 14 and 15), or retroperitoneal can occur iliopsoas (see pg. bleeds wall, abdominal tate VWD. seen in type 1 and type 2 rarely and are commonly type 3 VWD, in individuals with more Abdominal trauma and benign events such as forceful coughing or vomiting can precipitate an abdominal bleed. bleed. an abdominal can precipitate as forceful or vomiting coughing such and benign events Abdominal trauma - gastroen bleeds with Infants can have appear. signs and symptoms outward can be significant before loss Blood Diverticulum. intussusception or Meckel's teritis, then diagnostic x-rays, scans and endoscopy procedures can be carried out. procedures scans and endoscopy x-rays, then diagnostic Patients who present to the emergency department with abdominal or flank pain, melena or hematemesis should or hematemesis or flank pain, melena department with abdominal the emergency to present who Patients treatment and the recommended examination for immediate be triaged Bleeding may also occur with hemorrhoids or the passage of occur with hemorrhoids or the passage Bleeding may also or the center treatment Notify the stones. kidney hematologist. patient’s Acute abdominal pain in a patient with von Willebrand Disease may have many origins, such as gastrointestinal as gastrointestinal such many origins, may have Disease Willebrand with von a patient pain in Acute abdominal bleeding. retroperitoneal induced), iliopsoas or or trauma (both spontaneous (GI) tract Initial presentation Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content patient’shematologist treatmentcenter orthe -follow-upwiththe 12 (withinone week) -checkferritinlevel -checkhemoglobin Assess forsignsofanemia Gynecological Bleeding (DDAVP therapy topreventmorebleeding. therapy orIVPremarinasadjunctive Consider prescribingbirthcontrol fibrinolytic (pg.9). preparation available),startananti- In additiontoHumate-P For activemenorrhagia: and wilate Discharge Instructions ® orStimate ® orDesmopressin ® /Octostim ® , Alphanate ® as history recordbleeding,menstrual - Instructpatienttoaccurately -needforirontherapyeither -misseddaysatschool/workdue -length,regularityofmenses pads -numberofoverfloworflooded -presenceofclots,sizeclots -amountofbloodoneachpad - frequency of changing protection -padand/ortamponcountper Obtain accuratemenstrualhistory preparations) (orusesupplemental iron fluids,eatingironrich foods - Recommendrest,drinking currently orinthepast to menses (use apictorialchartifavailable) (include nights) hour orper24hrtimeperiod Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays , ®* should * Gynecological Bleeding Gynecological Recommended treatment table inside front cover 13 cover inside front table treatment Recommended * ) Consultation with an OB-GYN special- with an OB-GYN ) Consultation ® and wilate ® , Alphanate ®* ) Adjunctive treatment with intravenous or oral anti-fibrinolytics may be useful or oral intravenous with treatment ) Adjunctive ® and wilate ® be initiated immediately to elevate the von Willebrand factor levels. Life-threatening post-partum Life-threatening factor levels. Willebrand the von to elevate immediately be initiated (ex. Humate-P factor/FVIII concentrate Willebrand with a von treatment will require hemorrhage (see pg. 9). (see pg. – During pregnancy, the majority of the majority Disease, Bleeding – During pregnancy, Willebrand with von Postpartum women levels. estrogen due to increased factor and factor VIII levels Willebrand von normal type 1, will have on the use of some data are there few but very published are “There pregnancy, during desmopressin growth intrauterine labour, with premature causes uterine contraction desmopressin concerns that the use of to be cautious about it is advisable these reasons, For des- and hyponatremia. retardation can be used if desmopressin is clamped, Once the cord It is necessary. during pregnancy. mopressin is not contraindicated Desmopressin a caesarean. before to use desmopressin reasonable also probably during lactation.” with type et al. (2000). Gynecological and obstetrical morbidity in women Burkart, P., D., Phatak, P. P.A., Kouides, of disease : results Willebrand 1 von Hemophilia, 6(6), 643-648. a patient survey. - increas thereby delivery, 24 to 48 hours following will decrease factor levels Willebrand von The ing the risk of of In the event post-partum bleeding. treatment hemorrhage, a post-partum - Some women bleed excessively through their menstrual cycle. Others bleed between cycles cycles between Others bleed their menstrual cycle. through excessively bleed Menses - Some women iron menorrhagia, to the ED with may present women the month. These or continuously through Obtain an accurate ovulation. with bleeding due to increased or mittelschmerz anemia, deficiency, recommendations. for treatment menstrual history hematologist the patient’s and contact in just a fewAssess for signs of days, 2-3 g/ml Hgb, to can drop hemoglobin anemia, as the patient's menses. prolonged from ist and hematologist at a bleeding disorder treatment center is essential for ongoing follow-up. Oral Oral follow-up. is essential for ongoing center treatment disorder a bleeding at ist and hematologist may or subcutaneously) intranasally (IV, and desmopressin anti-fibrinolytic treatment contraceptives, basis. on an ongoing be recommended in just a fewAssess for signs of days, g/ml Hgb, 2-3 can drop hemoglobin anemia, as the patient's menses. prolonged from – a teenage girl with von Willebrand Disease can present to an emergency department at departmentat to an emergency Disease can present Willebrand von with girl – a teenage Menarche - treat Appropriate hemorrhage. occasionally life-threatening or soon after with a severe, menarche Willebrand a von with treatment requires bleeding Major vaginal immediately. ment should commence (ex. Humate-P factor concentrate Alphanate Prolonged and is one of is bleeding menstrual and heavy Prolonged common symptoms for females the most with bleeding disorders. Menstrual Bleeding Menstrual Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 14 R.I.C.E. Management: Notreatment, -tenderness,butnoseverepain -nofunctionalimpairment Soft tissuebleedsandbruising Soft Tissue/MuscleJointBleeding Discharge Instructions * Recommended treatment table inside front cover Management: Treatmentproduct -potentialairwaycompromise Neck swelling:EMERGENCY as perhematologist product Management: Treatment legontheaffectedside -pain/inabilitytoextendthe -flexedhip Iliopsoas bleeds jointsand crutchinstructions - - (Ace ** ** Crutches -forweightbearing RICE -Rest,Ice,Compression * asperhematologist ® wraps),Elevation *

