Emergency Care for Patients with Hemophilia

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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 e t n s i o L h P Hemophilia Treatment Centers

4ii 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.

3 Head injury

Treat all head injuries with or without swelling. d a e H

First, infuse a major factor dose.* Then perform diagnostic studies, such as CT scan, neuro exam.

Discharge Instructions Call the hemophilia treatment center or the patient's hematologist for follow-up factor doses.*

Report any signs or symptoms to the hemophilia treatment center or the patient's hematologist.

Head injury instructions for a two week period (instead of the usual instructions for 24 - 48 hour period).

4 *Dose chart inside front cover Head injury

Intracranial hemorrhage (ICH) is the leading cause of death from bleeding in all age groups. Without early recognition and treatment, death or severe neurologic impairment can occur. ICH may be spontaneous, without history of injury. Early neurologic symptoms may not be evident due to the slow, oozing nature of hemophilia bleeding. H e a Treatment d All significant head trauma, with or without hematoma, must be treated promptly with the major dose of factor replacement* before any diagnostic tests.

Diagnostic imaging Obtain an emergency CT scan to rule out ICH after the major factor dose* has been given. Notify the patient's hematologist or hemophilia treatment center of the ED admission and the diagnostic findings.

Possible admission The patient should be admitted to the hospital for observation if he suffered a severe blow to the head or if he exhibits any neurologic symptoms such as headache with increased severity, irritability, vomiting, seizures, vision problems, focal neurologic deficits, stiff neck, or changes in level of consciousness. Patients with a past history of ICH are at increased risk of repeated head bleeds.

Instructions If the patient is discharged home, instruct the family to monitor the patient for signs and symptoms of neurologic deterioration and report any abnormalities to the hematologist. Consult the hematologist for follow-up factor replacement doses if the patient is discharged home from the emergency department.

*Dose chart inside front cover 5 Joint bleeding t n i o J

Advanced joint bleed - heat - pain - swelling - dec reased range of motion Treatment: a major factor dose. *

Crutches for weight bearing joints and crutch instructions.

Early onset joint bleed - tingling - pain - limited range of motion Remembe r, toes and fingers Treatment: a routine factor dose. * are joints too.

Discha rge Instructions For the next 24 hours: - RICE ( rest, ice, comp ression - sling or splinting if support [ace wraps], elevation) is needed (i.e. Ai rcast® for ankles) Follow-up with the hemophilia t reatment center or with the patient's hematologist.

6 *Dose cha rt inside front c over Joint bleeding

The hallma rk o f hemophilia is joint and mus cle bleedin g. Spontaneous joint and mus cle bleeding can occur without a definite histo ry o f t rauma. The p atient may not be able to identify a specific e vent th at resulted in bleedin g.

While persons with hemophilia may bleed into a ny joint space, the joints whi ch most frequent ly bleed a re the ankles, knee s, and elbows. Other possi ble bleeding joint sites in clude the shoulders and hip s. As repe ated bleeding occur s, the syn ovial tissue thi ckens and de velops even mo re fri able blood vessel s. A vicious c ycle o f bleeding and rebleeding may set in and the affected joint is refer red to as a "ta rget joint." E ventual ly, repe ated bleeding into joints leads to a fo rm o f chronic arthritis with destruction o f cartil age and the e ventual destruction o f bone J resulting in dec reased joint mo bility and function. o i n t Signs and symptoms Out ward signs o f joint bleeding include restriction o f m ovement, swellin g, he at, and erythema on and a round the joint. The p atient may report symptoms o f a bu bbling or tin gling sens ation with no p hysical sign s. L ater symptoms in clude a feeling o f fullness within the joint and mode rate to se vere pain as the bleed worsen s.

