Emergency Care for Patients with Hemophilia · Hemophilia basics Definition Hemophilia is a genetic disorder characterized by a deficiency or absence of one of the clotting proteins

Emergency Care forPatients with Hemophilia

Slings, splints, immobilizersp.r.n. for joint bleeds

Head injury (pg. 4)

Always treat immediately with amajor factor dose

Mucous membrane bleeds (pg. 12)

Treat with a routine factor doseand anti-fibrinolytics

Joint bleeds (pg. 6)

Treat an early onset bleed with aroutine factor dose. Treat anadvanced joint bleed witha major factor dose

Determine if the patient has: Hemophilia A or factor VIII (8) deficiency Hemophilia B or factor IX (9) deficiency

Avoid intramuscularinjections due to thepossibility of causing amuscle bleed

Crutches hip, knee, ankle bleeds

Ice pack / Ace wrap joint bleeds

Minor cuts / bruises no treatment

Abdominal bleeds (pg. 10),

Trauma (pg. 23)

Treat with a major factor dose

Sixth Edition

Written by the Nursing Group of Hemophilia Region VIEditors: Karen Wulff, R.N.; Susan Zappa R.N., C.P.N., C.P.O.N.; Mack Womack, R.N.

An instructional manual for Medical Professionals

Dose

Ch

art

Hemophilia Treatment Center

Factor Replacement Doses

Add your HHTC information here

Add you treatment for both routine factor dose and major factor dose for both hemophilia A and Hemophilia B

8 gnideelb eussit tfos/ elcsuMCompartment syndrome, Iliopsoas

Copyright © 1999, 2010, 2017, 2019 The Nursing Group of Hemophilia Region VI.All rights reserved. Permission granted to photocopy for educational purposes only.

All brand names and product names used in this publication are trade names, service marks, trademarks, orregistered trademarks of their respecitve owners. These terms are used in this publication only in an editorialfashion and should not be regarded as affecting their validity.

i

Table of Contents

1 noitcudortnI

2 scisab ailihpomeH

4 yrujni daeH

6 gnideelb tnioJSigns & symptoms, Aspiration, Dislocated joints

Gastrointestinal / urinary tract bleeding 10Abdominal muscle wall, Hematuria

21gnideelb enarbmem suocuMAnti-fibrinolytics, Epistaxis, Retropharyngeal bleeds

61noitartsinimda rotcaFMixing, Dosage formula, Factor types

81ssecca suoneVSite for administration, Prophylaxis, Venous access devices

Invasive procedures / labs / x-ray 20Fractures, Lacerations, LP, Blood gases

22snoitacidem rehtOPain medications, Immunizations

32seicnegreme / amuarTBites, Burns, Falls, Fractures, Gunshot wounds, MVA, MI, Ocularinjuries, Puncture wounds

42srotibihnIDefinition, Treatment

tne

tn

oC

Hemophilia Treatment Centers

Ph

on

eLi

st

Hemophilia Treatment Center (HTC) addresses, telephone numbers and other contact information can be found at: www.cdc.gov/ncbddd/hemophilia/index.html

Canadian Hemophilia Society World Federation of Hemophilia CDCwww.hemophilia.ca www.wfh.org www.cdc.gov

National Hemophilia Foundation Hemophilia Federation of America1-800-42-HANDI www.hemophiliafed.org www.hemophilia.org

1

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, nurse practitioner and physician assistant has the final responsibility for appropriate diagnosis and treatment.

Purpose

This manual contributes to hemophilia care by enhancing the emergency department staff's understanding of hemophilia and its treatment. The goals ofthis manual are to:

• promoteunderstandingof thecomplexitiesof hemophiliatreatment

• provideareferencefortheemergencycenterstaff

• promoteaconsultativedialoguewiththeemergencydepartment,hemophilia treatment center, and patient/family

Use

Thismanualprovidesastandardizedformatforevaluationandtreatmentof hemophilia emergencies. The content is segmented by systems and complications of hemophilia. Turn to an area of interest. The illustration on the left page providesinformationpointsforquickreview.Thetextontherightpagegivesfurther 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.