(i.e.Aircast - Slingorsplintingifsupport isneeded orthepatient’s hematologist - follow-upwiththetreatment center Management: Treatmentproduct -swelling -heatpain Advanced jointbleed -limitedrangeofmotion -tinglingpain Early onsetjointbleed immobilization forcomfort. as perhematologist.Iceand Management: Treatmentproduct ofcompartmentsyndrome -observeforsignsandsymptoms -impairedmobility -with/withoutswelling -pain Thigh/calf/buttock bleed as perhematologist ® forankles) as perhematologistR.I.C.E. Management: Treatmentproduct* compartmentsyndrome -observeforsymptomsof -increasedswellingandbruising Deltoid /forearmbleed ** *

*

Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays - Anti-fibrinolytics may also * should be initiated and the patient should and the patient should be initiated * Recommended treatment table inside front cover 15 cover inside front table treatment Recommended * Soft Tissue / Muscle / Joint Bleeding / Joint / Muscle Tissue Soft in order to minimize pain and joint destruction. Extreme pain, to minimize pain and joint destruction. Extreme in order * . * - treat with conservative often will resolve more but treatment may require bleeds : Occasionally muscle and follow-up care Treatment If and ice. as rest ment such treatment is suspected, appropriate compartment syndrome manent disabilities such as foot drop and wrist contracture. It is important that the patient’s hematologist be consulted before any be consulted before hematologist the patient’s It is important that and wrist contracture. drop as foot such manent disabilities procedures. invasive Consequences of may loss blood in serious consequences if can result Extensive bleeds Muscle bleeds: muscle promptly. not treated and, if vessels blood and on nerves can place pressure in per may result bleeding untreated, Muscle groups. muscle occur in large be helpful. Initiate treatment before any diagnostic procedures such as x-ray. Before dislocated joints are reduced, infuse- reduced, with the recommend joints are dislocated Before as x-ray. such procedures any diagnostic before treatment Initiate ed treatment swelling, heat, and immobility are signs and symptoms of signs and symptoms are has filled the joint and immobility heat, occurs only after blood which swelling, joint bleed an advanced space. The aspiration of joint bleeds in VWD is contraindicated unless recommended by the treatment center. of aspiration The center. the treatment by unless recommended in VWD is contraindicated joint bleeds Joint Aspiration: Caution! Aspiration: Joint Muscles that exhibit warmth, pain, and swelling should be managed with the recommended treatment. recommended with the should be managed pain, and swelling warmth, exhibit that Muscles Persons with the most severe type of spontaneously mini- or with most severe with the bleeding Persons can experience muscle type 3, Disease, Willebrand von thigh, and calf forearm, the upper arm, sites include bleeding Common to bleeding. be subject may group Any muscle mal trauma. muscles. Muscle bleeding is usually only associated with trauma in persons with mild von Willebrand Disease. Disease. Willebrand with mild von in persons is usually with trauma only associated bleeding Muscle Muscle bleeds Muscle - and eleva rest, ice to well respond and bruises Superficial hematomas treatment. aggressive usually do not require bleeds Soft tissue tion. Ifmay be required. or function, treatment movement impairing size, in to increase bruising continue and hematoma the Soft tissue and superficial bleeds superficial Soft tissue and : Some patients may present for treatment with no other outward signs of outward with no other for treatment of may present : Some patients range than decreased Treatment bleeding motion and a complaint of of is indicative This should patient The pain or tingling. and is the optimal time to treat. joint bleed onset an early treatment with the recommended as possible be infused as quickly : Outward signs ofSigns and symptoms: Outward of restriction include and erythema joint bleeding on and around heat, swelling, movement, a symptoms include symptoms of may report Later patient the joint. The signs. with no physical sensation or tingling a bubbling feeling of worsens. pain as the bleed to severe fullness within the joint and moderate The joints most commonly affected are the elbows, knees, and ankles. Less common sites include the shoulders and hips. As the shoulders and hips. sites include Less common and ankles. joints most commonly knees, affectedThe the elbows, are of cycle A vicious vessels. blood bleeding friable more even and develops tissue thickens the synovial occurs, bleeding repeated into joints leads to bleeding repeated joint.” Eventually, in and the affected may set a “target to as joint is referred and rebleeding of with destruction arthropathy of erosion and the eventual and function. joint mobility cartilage is decreased end result The bone. Joint bleeding is uncommon in individuals with type 1 and 2 von Willebrand Disease and is usually associated with trauma. Indi- with trauma. Disease and is usually associated Willebrand in individuals with type 1 and 2 von is uncommon bleeding Joint can occur into any and bleeding with or without trauma, Disease can experience joint bleeding Willebrand viduals with type 3 von joint space. Joint Bleeding Joint be admitted with an emergency consult to hematology. hematology. consult to be admitted with an emergency