Treatment Some p atients may p resent for t reatment with no other out ward signs o f bleeding than dec reased ran ge o f motion and a complaint o f pain or tin glin g. This is indic ati ve o f an ea rly onset joint bleed and is the optimal time to t reat. The p atient should be in fused as qui ckly as possi ble with a routine dose o f factor * in o rder to minimize pain and joint destruction. Ext reme pain, swellin g, he at, and immo bility a re signs and symptoms o f an ad vanced joint bleed whi ch occurs on ly after blood has filled the joint spac e. Symptoms su ggesti ve o f an advanced joint bleed requi re a major factor dos e.*

In fuse befo re a ny di agnostic p rocedu res su ch as x- ray. I f a joint bleed is t reated ea rly befo re obvious out ward signs occu r, then the need for expensi ve fol low-up in fusions may be lessened or avoided alto gethe r. Be fore disloc ated joints a re reduced, in fuse with a major factor dos e.*

Joint aspi ration: Caution! Joint bleeds in hemophilia a re gene ral ly not t reated by aspi rating the joint. This p rocedu re can ma ke m atters worse by c reating another site f rom whi ch the p atient may bleed. I f joint aspi ration is deemed necessar y, su ch as in suspected joint s epsi s, then the joint should be aspi rated by an orthopedic su rgeon associ ated with a hemophilia t reatment cente r. The patient's hem ato logist must be i nvol ved in o rder to ar ran ge for factor c overage befo re and after the p rocedu re.

Dis cha rge and fol low-up ca re Upon dis cha rge, the hemophilia p atient with a joint bleed should be instructed to keep the affected joint at rest, ele vate the a ffected lim b, and app ly ice pa cks . A dditional support to the affected joint may be applied by w rapping with an ace band age. For an kle bleed s, an Ai rcast® may be a use ful splinting devic e. Crut ches a re use ful to help individuals when they h ave lower ext remity joint bleeds and need to be non- weight bearin g. Fol low-up should be made to the local hemophilia t reatment center or to the p atient's hem ato logist as soon as possi ble.

*Dose cha rt inside front c over 7 Muscle / soft tissue bleeding

Deltoid / fo rea rm bleed Neck swelling: EMERGENCY - routine factor dose * - potential ai rway - major factor dose * if a comp romise compartment synd rome - major dose of factor * is suspected e l c s u M

Soft tissue bleeds and bruising - no functional impairment - tenderness, but no seve re pain Buttock bleeds - no factor needed - pain - with/without swelling - routine dose of factor * Iliopsoas bleeds - major dose of factor * if - flexed hip the leg on the a ffected - pain/inability to extend side exhibits tingling or the leg on the a ffected swelling side - major factor dose * Thigh/calf bleed and hospital admission - pain - with/without swelling - impai red mobility - routine factor dose * - major factor dose * if a compartment synd rome is suspected

Discha rge Inst ructions - rest - ice - non-weight bearing - follow-up with the hemophilia t reatment center or the patient's hematologist

8 *Dose cha rt inside front c over Muscle / soft tissue bleeding

Signs, symptoms, and sites Mus cle bleeding is common in persons with hemophilia. Any mus cle g roup may be s ubject to bleedin g. Mus cles th at exhi bit warmth, pain, and swelling should be t reated with a routine dose of facto r.* Common bleeding sites in clude the upper arm, fo rea rm, thigh, and calf mus cle s. Toddlers f requent ly h ave butto ck and g roin bleed s. Abdominal wall mus cles and iliopsoas mus cles a re also common bleeding site s. These abdominal mus cles gene ral ly do not have obser vable swellin g, yet they may hold a la rge amount o f blood. Patients who complain of low abdomen or g roin pain, especial ly with signs o f ner ve comp ression, a re p rob ably experiencing an iliopsoas bleed. These p atients should recei ve an eme rgency hem ato logy consult and possi ble admission for obser vation and se veral major doses o f factor c overage.* The hallma rk sign o f iliopsoas bleeding is spontaneous flexion o f the leg on the a ffected side M

with an in ability to extend the leg without pain. u s c l e

Consequences of mus cle bleeds Mus cle bleeds can result in serious consequences i f not t reated p rompt ly. Extensi ve blood loss may occur in la rge mus cle g roup s. Mus cle bleeding places p ressu re on ner ves and blood vessels and, i f left unt reated, these bleeds may result in pe rmanent dis abilities su ch as foot d rop and Volkmann’s cont ractu re. It is important th at the p atient's hem ato logist be consulted befo re a ny su rgical consult s. Most mus cle bleeds respond well to medical man agement and do not requi re fasciotom y. Su ch an ext reme measu re will usual ly gene rate p roblems for the p atient and requi re a t remendous amount o f post-su rgical factor in fusion s, mo re so than i f the patient was treated medical ly.