Intro

Introduction

2

Hemophilia basics

Definition

Hemophiliaisageneticdisordercharacterizedbyadeficiencyorabsenceof oneof the clotting proteins in plasma. The result is delayed clotting. Deficiencies of factor VIII (8) [Hemophilia A or Classic Hemophilia] and factor IX (9) [Hemophilia B or Christmas Disease] are the most common and referred to as hemophilia. HemophiliamostlyaffectsmalesduetotheX-linkedinheritancepattern.

Effects of hemophilia

Hemophiliapreventstheformationof afirm,fibrinclotandresultsinasoft,unstable clot. Persons with hemophilia do not bleed faster than others; rather the bleeding is continuous. Significant blood loss can occur if treatment is delayed.

Incidence

Theincidenceworldwideisestimatedtooccurin1:5,000livemalebirths;allraces and ethnic groups are affected. Factor VIII (8) deficiency is four times more common than factor IX (9) deficiency but the clinical presentations and inheritance patterns are the same.

Severity

Theamountof bleedingexpectedinanindividualwithhemophiliadependsupontheseverityof thedeficiency.Normalplasmalevelsof factorVIII(8)andIX(9)range from 50-150%.

Thosewithlessthan1%factorVIII(8)andIX(9)areconsideredtohavesevere hemophilia.Frequentbleedingepisodesarecommon,particularlyintojoints.Bleeding can occur spontaneously or from trauma.

Personswithfactorlevelsof 1-5%areconsideredtohavemoderate hemophilia. Thesepersonsmayexperiencebleedingafterminortraumabutshouldnotbleedspontaneously.Afterrepeatedbleedingintothesamejoint,personswithmoderatehemophiliamayexperiencespontaneousbleedinginthatjoint.

Personswithmorethan5%factoractivityareconsideredtohavemild hemophilia and bleed only after significant trauma or with surgery. Some carriergirlsandwomen(calledsymptomaticcarriers)canhavelowerthannormalplasmalevelsof factorVIII(8)orIX(9)andthusmayexhibitsymptomsof mildhemophilia.

Bas

ics

3

Serious bleeding sites

The seven major sites of serious bleeding which threaten life, limb, or function are:

• intracranial• spinal cord• throat• intra-abdominal• limb compartments• ocular• gastrointestinal

All of the above require immediate assessment and intervention, and are characterized by:

• bleeding into an enclosed space• compression of vital tissues• potential loss of life, limb, or function

Treatment

Treatmentforbleedinginvolvesreplacingthedeficientfactorasthefirst course of action.Thisrequiresintravenousinfusionof commercialfactorconcentrates.Specificdoses,additionaldrugsandmedicalinterventionsdependuponthesiteandseverityof bleeding. Once factor replacement therapy has been infused, diagnostic procedures andexaminationscanbegin.Peoplewithhemophiliaareatriskforjointandmuscledamage.Aggressivetreatmentandfollow-upisrecommended.ConsultwithHemophiliaTreatmentCenterforanysignificantmusculo-skeletalbleeding.Whenindoubt-infusefactorreplacement.Femalesmayhavelowfactorlevelsandrequiretreatment.

Family

Parentsandpersonswithhemophiliaareknowledgeableaboutthemanagementof thedisorder and their input should be sought and heeded. Most hemophilia families are medicallysophisticatedandshouldnotbedismissedasnovices.

InterviewthefamilyaboutwhetherfactorconcentratehasbeenadministeredpriortoarrivingattheER;if so,determinewhenandatwhatdose.Additionalfactormayberequired,dependinguponthetimelagandseverityof thebleed.Establishwhothetreating hematologist or hemophilia treatment center is and contact them for other than routine bleeding.

Hemophilia basics

Basics

4

Discharge Instructions

Head injury

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

Treat all head injuries with or without swelling.