* ** Deltoid / forearm bleed - increased swelling and bruising - observe for symptoms of compartment syndrome Management: Treatment product* as per hematologist R.I.C.E. as per hematologist Thigh/calf/buttock bleed - pain - with/without swelling - impaired mobility - observe for signs and symptoms of compartment syndrome Management: Treatment product Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content insufficiency. Hypersensitivity, infantsunder3months, patientssuffering fromdehydration, historyofseizure,coronary artery Contraindications Treatment ofvon Willebrand Disease Type 1andcertainformsof Type 2 Indications lines. Unreliable absorptioniftheintranasalrouteiscompromised. Hematologistshouldbeconsultedfortreatmentguide Recommended doseforpatientsunder50kg:150mcg (1sprayinonenostril,totalofspray) Recommended doseforpatientsover50kg:300mcg (1spraypernostril,totaloftwosprays) OCTOSTIM Nasal Spray dose ofDDAVP 150 mcg/0.1mlpersinglespray(OCTOSTIM Ampules: 4mcg/mlDDAVP DESMOPRESSIN -brand names:DDAVP Supplied SC ROUTE: The subcutaneous routeisadvantageoustominimizedrugsideeffects. recommended toadministerthemedicationwithindividualinasupineposition. IV ROUTE:Dilutewithnormalsaline(50-100ml).Infuseover30-60minutes.Nolessthan30Itis SC/IV: 0.3mcg/kg/dose.Recommendation:amaximumdoseof20mcg. Dose shortterm flushing, facial increased heart Expected sideeffects: rate, red conjunctiva, andheadache. factor VIII,suchfactor asHumate –P concentrates factor containingbothvon Willebrand protein and of pressin, hemostasiscanbemaintainedwithinfusions thepatient doesnotrespond todemo- after administration andhemostasisismaintainedforapproximately 24hours. If isreached thepatient 30to90minutes doesrespond effect todesmopressin, thefull and recorded by ahematologist. If can vary greatly witheach individual.Therefore, priortouse, adesmopressin trialshouldbedonewithresults reviewed Desmopressin isthepreferred treatment fortype1VWDandcertainpatients withtype2.The response todesmopressin tor from theendothelialcellstorage sites. Itcanincrease theVWFlevel by asmuch asthree tofive fold. VIIIandvon factor Willebrand- fac antidiuretic hormone which causestherelease of Desmopressin isasyntheticform of 16 Desmopressin * Recommended treatment table inside front cover

® orSTIMATE ® thatwillstopthebleeding. ® , 15mcg/mlOCTOSTIM ®* , Alphanate ® nasalspraymustbe ® , OCTOSTIM ® , andwilate ® ,STIMATE ® ,STIMATE ® . brand specifictoensurepatientreceivesthecorrect ® ® )

®

- Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays 17 Desmopressin Accurate intake and output should be recorded on any output should be recorded and intake Accurate and fibrin glue (Tisseal-R) may also be used to help control mucous may also be used to help control glue (Tisseal-R) and fibrin ® . Infants and children will require careful fluid intake restriction restriction careful intake fluid will require . Infants children and Topical Preparations age Mix 5 ml Tranexamic acid, use the IV preparation 100mg/ml (5 ml acid, use the IV preparation Mix 5 ml Tranexamic for injection ampule size) and 5 ml sterile water to area. and apply in solution, squeeze out excess Soak gauze solution after 24 hours Discard Mix 2 ml Amicar (IV preparation 250 mg/ml), and 3 ml sterile water for 250 mg/ml), and 3 ml sterile water Mix 2 ml Amicar (IV preparation injection to area. and apply in solution, squeeze out excess Soak gauze solution after 24 hours Discard and Gelfoam ® disease cardiovascular • Hypertensive Tranexamic Acid 5% Tranexamic solution