Treatment and fol low-up ca re Most mus cle bleeds a re t reated with a routine dose o f facto r.* La rge abdominal mus cle g roups and iliopsoas bleeds should be t reated with a major dose o f facto r.* Rest and ice pac ks a re also help ful. The p atient should be refer red ba ck to his hemophilia t reatment center or to his hem ato logist for fol low-up as soon as possi ble. If a ny suspicion of compartment synd rome and nerve comp ression exist s, then the p atient should h ave an eme rgency hem atol ogy consult and should be admitted to the hospit al.

Soft tissue and superficial bleeds Soft tissue bleeds usual ly do not requi re aggressi ve t reatment. Superficial hem atomas and bruises may appear a nywhe re on the body and, i f they do not th reaten function and mo bilit y, they do not need to be t reated.

*Dose cha rt inside front c over 9 Gastrointestinal / urinary tract bleeding

Nausea and vomiting may indicate intracranial hemorrhage as well as gastrointestinal problems. U G / I G

Abdominal pain Treat immediately with a major dose of factor* for: - flank pain - melena - vomiting blood Iliopsoas bleeding - flexed hip - pain on extension - major dose of factor* Hematuria - bed rest for 24 hours - force fluids - consult the hemophilia treatment center or the patient's hematologist

Discharge Instructions - force fluids for hematuria - report any symptoms - rest - follow-up with the hemophilia treatment - no weight lifting center or the patient's hematologist

10 *Dose chart inside front cover Gastrointestinal / urinary tract bleeding

Initial presentation Acute abdominal pain in a patient with hemophilia may have many origins, such as GI tract hematomas (both spontaneous or trauma induced), pseudotumors, iliopsoas or retroperitoneal bleeding. Bleeding may also occur with hemorrhoids or the passage of kidney stones. Notify the hemophilia treatment center or the patient's hematologist.

Patients who present to the emergency department with abdominal or flank pain, melena or hematemesis should be triaged for immediate examination and given factor replacement therapy at the major dosage.* After factor therapy, then diagnostic x-rays, scans and endoscopy procedures can be carried out.

Abdominal trauma and benign events such as forceful coughing or vomiting can precipitate an abdominal bleed. Blood loss can be significant before outward signs and symptoms appear. Infants can have bleeds with gastroenteritis, intussusception or Meckel's Diverticulum. G I / G

A history of lifting heavy objects, weight lifting, falling on bicycle handlebars or stretching U the groin can precipitate abdominal wall, iliopsoas (see pages 8 and 9), or retroperitoneal bleeding.

Symptoms Symptoms of abdominal muscle bleeding (rectus, pectorals, latissimus, obliques) are a palpable mass, rigidity, and pain. Concurrent bleeding in the abdominal cavity may be present and go unnoticed for days with a steadily dropping hemoglobin. Rupture of the liver, spleen, or pancreas should be considered when the hemoglobin falls dramatically following trauma.

For nausea and vomiting without an obvious cause, consider that these may be symptoms of intracranial bleeding. Inquire about head injury, mental status changes, and other neurologic signs and symptoms, and consider CT scan of the head.

Genitourinary bleeding

Hematuria is often frightening to the patient but not a serious event. Instruct the patient to remain at bed rest and force fluids the next 24 hours. Protracted hematuria may require a routine dose of factor coverage.* Anti-fibrinolytics are contraindicated with hematuria. Contact the hematologist.