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).

*Dose chart inside front cover

Hea

d

5

Head injury

Intracranial hemorrhage (ICH) is the leading cause of death from bleeding in all agegroups.Withoutearlyrecognitionandtreatment,deathorsevereneurologicimpairmentcanoccur.ICHmaybespontaneous,withouthistoryof injury.Earlyneurologicsymptomsmaynotbeevidentduetotheslow,oozingnatureof hemophilia bleeding.

Treatment

All significant head trauma, with or without hematoma, must be treated promptly withthemajordoseof factorreplacement* before any diagnostic tests. Additional days/weeksof factorreplacementwillbenecessary.ConsultHemophiliaTreamentCenter.

Diagnostic imaging

ObtainanemergencyCTscantoruleoutICHafterthemajorfactordose* has beengiven.Notifythepatient'shematologistorhemophiliatreatmentcenterof theEDadmissionandthediagnosticfindings.

Possible admission

Thepatientshouldbeadmittedtothehospitalforobservationif hesufferedasevereblowtotheheadorif heexhibitsanyneurologicsymptoms.Thismayincludeheadachewithincreasedseverity,irritability,vomiting,seizures,visionproblems,focalneurologicdeficits,stiff neck,orchangesinlevelof consciousness.Patients with a past history of ICH are at increased risk of repeated head bleeds. Aggressivefactorreplacementwillberequiredfordays/weeks.

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.


Head

6

Early onset joint bleed- tingling- pain- limited range

of motionTreatment: a routine factor dose.*

Joint bleeding


Advanced joint bleed- heat- pain- swelling- decreased range

of motionTreatment: a major factor dose.*

Remember, toes and fingers are joints too.

Crutches for weight bearing joints and crutch instructions.

- RICE (rest, ice, compression [ace wraps], elevation)

For the next 24 hours:

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


- sling or splinting if supportis needed (i.e. Aircast® for ankles)

Join

t

7

Remember, toes and fingers are joints too.

Thehallmarkof hemophiliaisjointandmusclebleeding.Spontaneousjointandmusclebleeding can occur without a definite history of trauma. The patient may not be able to identifyaspecificeventthatresultedinbleeding.

Whilepersonswithhemophiliamaybleedintoanyjointspace,thejointswhichmostfrequentlybleedaretheelbows,knees,andankles.Otherpossiblebleedingjointsitesincludetheshouldersandhips.Asrepeatedbleedingoccurs,thesynovialtissuethickensanddevelopsevenmorefriablebloodvessels.Aviciouscycleof bleedingandrebleedingmaysetinandtheaffectedjointisreferredtoasa"targetjoint."Eventually,repeatedbleedingintojointsleadstoaformof chronicarthritiswithdestructionof cartilageandtheeventualdestructionof boneresultingindecreasedjointmobilityandfunction.

Signs and symptoms

Outwardsignsof jointbleedingincluderestrictionof movement,swelling,heat,anderythemaonandaroundthejoint.The patient may report symptoms of a bubbling or tingling sensation with no physical signs. Later symptoms include a feeling of fullness withinthejointandmoderatetoseverepainasthebleedworsens.

Treatment

Some patients may present for treatment with no other outward signs of bleeding than decreasedrangeof motionandacomplaintof painortingling.Thisisindicativeof anearly onset joint bleed andistheoptimaltimetotreat.Thepatientshouldbeinfusedasquicklyas possible with a routine dose of factor*inordertominimizepainandjointdestruction.Extremepain,swelling,heat,andimmobilityaresignsandsymptomsof anadvanced joint bleed whichoccursonlyafterbloodhasfilledthejointspace.Symptomssuggestiveof anadvancedjointbleedrequireamajorfactordose.*

Infusebeforeanydiagnosticproceduressuchasx-ray.If ajointbleedistreatedearlybeforeobviousoutwardsignsoccur,thentheneedforexpensivefollow-upinfusionsmaybelessenedoravoidedaltogether.Before dislocated joints are reduced, infuse with a major factor dose.*

Joint aspiration: Caution!