topical Amicar 10% topical Amicar 10% topical solution 24 hours after receiving the drug after receiving 24 hours or finger once or twice a day. once or twice a day. or finger ® : in Type 2B von Willebrand Disease Willebrand 2B von : in Type : pain and erythema at injection site or in nasal mucosa if injection site or in nasal mucosa : pain and erythema at Local is used. spray intranasal : headache, tremor, seizures. tremor, : headache, Neurologic : nausea, vomiting. Gastrointestinal : facial flushing, sweating, dizziness, transient hypertension, hypotension, and tachycardia, hypotension, and tachycardia, hypertension, transient dizziness, sweating, : facial flushing, Cardiovascular hyponatremia. • Elderly 2B Type Disease Willebrand with von • Patients of especially under 2 years children • Young pressure blood • Individuals with low-normal to prevent possible hyponatremia and water intoxication. intoxication. water and hyponatremia possible to prevent Adverse Effects Adverse Use With Caution Use With patients receiving IV fluids. receiving patients Patients should be advised to avoid alcohol and restrict their fluid intake to intake their fluid and restrict alcohol to avoid should be advised effect. antidiuretic has an Desmopressin Patients for only thirst Topical agents such as topical Thrombin such agents Topical Request from Pharmacy from Request and acid 500 mg tablets ten (10) Tranexamic take Acid Nasal gel, a 5% Tranexamic make Acid 5% Nasal Gel : To Tranexamic small amount ofcrush with a very of out 100 grams 70% alcohol. Measure the Intrasite add Gradually (methylcellulose). gel Intrasite with Apply longer). much (probably for 10 days refrigerated Stable put in an ointment jar. Once mixed to the crushed tablets/paste. gel Q-tip Antibiotics and pain medications may also be indicated in the treatment of in the treatment may also be indicated and pain medications Antibiotics bleeds. mucosal membrane bleeding. membrane Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 18 institution's policy. possible, peryour assist withvenipuncture theprepared concentrate, factor andallow toinfuse thepatient if orfamily cost. Occasionally, patients will bringprepared concentrate home venipuncture factor afterunsuccessful attempts. Please Some patients are instructedtobring unmixed concentrate factor withthemtotheEDminimize treatment delayand information concentrate's canbefoundonthefactor box. This unitsinfused. Document thelot number(s),expiration date(s), concentrate factor trade nameandtotalnumberof be discarded! normal coagulation.dosage. concentrate Duetoitscost,factor shouldnever Alarger prolong dosewillonly theperiodof itexceeds thecalculated shouldbeused,even allthe vials reconstituted foraninfusion if The ENTIREcontentsof virally inactivated. units(VWF:RCotin co-factor I.U.). The von Willebrand concentrate factor/FVIII isaplasma-derived that factor hasbeen The dosage tobeadministered isbasedonthepatient’s bodyweight inkilograms (kg) andisnormally ordered inristoce- national units(FVIIII.U.). international units(vWF:RCo I.U.) VIIIinter- andfactor expressed inbothvon Willebrand ristocetinco-factor concentrate factor islabeled withtheactivity Each bottleof Dosage Factor Administration treatment center. It isbesttofollowtreatmentrecommendationsthatthepatientmaycarryorconsultwithpatient's Mixing instructionsandtherateofadministrationare foundonthedruginsert. Products containingvonWillebrandfactorareplasmaderivedproducts. Reconstitute perpackageinsert. 10 ml,2030ml The volumemaybe