Scrotal bleeding may occur after trauma, especially in toddlers. Infuse with a routine factor dose* and have the family contact the patient's hematologist for follow-up care.

*Dose chart inside front cover 11 Mucous membrane bleeding

A Dental or E.N.T. consult may be needed.

Nose bleeds may respond to other measures. Refer to . s r u

b "Controlling Epistaxis" table on o m c e u page 14. M M

Mouth bleeds (gum, tooth, frenulum or tongue laceration) need factor* and anti-fibrinolytics. Refer to the "Anti-fibrinolytics" table on page 15.

Anti-fibrinolytics are not routinely available from all pharmacies. Pharmacies associated with the hemophilia treatment centers will be able to fill a prescription.

Discharge Instructions

Patients should follow-up with their Instruct the patient on: hemophilia treatment center or Controlling Epistaxis, Anti-fibrinolytics, Diet hematologist the next day. Modification tables (pages 14 - 15) as needed.

12 *Dose chart inside front cover Mucous membrane bleeding

Mucous memb rane bleeding may requi re medical ca re in the eme rgency d epartment. Patients should recei ve factor who: • a re experiencing p rofuse and/or p rolon ged bleeding • h ave sustained a known inju ry to the mouth, tongu e, or nose • h ave se vere swelling in the mouth or naso-pharynx a rea • a re experiencing respi rato ry dist ress • h ave di fficulty swal lowing

The p atient may not know the reason for the symptom or bleedin g. It may h ave been caused by t rauma, in fection, or the bleed may be spontaneou s. I f air way blockage is suspected, the patient should immedi ately recei ve a major dose o f facto r.* After the factor le vel has been raised, further inter ventions in cluding i nvasi ve p rocedu res may be don e.

Do not ma ke a person with hemophilia wait for factor replacement. The lon ger he wait s, the mo re bleeding ta kes plac e. If the bleed is in a closed spac e, the accu mul ation of blood will cause sur rounding tissue dam age, air way obstruction, and enhance pain.

Epistaxis M M u e c m

A person with epistaxis who is un able to cont rol the bleed himsel f may need a routine dose o b u r s of factor * and anti-fibrino lytic t reatment (Amicar). Be su re the person knows how to cont rol . and stop the bleedin g. (See t able on p age 14.)

Oral C avity

Bleeding in the mouth can be ha rd to cont rol. The p atient will p rob ably need facto r. A sin gle in fusion o f a routine dose o f factor * may tempo rari ly stop the bleedin g, but clots b reak down no rmally on days 3-5 and bleeding may start again at th at tim e. An anti-fibrino lytic may be indic ated to maintain hemostasi s. (See t able on p age 15.) Anti-fibrino lytics may be avail able th rough the patient's home health compa ny for next day deli very. A modified diet should be started at the same time as factor the rapy. (See t able on p age 15.)

Bleeding may occur with erupting or exfoli ating teeth. It is mo re common with exfoli ating teeth, especial ly a tooth th at is very loos e. A dental consult may be needed to ext ract the tooth since it will continue to lace rate the tooth so cket as long as it is in plac e. A major or routine dose of factor * should be gi ven prior to ext raction. A f renulum or tongue lace ration will requi re a major or routine dose o f facto r.*

Ret ropharyn geal

After the major dose o f factor * is gi ven, further obser vation, x- rays and admission may be requi red.

*Dose cha rt inside f ront co ver 13 Mucous membrane bleeding

Cont rolling Epistaxis Instruct the patient:

1. To gently blow his nose to remove mucous and unstable clots which will inte rfe re with hemostasis.

2. Tilt his head forwa rd so any blood will come out the nares and not down the back of the th roat.

3. Apply fi rm pressure to the enti re side of the nose that is bleeding for 15 minutes.

4. Release the pressure to see if bleeding has stopped, blow out any soft clots. . s r u b o m c 5. If the bleeding continues, reapply pressure for another five minutes. e u M M 6. Factor replacement * at a routine dose and/or anti-fibrinolytic agents (see next page) may be needed.

