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Emergency Care for Patients with Hemophilia

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Emergency Care for Patients with Hemophilia Eme rgency Ca re for Patients with Hemophilia An instruction al ma nual for Medic al Profession als Third Edition Written by the Nursing G roup of Hemophilia Region VI Editors: Karen Wulff , R.N.; Susan Z appa R.N., C. P.N., C. P.O.N.; Mack Womack , R.N. Determine if the patient has: Hemophilia A or factor VIII (8) deficiency Hemophilia B or factor IX (9) deficiency Head inju ry (pg. 4) Always t reat immediately with a major factor dose Mucous membrane bleeds (pg. 12) Treat with a routine factor dose and anti-fibrinolytics Slings, splints, immobilizers p. r.n. for joint bleeds Joint bleeds (pg. 6) Treat an early onset bleed with a routine factor dose. Treat an advanced joint bleed with Avoid intramuscular a major factor dose. injections due to the possibility of causing a muscle bleed Abdominal bleeds (pg. 10), Trauma (pg. 23) Crutches Treat with a major factor dose hip, knee, ankle bleeds Minor cuts / bruises no t reatment Ice pack / Ace wrap joint bleeds Factor Replacement Doses TREATMENT FOR MINOR BLEEDS Minor bleeds include: Nose (epistaxis), Mouth (including gums) Joints (hemarthroses), Abrasions and superficial lacerations Hemophilia A or Factor VIII (8) Recombinant Factor VIII concentrate Severe to moderate deficiency: Dosage 20 - 30 units per kilogram Hemophilia A or Factor VIII (8) DDAVP responsive: DDAVP dosage: 0.3 micro- Mild deficiency: grams per kilogram (Maximim dose: 20 micrograms) DDAVP non-responsive: Recombinant Factor VIII concentrate. Dosage 20 - 30 units per kilogram Hemophilia B or Factor IX (9) deficiency Recombinant Factor IX concentrate Severe/Moderate/Mild: Dosage 35 - 50 units per kilogram For mucosal bleeds in all of the above add: Tranexamic Acid (Cyklokapron) “Topical (mouth- wash) or systemic” as per bleeding site (Contraindicated if hematuria) TREATMENT FOR MAJOR BLEEDS/LIFE-THREATENING BLEEDS Head (intracranial) and Neck, Chest, Abdomen, Pelvis, Spine, Iliopsoas Muscle and Hip, Extremity muscle compartments, fractures or dislocations, any deep lacerations Hemophilia A or Factor VIII (8) Recombinant Factor VIII concentrate Severe/Moderate/Mild deficiency Dosage 40 - 50 units per kilogram Hemophlia B or Factor IX (9) Recombinant Factor IX concentrate Severe/Moderate/Mild deficiency Dosage 100 - 120 units per kilogram HemophiliaProgramof BC Adult Division St. Paul's Hospital 1081 Burrard Street Comox Building, Room 217 Vancouver, B.C. V6Z 1Y6 Ph 604-806-8855 • Fax 604-806-8784 Toll-free 1-877-806-8855 Medical Director Dr. Linda Vickars Clinical Nurse Specialist Deb Gue, RN, MSN [email protected] Patient/Nurse Educator Kam McIntosh, RN, BSN [email protected] Program Secretary t r Alex Weatherston [email protected] a h C All major bleeding episodes should be considered potentially critical. e s The goal is to raise the factor level to 80 - 100%. o D Note: For patients with Factor 8 and 9 inhibitors, it is imperative that you contact the patient’s hemophilia physician before any infusions. Contact information and numbers are located above. C o n t e Table of Contents n t Introduction 1 Hemophilia basics 2 Head injury 4 Joint bleeding 6 Signs & symptom s, Aspi ration, Disloc ated joints Muscle /soft tissue bleeding 8 Compartment synd rome, Iliopsoas Gast rointestin al / urinary t ract bleedin g 10 Abdomin al mus cle wall, Hem aturia Mucous membrane bleeding 12 Anti- fibrino lytic s, Epistaxi s, Ret ropharyn geal bleeds Factor administration 16 Mixin g, Dos age for mula, Factor t ypes Venous access 18 Site for administr ation, P rop hylaxi s, Venous access devices Invas ive p rocedu res / l abs / x- ray 20 Fractu res, Lace ration s, L P, Blood gases Other medications 22 Pain medic ation s, Im muniz ations Trauma / emergencies 23 Bite s, Burn s, Fall s, Fractu res, Gunshot wound s, M VA, MI, Ocular injurie s, Punctu re wounds Inhibitors 24 De finition, Treatment Co pyright © 1999, 2010 The Nursing G roup o f Hemophilia Region VI. All rights reser ved. Permission g ranted to photoco py for educational pu rposes on ly. All b rand names and p roduct names used in this p ublic ation a re t rade name s, service ma rks, t radema rks, or registe red t radema rks o f their respecit ve owner s. These te rms a re used in this pu blic ation on ly in an editorial fashion and should not be regarded as a ffecting their validit y. i Phone List 4 ii Hemophilia Treatment Centers Treatment Introduction I n t r Purpose o This manual contributes to hemophilia care by enhancing the emergency department staff's understanding of