(Human) Dried,Pasteurized Labeled: AntihemophilicFactor/vonWillebrandFactorComplex Examples: Humate-P ® , Alphanate ® , wilate Information onfrontofbox 60 ristocetin co-factor unitsX50kg60 ristocetinco-factor = Order: units/kg 60ristocetinco-factor IV Patient’s weight =50kilograms 2625 I.U.VWF:RCo/vial Lot Number:22566611A Expires: June14,2007 866 I.U.FVIII/vial ® Example fordosecalculation 3,000 ristocetin co-factor units 3,000 ristocetinco-factor Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays 19 Other Medications Other ). ® , Percodan ® Ice for 15 -20 minutes minutes after a needle stick Pressure for a minimum of 5 , Excedrin ® Patients with VWD can receive routine medications (e.g. pain medications, antibiotics, etc.) that do not interfere do not interfere that etc.) antibiotics, pain medications, (e.g. medications routine with VWD can receive Patients - with aspirin-re (NSAIDS), ASA and any product anti-inflammatories non-steroidal function. Avoid with clotting Pepto-Bismol (e.g. ingredients lated Routine medications Routine Some patients with VWD may have liver disease from hepatitis or may have been exposed to HIV. Use caution been exposed to HIV. or may have hepatitis from disease liver with VWD may have Some patients serious drug interactions. or could cause potential toxicity may cause liver drugs that prescribing when Caution For any needle stick, pressure for a minimum of 5 minutes afterward will minimize soft tissue or muscle bleeding. ofminimum for a pressure any needle stick, For bleeding. will minimize soft tissue or muscle 5 minutes afterward injections of giving intramuscular because of or immunizations pain medications, Avoid antibiotics, - the possibil for 15 - 20 minutes. an ice pack can also apply ity of You bleed. causing a muscle Medications which can be given PO, SC, or IV are preferred. If preferred. or IV are SC, series is needed, an expe- PO, vaccination can be given the rabies which Medications prior to and after the injections in be contacted for advice should the patient’s) (preferably rienced hematologist . to prevent order Routes of administration Routes Acetaminophen can be given for fever or pain. Narcotics/opioids can be given to control pain experienced by the pain experienced by to control can be given or pain. Narcotics/opioids for fever Acetaminophen can be given injections of giving intramuscular Avoid of because pain medications disorder. with a bleeding patient the pos- ofsibility bleed. causing a muscle Medications for fever or pain Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 20 Fracture Invasive Procedures,Labs,X-rays Head injury Discharge Instructions the nextday. treatment centerorhematologist Patient shouldfollow-upwiththe * drawn orcompleted. Treatment shouldneverbedelayedforlaboratorystudiesto Recommended treatment table inside front cover treatment First givetherecommended First givetherecommended treatment * . * . appropriately. . .thenimmobilize weeks insteadof48hours). (patient shouldbeassessed fortwo routine post head injury instructions Head injury:Dischargewith CT scan. . .thenperforma Centers Intro Mucous Joint / Muscle Factor Labs Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays Invasive Procedures, Labs, X-rays Labs, Procedures, Invasive Recommended treatment table inside front cover 21 cover inside front table treatment Recommended * should be given prior to the procedure. prior to the procedure. should be given * and then proceed with the procedure. Contact the patient’s hematologist hematologist Contact the patient’s with the procedure. and then proceed * , then X-ray and set the bone. and set the bone. , then X-ray * before sending the patient to CT scan. sending the patient before * and precautions should be taken before the procedure begins. the procedure before should be taken and precautions * Invasive procedures should be performed as clinically indicated, i.e. lumbar puncture with symptoms of lumbar puncture i.e. indicated, as clinically should be performed procedures Invasive menin- factor treatment