7. During active bleeding, or when the bleeding has stopped, spray or apply two drops of oxymetazoline (ex. NeoSynephrine ®, Dristan ®, or Afrin ®) nasal spray/d rops to the side that was bleeding. These can be used at home PRN for epistaxis.

8. Instruct the patient to use olive oil in the na res to keep the membranes soft and moist, and prevent the fo rmation of ha rd crusts which might crack and restart bleeding.

9. An ENT consult may be r equi red for possible cauterization of a vessel.

14 *Dose cha rt inside front c over Mucous membrane bleeding

Anti-Fibrinolytics Anti-fibrinolytics may also be indicated in nasal or oral bleeding. Amicar and Cyklokapron are both anti-fibrinolytic agents. Either may be prescribed for mucous membrane bleeding to promote clot adhesion in conjunction with factor replacement at a routine dose.* In some cases they may be prescribed without factor replacement.

Amicar - aminocaproic acid Recommended dosage: child: oral dose 50-100 mg/kg (not to exceed 4 Gms) every 6 hours for 3 - 10 days adult: oral dose 3-4 Gms every 6 hours for 3 -10 days

Cyklokapron - tranexamic acid (may not be available in the USA) Recommended dosage: child and adult: oral dose 25 mg/kg every 8 hours for 3 - 8 days

These medications must be given around the clock to keep blood levels constant. M M u These medications may be available through the family's home health company, e c m o b the hemophilia treatment center, or the family may have a supply at home. They u r s are difficult to obtain from most pharmacies. .

Follow-up through the hemophilia treatment center or patient's hematologist.

Topical agents such as Topical Thrombin and Gelfoam may also be used to help control mucous membrane bleeding.

Antibiotics and pain medications may also be indicated.

Diet Modifications Directions for the patient:

1. Diet should be restricted to soft, cool, or lukewarm foods until the area is fully healed. Suggested foods: Jello, noncarbonated drinks, sherbert, lukewarm soups (not cream soups), baby foods, blenderized or pureed foods, spaghetti.

2. Avoid milk products and foods made with milk. Milk products may contribute to clot breakdown and may also cause nausea and vomiting if the patient has swallowed blood.

3. Avoid using a straw. Negative pressure from the sucking action can dislodge the clot and aggravate the bleeding site.

4. Avoid hard foods like chips, popcorn, tacos, etc.

*Dose chart inside front cover 15 Factor administration

Factor Reconstitution

Examples: Antihemophilic factor (AHF) or factor VIII (8) * plasma derived or recombinant r o t c a Coagulation factor IX (9) * F plasma derived or recombinant

Factor box top Mixing instructions and the rate of administration a re found on the d rug insert. Document the lot number(s), expiration date(s), factor concentrate trade name and total number of units infused.

Patients may request the box top for their reco rds.

Some patients a re instructed to bring unmixed factor concentrate with them to the ED to minimize t reatment delay and cost. Occasionall y, patients will bring prepa red factor concentrate after unsuccessful home venipunctu re attempts. Please assist with venipunctu re and allow the patient or family to infuse the prepa red factor concentrate if less than 3 hours have elapsed since reconstitution.

16 *Dose chart inside front c over Factor administration

Dos age Ea ch bottle o f factor concent rate is l abeled with the activity exp ressed as Intern ational Units (I U, example: 287 IU). The dos age to be administe red is based on the p atient's body weight in kilograms (kg) *.

IMPO RTANT! Factor VIII (8) is calcul ated using the fo rmula: 1 IU/ kg = 2% rise in Factor VIII (8) activity These are example s. Pleas e 50 IU/ kg = 100% cor rection (example) refer to the inside fron t cover for specific do se ran ges set by your loca l Factor IX (9) is calcul ated using the fo rmula: hemophilia treatmen t 1 IU/ kg = 1% rise in Factor IX (9) activity cente r. 80 IU/ kg = 80% cor rection (example)

The ENTIRE contents o f all the vials reconstituted for an in fusion should be used, e ven i f it exceeds the calcul ated dos age. A la rger dose will on ly p rolong the period o f no rmal co agul ation. Due to its expens e, factor concent rate should ne ver be disca rded !