Jointbleedsinhemophiliaaregenerallynottreatedbyaspiratingthejoint.Thisprocedurecanmakemattersworsebycreatinganothersitefromwhichthepatientmaybleed.If jointaspirationisdeemednecessary,suchasinsuspectedjointsepsis,thenthejointshouldbeaspirated by an orthopedic surgeon associated with a hemophilia treatment center. The patient'shematologistmustbeinvolvedinordertoarrangeforfactorcoveragebeforeandafter the procedure.

Discharge and follow-up care

Upondischarge,thehemophiliapatientwithajointbleedshouldbeinstructedtokeeptheaffectedjointatrest,elevatetheaffectedlimb,andapplyicepacks.Additionalsupporttotheaffectedjointmaybeappliedbywrappingwithanacebandage.Foranklebleeds,anAircastmaybeausefulsplintingdevice.Crutchesareusefultohelpindividualswhentheyhavelowerextremityjointbleedsandneedtobenon-weightbearing.Follow-up should be made to the local hemophilia treatment center or to the patient's hematologist as soon as possible.


Join

t

Joint bleeding

8

Deltoid / forearm bleed- routine factor dose*- major factor dose* if a

compartment syndromeis suspected

Thigh/calf bleed- pain- with/without swelling- impaired mobility- routine factor dose*- major factor dose* if a

compartment syndromeis suspected

Neck swelling: EMERGENCY- potential airway

compromise- major dose of factor*

Soft tissue bleedsand bruising

- no functionalimpairment

- tenderness, butno severe pain

- no factor needed

Buttock bleeds- pain- with/without swelling- routine dose of factor*- major dose of factor* if

the leg on the affectedside exhibits tingling orswelling

- common in toddler age

Iliopsoas bleeds: EMERGENCY- flexed hip- pain/inability to extend

the leg on the affectedside

- major factor dose*and hospital admissionDays to weeks of factorreplacement will berequired


- rest- ice- non-weight bearing- follow-up with the hemophilia treatment

center or the patient's hematologist


Musc

le

Muscle / soft tissue bleeding

9

Signs, symptoms, and sites

Musclebleedingiscommoninpersonswithhemophilia.Anymusclegroupmaybesubjecttobleeding.Musclesthatexhibitwarmth,pain,andswellingshouldbetreated. Common bleedingsitesincludetheupperarm,forearm,thigh,andcalf muscles.Toddlersfrequentlyhavebuttockandgroinbleeds.Abdominalwallmusclesandiliopsoasmusclesarealsocommonbleedingsites.Theseabdominalmusclesgenerallydonothaveobservableswelling,yet they may hold a large amount of blood. Patients who complain of low abdomen or groin pain,especiallywithsignsof nervecompression,areprobablyexperiencinganiliopsoasbleed.Thesepatientsshouldreceiveanemergencyhematologyconsultandpossibleadmissionforobservationandseveralmajordosesof factorcoverage.*Thehallmarksignof iliopsoasbleedingisspontaneousflexionof thelegontheaffectedsidewithaninabilitytoextendtheleg without pain.

Consequences of muscle bleeds

Musclebleedscanresultinseriousconsequencesif nottreatedpromptly.Extensivebloodlossmayoccurinlargemusclegroups.Musclebleedingplacespressureonnervesandbloodvesselsand, if left untreated, these bleeds may result in permanent disabilities such as foot drop and wrist contracture. It is important that the patient's hematologist be consulted before any surgicalconsults.Mostmusclebleedsrespondwelltomedicalmanagementanddonotrequirefasciotomy.Suchanextrememeasurewillusuallygenerateproblemsforthepatientandrequirea tremendous amount of post-surgical factor infusions, more so than if the patient was treated medically.