replacement However, gitis. Invasive procedures Invasive for follow-up treatment instructions. No treatment is usually needed for suture removal. is usually needed for suture No treatment instructions. treatment for follow-up Give the recommended treatment the recommended Give Fractures Sutures and staples can be used. If and staples Sutures should first the patient sutures, require is significant enough to the laceration treatment the recommended receive Lacerations and sutures Lacerations is necessary. and no pre-treatment excellent generally hands are any location; may be done at Venipuncture is no further noted at until there minutes or oozing several for pressure Apply for deep veins. “digging” Avoid sites. and IV removal the venipuncture A CT of the maxi- Give hemorrhage. dealing with a potential intracranial 4) is necessary when the head (see pg. treatment recommended mum Remember that a swollen joint or extremity can be the result of can be the result joint or extremity a swollen that of X-rays Remember bleeding. internal the joint can be is treatment when not useful onset bleeds early in detecting generally are but used to document a joint bleed, optimal. X-rays and other radiological studies and other radiological X-rays If the only complaint is an acute joint or muscle bleed, no laboratory studies are necessary. IfIf necessary. studies are no laboratory or GI, uterine, bleed, the only is an acute joint or muscle complaint indicated count may be blood a complete and has potentially is suspected extensive, been bleeding cavity oral studies to be drawn for laboratory if be delayed to determine should never Treatment anemic. the individual is or completed. Laboratory studies Laboratory In general, patients with VWD who are experiencing an acute bleeding episode may need treatment as well as well as episode treatment may need acute bleeding experiencing an are with VWD who patients In general, testing. perform to treatment Do not delay aid measures. basic first If option is available. unless no other sticks arterial then be done, must Do not perform an arterial stick treatment the recommended Arterial sticks and venipunctures Trauma Labs Joint / Muscle Mucous Centers Factor Desmopressin Gyn. GI / GU Head Bibliography X-rays Meds Soft Tissue Membr. Content 22 Trauma /Emergencies Alphanate For any serious injury, a major dose of a factor VIIIproduct afactor containingvon WillebrandFor (eg. seriousinjury, factor any Humate-P amajordose of Treatment emergencies different Many /trauma mayoccurtopersonswithvon Willebrand Disease, justastoothers. mopressin (pg.16-17), (pg. anti-fibrinolytics 9). For lessseriousinjuries, other treatment optionsmaysufficeandcan beconsidered: local treatment (pg. 8),des- etc. * Recommended treatment table inside front cover Fls •Ocularinjuries •Gunshotwounds •Puncture wounds •Motorvehicle accidents Fractures (seepg. 20) • Falls • Burns • Animalbites • ® , wilate ® ) should be infused priortoblood) shouldbeinfused work, CTscan,X-rays orotherscans, debriding, sutures, ® *, Centers Intro Mucous Joint / Muscle Factor Labs Trauma Head GI / GU Gyn. Desmopressin Content Basics Membr. Soft Tissue Meds X-rays Bibliography 23 ). , 10, ® Selected Bibliography Selected 42-51. anticoagulant-treated patients undergoing oral surgery. New England Journal of Journal New England 320, 30-3. Medicine, surgery. oral undergoing patients anticoagulant-treated of Medical Journal anticoagulants. 153: 630. Australia, (Humate-P using factor VIII/VWF concentrate surgery urgent disease undergoing Willebrand with von the treatment of hemophilia and other bleeding disorders – Document # 151. New York, NY : National hemophilia NY : National ofthe treatment # 151. New – Document York, disorders hemophilia and other bleeding Foundation. Hemophilia Monograph No. 35). Montreal, Canada: World Federation of Federation Canada: World Hemophilia. 