The half-li fe o f factor VIII (8) is 8-12 hours; the half-li fe o f factor IX (9) is 18-24 hour s.

Factor types F a c t

Recom binant o Refers to genetically enginee red Factor VIII (8), IX (9) or VII (7) concent rates whi ch a re not r deri ved f rom plasma.

Plasma derived p roduct Factor VIII (8) or IX (9) concent rates o f high specific activity and purity o f the specific clotting factor a chie ved th rough the use o f an a ffinity column m atrix. Some contain von Willebrand factor and are used to treat von Willebrand Disease.

Acti vated p roth rom bin complex concent rates - plasma p roduct Proth rom bin complex concent rates pu rpose ly "acti vated" so they contain some Factor IX (9), Factor X (10), et c. in the acti ve fo rm for use with Factor VIII (8) inhi bitor p atients on ly. The b rand is FEI BA. Dose is 75 IU/kg.

Factor IX (9) p roth rom bin complex concent rates (PCCs) - plasma p roduct Also contains some other clotting factors and small amounts o f acti vated co agul ation factor s. Used to t reat Hemophilia B and some p atients with Factor VIII or IX inhi bitor s.

*Dose cha rt inside front c over 17 Venous access

Do not use the affected or inju red limb for venous access.

No jugular or femoral sticks except in life-saving situations.

Use 25g or 23g "winged" needles. After the needlestick, apply p ressu re and bandage.

See factor administration (pages 16 - 17) for further information. Scalp vein (infants only)

Hickman, B roviac or Port Groshong catheter (use non-coring needle only)

Antecubital fossa (caution in infants) s s u s e o c n c e A V Pe rcutaneous intravenous central catheter ( P.I.C.C.) line

Dorsum of hand

Dorsum of feet (infants/child ren)

18 Venous access

Intravenous access for a person with hemophilia is basically the same as for any patient. The use of 23 gauge or 25 gauge "winged" needles is preferable, especially for children.

Sites for access

Sites to consider for a peripheral IV include:

• dorsum of the hands • antecubital fossa (caution in infants due to the risk of compartment syndrome) • dorsum of feet (infants and children) • scalp veins (infants only)

Groin and neck veins are contraindicated except in life-threatening situations.

The patient's IV should not be started in the affected limb. The injured area should be minimally manipulated, if at all.

Venous access device

Some venous access devices currently used in hemophilia care include:

Port or peripheral port - access with non-coring needle as per your institution's procedure

External central catheter - Hickman, Broviac, or Groshong; access per your institution's V A e c n procedure c o e s u s P.I.C.C. line - access per your institution's procedure s

Heparin flush

It is recommended to do a final flush with heparin for any venous access device (except for Groshong's which are only flushed with normal saline). Check with the parent or the patient's institution for the amount of heparin flush to use. If these options are not available, use your institution's procedure for the amount and concentration of heparin. This small amount of heparin will not harm the patient (remember - you've just given him factor). The access device needs to stay patent; this is accomplished with the heparin.

The patient on "prophylaxis"

Some patients with hemophilia now receive "prophylactic" doses of factor replacement on an every-other-day or 2-3 times a week routine schedule. This should be asked of the patient. These patients usually have venous access devices for infusions. So, always check to see if the patient has a venous access device before starting a peripheral infusion. 19 Invasive procedures, labs, x-rays

X-rays and lab are not indicated for a joint or muscle bleed.

Head injury (see page 5)

First give a major . . . then perform dose of factor* . . . a CT scan.

Fracture

First give a major . . . then apply y s dose of factor* . . . the cast. a b R a - L X

Discharge Instructions Patient should follow-up with the Sutures: Remind the patient he will hemophilia treatment center or his need factor for suture removal. hematologist the next day.