hemophilia and its treatment. The goals of this manual are to: • promote understanding of the complexities of hemophilia treatment • provide a reference for the emergency center staff • promote a consultative dialogue with the emergency department, hemophilia treatment center, and patient/family Use This manual provides a standardized format for evaluation and treatment of hemophilia emergencies. The content is segmented by systems and complications of hemophilia. Turn to an area of interest. The illustration on the left page provides information points for quick review. The text on the right page gives further detail of bleeding presentations, their possible complications and treatment. It is suggested that the patient's hemophilia treatment center or hematologist be consulted for anything other than routine bleeding episodes. To The Attending Medical Staff: This manual is a guide for medical personnel who may be less familiar with hemophilia treatment. Its content consists of guidelines, recommendations and suggestions only. The attending physician has the final responsibility for appropriate diagnosis and treatment. 1 Hemophilia basics s c i s a B Definition Hemophilia is a genetic diso rder cha racterized by a deficiency or absence o f one of the clotting proteins in plasma. The result is dela yed clottin g. Deficiencies of factor VIII (8) [Hemophilia A or Classic Hemophilia] and factor IX (9) [Hemophilia B or Christmas Disease] a re the most common and refer red to as hemophilia. Hemophilia most ly a ffects males due to the X-lin ked inheritance p attern. Effects of hemophilia Hemophilia pre vents the fo rmation o f a fi rm, fibrin clot and results in a soft, unst able clot. Persons with hemophilia do not bleed faster than others; rather the bleeding is continuou s. Significant blood loss can occur i f t reatment is dela yed. Incidence The incidence worldwide is estim ated to occur in 1:7,500 li ve male births; all races and ethnic g roups a re a ffected. Factor VIII (8) deficiency is four times mo re common than factor IX (9) deficiency but the clinical p resent ations and inheritance patterns a re the sam e. Severity The amount o f bleeding expected in an individual with hemophilia d epends upon the se verity o f the deficienc y. No rmal plasma le vels o f factor VIII (8) and IX (9) ran ge f rom 50-150%. Those with less than 1% factor VIII (8) and IX (9) a re conside red to h ave se vere hemophilia . Frequent bleeding episodes a re common, particula rly into joint s. Bleeding can occur for no known reason or f rom t rauma. Persons with factor le vels o f 1-5% a re conside red to h ave mode rate hemophilia . These persons may experience bleeding after minor t rauma. After repeated bleeding into the same joint, persons with mode rate hemophilia may experience bleeding in th at joint with minor trauma. Persons with mo re than 5% factor activity a re conside red to h ave mild hemophilia and bleed on ly after significant t rauma or with su rg er y. Some carrier gi rls and women (called symptom atic carriers) can h ave lower than no rmal plasma le vels o f factor VIII (8) or IX (9) and thus may exhi bit symptoms o f mild hemophilia. 2 Hemophilia basics Serious bleeding sites B The six major sites of serious bleeding which th reaten li fe, lim b, or function a s i c are: s • int rac rani al • spin al co rd • th roat • int ra- abdomin al • limb compartments • ocular All of the above requi re immedi ate assessment and inter vention, and a re cha racteri zed by: • bleeding into an enclosed space • comp ression of vit al tissues • potenti al loss of li fe, lim b, or function Treatment Treatment for bleeding invol ves replacing the deficient factor as the first course of action. This requi res intr avenous in fusion o f comme rcial factor concent rate s. Specific dose s, additional drugs and medical inter ventions depend upon the site and se verity of bleedin g. Once factor replacement the rapy has been in fused, di agnostic p rocedu res and examin ations can begin. Fami ly Parents and persons with hemophilia a re knowled geable about the man ag ement o f the diso rder and their input should be sought and heeded. Most hemophilia families a re medical ly sophistic ated and should not be dismissed as n ovice s. Intervi ew the fami ly about whether factor concent rate has been administe red prior to arriving at the ED; i f s o, dete rmine when and at what dos e. A dditional factor may be requi red, d epending upon the time l ag and se verity o f the bleed. Est ablish who the treating hem ato logist or hemophilia t reatment center is and contact them for other than routine bleedin g.
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