35). Montreal, No. Hemophilia Monograph Behring Foundation for research and advancement of and advancement for research Behring Foundation care]. patient of disease : results Hemophilia, 6(6), 643-648. Willebrand survey. a patient ofdisease: a survey Hemophilia, 10(2), 158-161. in the United States. centres hemophilia treatment in enrolled 75 women 1277-1299. Ltd. of Publishing Textbook Blackwell Massachusetts: 279-284). Malden, (Eds.), Hemophilia (pp. Ltd. of Publishing Textbook Blackwell 272-278). Malden, Massachusetts: (Eds.), Hemophilia (pp. Behring L.L.C. 103-109). King of Aventis Prussia, PA: disease: 1. Diagnosis, comprehensive care and assessment (2nd Edition) . Toronto, Canada : Association Of Canada : Association Hemophilia Edition) . Toronto, and assessment (2nd care comprehensive Diagnosis, disease: 1. OfClinic Directors Canada Of : Association Canada Of Directors Hemophilia Clinic (2nd Edition) . Toronto, disease: 2. Management Canada Street, A. N., & Leung, W. (1990). Use of Tranexamic acid mouthwash in dental procedures in patients taking oral oral (1990). Use of taking in patients in dental procedures acid mouthwash W. & Leung, A. N., Tranexamic Street, for patients A. (2004). Successful treatment B. & Schwartz, G., M., Mueller-Veltens, B. Ewenstein, A.R., Gill, J.C., Thomson, Sindet-Pederssen, S., Ramstrom, G., Bernvil, S., et al. (1989). Hemostatic effects al. (1989). Hemostatic et of S., Bernvil, G., Ramstrom, S., in acid mouthwash Sindet-Pederssen, tranexamic Medical and Scientific Advisory Council ofMedical and Scientific Advisory concerning Recommendations (2003). MASAC Foundation. National Hemophilia of 42, 29-35. Seminars in Hematology (2005). Treatment disease. & Castaman, G. Willebrand von F., Rodeghiero, Mannucci ,P.M. (2001). Treatment of von Willebrand Disease. Thrombosis and Haemostasis, 80, 149-53. of and Haemostasis, Thrombosis (2001). Treatment Disease. Willebrand von Mannucci ,P.M. Mannucci, P.M. (2004). Treatment of of Journal New England The (2004). Treatment Disease. Willebrand’s von 351, 683-694. Medicine, Mannucci, P.M. Lillicrap, D. (2004). The Basic Science: Diagnosis and Clinical Management of von Willebrand disease. (Treatment of of and Clinical Management (Treatment Basic Science: Diagnosis disease. The (2004). Willebrand von D. Lillicrap, Kouides, P.A., Phatak, P. D., Burkart, P., et al. (2000). Gynecological and obstetrical morbidity in women with type 1 von with type 1 von in women and obstetrical morbidity et al. (2000). Gynecological P., Burkart, D., P. Phatak, P.A., Kouides, of management in clinical Willebrand (2004). Trends with von women B. Evatt, C., A., Lally, Dilley, S., A., Crudder, Kirtava, 42, 42-48. in Hematology, health. Seminars disease and women’s Willebrand (2005). Von R. & Abdul-Kadir, C.A, Lee, Kasper, Carol K. (2004). Von Willebrand Disease.[Monograph]. Los Angeles, USA:.Orthopedic Hospital, [Published by Aventis Aventis by USA:.Orthopedic Hospital, [Published Los Angeles, Disease.[Monograph]. Willebrand K. (2004). Von Carol Kasper, Federici, A. B. (2005). Classification and clinical aspects of von Willebrand disease. In C.A. Lee, E. E. Berntorp, & W. K. Hoots & aspects ofclinical and Classification (2005). E. E. Berntorp, C.A. Lee, In A. B. disease. Willebrand Federici, von K. Hoots & W. E. E. Berntorp, Lee, In C.A. diagnosis. disease: biological Willebrand (2005). Von D. E., & Meyer, Fressinaud, (pp. Principles and Practices Bleeding Disorders: Gioia (Ed.), Congenital Disease . In K. P. Willebrand (2000). Von Gioia, K. P. 36, 28 -35. in Hematology, Seminars and delivery. Pregnancy Bleeding Disorders: and Inherited R.A. (1999). Women Kadir, Cox Gill, J. (2004). Diagnosis and treatment of Clinics of and treatment (2004). Diagnosis Hematology/Oncology Gill, J. disease. Cox Willebrand von America, 18, North Association ofAssociation of Clinic Directors Hemophilia Willebrand’s and von guidelines: hemophilia Clinical practice Canada. (1999). ofAssociation of Clinic Directors Hemophilia Willebrand’s hemophilia and von guidelines: (1999). Clinical practice Canada. of (2006). Compendium Association Canadian Pharmacists Canada. 41th ed. Ottawa, and specialties. pharmaceuticals Acknowledgements