Head injury: Discharge with routine post head injury instructions (patient to follow-up for two weeks instead of 48 hours).

20 *Dose chart inside front cover Invasive p rocedu res, labs, x-rays

In gene ral, p atients with hemophilia who a re experiencing an acute bleeding episode need factor on ly. Other p rocedu res should not be done unless the re is another clinical indic ation for the stud y. In a ny situ ation, the in fusion o f factor should never be del ayed if a ny bleeding is suspected. Delaying the in fusion simp ly inc reases bleeding th at will result in g reater mor bidit y.

Labo ratory studies If the on ly complaint is an acute joint or mus cle bleed, no l abo rato ry studies are necessar y. I f GI, abdominal, la rge mus cle, or o ral c avity bleeding is suspected and has potential ly been extensi ve, a CBC may be indic ated to dete rmine i f the individual is anemi c. Factor le vels and inhi bitor le vels are not necessa ry for t reatment in an acute eme rgency settin g. Factor should not be del ayed for l abo ratory studies to be d rawn or completed.

X- rays and other radio logical studies Give factor first , then decide i f a radio logical study is indic ated. Remember th at a swollen joint or ext remity is usually the result o f internal bleedin g, not a f ractu re. X- rays o f the joint can be used to document a joint bleed, but a re gene ral ly not use ful in detecting ea rly onset bleeds (and th at is when treatment is optimal). The p atient will be aware o f joint bleeding befo re radio logical chan ges a re evident.

A CT o f the head (see p age 5) is necessa ry when dealing with a potential intracranial hemorrhage. It can document l oc ation and extent o f bleeding and help di rect further t reatment. First gi ve a major factor dos e.* Fractu res Gi ve a major dose o f factor * replacement, then x- ray and set the bon e.

Lace rations and sutu res Sutu res and st aples should be used as on a ny other p atient. I f the lace ration is significant enough to X requi re sutu res, the p atient should first recei ve a routine dose o f factor * then the p rocedu re. Contact L - a R b the p atient's hem ato logist for fol low-up factor in fusion instruction s. For rem oval o f sutu res, a routine a s y dose o f factor * is usual ly needed.

Invas ive procedu res Invasi ve p rocedu res should be perfo rmed as clinical ly indic ated, i. e., lumbar punctu re with symptoms of meningiti s. Ho wever, a major dose o f factor * should be gi ven befo re the p rocedu re begin s.

Arterial sticks and venipunctu res Do not do arterial sti cks unless no other option is avail able. I f an arterial sti ck must be don e, then a major factor dose * must be gi ven first.

Venipunctu re (see p age 19) may be done at any loc ation; hands a re gene ral ly e xcellent and no factor treatment is necessa ry. Avoid "di gging" for de ep vein s. App ly p ressu re for se veral minutes after the punctu re.

*Dose cha rt inside front c over 21 Other medications

Pressure and ice pack after injections

Routine medications

Patients with hemophilia can receive routine medications (e.g. pain medications, antibiotics, etc.) that do not interfere with clotting function. Avoid non-steroidal anti-inflammatories (NSAIDS), ASA and any product with aspirin-related ingredients (e.g. Pepto-Bismol, Excedrin, Percodan).

Medications for fever or pain

Acetaminophen can be given for fever or pain. Narcotics/opioids are often recommended to control the pain experienced by a patient with hemophilia. Avoid giving intramuscular injections of antibiotics, pain medications, or immunizations because of the possibility of causing a muscle bleed. / a s

m Routes of administration d u e a r M T Medications which can be given PO, SC, or IV are preferred. Routine immunizations and tetanus toxoid may be given subcutaneously. If the rabies vaccination series is needed, an experienced hematologist (preferably the patient's) should be contacted to arrange factor infusions prior to and after the injections in order to prevent internal bleeding.

For any needle stick, pressure and an ice pack afterward will minimize soft tissue or muscle bleeding.