Authors and editors:

Susan C. Zappa, RN, CPN, CPON Sherry L. Purcell, RN Bleeding Disorders Nurse Coordinator Nurse Coordinator Cook Children’s Medical Center Bleeding Disorders Clinic Fort Worth, Texas USA Kingston General Hospital Kingston, Ontario, Canada Lucie Lacasse, RN, BScN Hemophilia Nurse Specialist Karen Wulff, RN The Ottawa Hospital Nurse Coordinator Ottawa, Ontario, Canada The Louisiana Center for Bleeding and Clotting Disorders Rose Jacobson, RN, Nurse Clinician Tulane University School of Medicine MB Bleeding Disorders Program New Orleans, Louisiana USA Health Sciences Centre Winnipeg, Manitoba, Canada

Reviewers:

David Lillicrap, MD, FRCPC Jim Munn, RN, MS Director, Kingston/Belleville Program Coordinator Regional Hemophilia Program University of Michigan Professor, Department of Hemophilia and Coagulation and Molecular Medicine Disorders Program Canada Research Chair in Molecular Ann Arbor, Michigan USA Hemostasis Kingston, Ontario, Canada Annette Smith VWD Type 1 Marcela Torres, MD New Mexico, USA Pediatric Hematology and Oncology Director of Hematology Canadian consumer Cook Children’s Medical Center VWD Type 3 Fort Worth, Texas USA Toronto, Ontario, Canada

Clare Cecchini Program Development Coordinator Canadian Hemophilia Society Montreal, Quebec, Canada

Sponsor Information

Emergency Care for Patients with von Willebrand Disease was made possible by a generous grant from the ZLB Behring Foundation for Research and Advancement of Patient Health.

Production Team - Project Manager: Art Gardner Illustrations: Bob Aul and Mark Gilmore Colorizing: Heather Swaim Design & Layout: Art Gardner