Caution

Some patients with hemophilia may have liver disease from hepatitis. Use caution when prescribing drugs that may cause liver toxicity. Other patients may be on other therapies for hemophilia-related complications such as HIV or hepatitis. Be aware of potential serious drug interactions.

22 Trauma / emergencies

Trauma / emergencies

Many different emergencies/traumas may occur to persons with hemophilia, just as to others. The more common are:

• Animal bites • Motor vehicle accidents M e

• Burns • Myocardial infarctions d • Falls • Ocular injuries s /

• Fractures (see page 21) • Puncture wounds T r a

• Gunshot wounds • Dislocated joints u m a Treatment

A major dose of factor* should be infused as soon as possible (before any test, x-rays, debriding, sutures, etc.).

*Dose chart inside front cover 23 Inhibitors

Contact the hemophilia t reatment center or the patient's hematologist due to the complexity of managing inhibitors.

High Responding Inhibitors Products used to t reat bleeding:

Activated PCC e.g. FEIBA

Factor IX complex concentrates (PCC) Used for Factor VIII (8) or IX (9) patient e.g. Profilnine, Bebulin

Recombinant F VIIa (7) (activated) e.g. NovoSeven

Low Responding Inhibitors

Factor p roduct and dose is variable. Dose may be 2-3x routine range. * s r o t i b i h n I

24 *Dose cha rt inside front c over Inhibitors

Definition An inhibitor is an antibody that some individuals with hemophilia develop against factor VIII (8) or IX (9). These antibodies neutralize the factor procoagulant activity, thus counteracting the desired effect of an infusion of factor concentrate.

How inhibitors are measured

Labs in the U.S. express the presence of an inhibitor in terms of Bethesda units (BU). One Bethesda unit is the amount of antibody that destroys half of the factor VIII in an equal mixture of normal and patient plasma in two hours.

Low responding inhibitor

Measures less then 10 BU.

High responding inhibitor

Measures greater than 10 BU. An infusion of factor concentrate further stimulates the inhibitor antibodies, causing a rise in BUs.

When to suspect an inhibitor

Suspect an inhibitor if bleeding doesn't stop after several infusions of factor concentrates.

Call the patient's hematologist if the inhibitor is known or suspected before attempting treatment. Be sure to also ask the patient and family if they have been told the patient has an inhibitor.

Treatment of inhibitors

Inhibitor management is difficult for the experienced hematologist. Contact the patient's hematologist or hemophilia treatment center when these patients present in the emergency department for treatment. Bleeding in an inhibitor patient can quickly lead to serious life- or limb-threatening complications without expert management. I n h i b i t o r s

25 Acknowledgements

Emergency Care for Patients with Hemophilia was written and prepared by the Nursing Group of Hemophilia Region VI (AR, LA, OK, TX) in order to help facilitate prompt care of patients through their emergency departments. The group wishes to thank the following for their contributions: Reviewers: Nurses from Region VI Hemophilia Treatment Centers: Howard Britton, M.D. Cook Children's Medical Center, Ft. Worth, TX Medical Director, South Texas Comprehensive Gulf States Hemophilia Diagnostic and Treatment Center, Houston, TX Hemophilia Center, San Antonio, TX Cindy Leissinger, M.D. Hemophilia Center of Arkansas, Little Rock, AR Medical Director, Louisiana Comprehensive Louisiana Comprehensive Hemophilia Care Center, New Orleans, LA Hemophilia Care Center, New Orleans, LA North Texas Comprehensive Hemophilia Center, Arlo Weltge, M.D. Adult Program, Dallas, TX Associate Professor of Surgery/ER Medicine, The University of Texas Medical School, Houston, TX North Texas Comprehensive Hemophilia Center, Rita Gonzales Pediatric Program, Dallas, TX consumer parent and past President, Lone Star The Oklahoma Center for Bleeding Disorders, Oklahoma City, OK Hemophilia Chapter, Houston, TX South Texas Comprehensive Hemophilia Center, San Antonio, TX