Treatment and follow-up care

Mostmusclebleedsaretreatedwithamajordoseof factor.* Large abdominal muscle groups andiliopsoasbleedsshouldbetreatedwithamajordoseof factor.* Aggressive factor replacement will be required due to possible large volume of blood loss.Restandicepacksarealsohelpful.Thepatientshouldbereferredbacktohishemophiliatreatmentcenteror to his hematologist for follow-up as soon as possible. If any suspicion of compartment syndrome and nerve compression exists, then the patient should have an emergency hematology consult and should be admitted to the hospital.

Soft tissue and superficial bleeds

Softtissuebleedsusuallydonotrequireaggressivetreatment.Superficialhematomasandbruises may appear anywhere on the body and if they do not threaten function and mobility, they do not need to be treated.


Muscle

Muscle / soft tissue bleeding

10

Abdominal painTreat immediately with a major dose of factor* for:- flank pain- melena- vomiting blood- several days/weeks of factor replacement will be required

Hematuria- bed rest for 24 hours- force fluids- consult the hemophilia treatment center or the patient's hematologist

Iliopsoas bleeding- flexed hip- pain on extension- major dose of factor*


- report any symptoms - follow-up with the hemophilia treatment center or the patient's hematologist

- force fluids for hematuria- rest- no weight lifting- no antifibrionlytics

Nausea and vomiting may indicateintracranial hemorrhage as well asgastrointestinal problems.


GI/

GU

Gastrointestinal / urinary tract bleeding

Gastrointestinal Bleeding- esophageal- stomach- intestines- melena- bright red blood per rectum- nausea, relflux- post colonoscopy/polypectomy

11*Dose chart inside front cover

GI/G

U

Initial presentation

Acuteabdominalpaininapatientwithhemophiliamayhavemanyorigins,suchasGItracthematomas (both spontaneous or trauma induced), pseudotumors, iliopsoas or retroperitoneal bleeding.Bleedingmayalsooccurwithhemorrhoidsorthepassageof kidneystones.Notifythehemophilia treatment center or the patient's hematologist.

Patientswhopresenttotheemergencydepartmentwithabdominalorflankpain,melenaorhematemesisshouldbetriagedforimmediateexaminationandgivenfactorreplacementtherapyatthemajordosage.*Afterfactortherapy,thendiagnosticx-rays,scansandendoscopyprocedurescan be carried out.

Abdominaltraumaandbenigneventssuchasforcefulcoughingorvomitingcanprecipitateanabdominal bleed. Blood loss can be significant before outward signs and symptoms appear. Infants canhavebleedswithgastroenteritis,intussusceptionorMeckel'sDiverticulum.

Ahistoryof liftingheavyobjects,weightlifting,fallingonbicyclehandlebarsorstretchingthegroin can precipitate abdominal wall, iliopsoas (see pages 8 and 9), or retroperitoneal bleeding.

Symptoms

Symptomsof abdominalmusclebleeding(rectus,pectorals,latissimus,obliques)areapalpablemass,rigidity,andpain.Concurrentbleedingintheabdominalcavitymaybepresentandgounnoticedfordayswithasteadilydroppinghemoglobin.Ruptureof theliver,spleen,orpancreasshould be considered when the hemoglobin falls dramatically following trauma.

Fornauseaandvomitingwithoutanobviouscause,considerthatthesemaybesymptomsof intracranialbleeding.Inquireaboutheadinjury,mentalstatuschanges,andotherneurologicsignsand symptoms, and consider CT scan of the head.

Genitourinary bleeding

Hematuriaisoftenfrighteningtothepatientbutnotaseriousevent.Instructthepatienttoremainatbedrestandforcefluidsthenext24hours.Protractedhematuriamayrequirearoutinedoseof factorcoverage.* Anti-fibrinolytics are contraindicated with hematuria. Contact the hematologist.

Scrotal bleeding may occur after trauma, especially in toddlers. Infuse with a routine factor dose* andhavethefamilycontactthepatient'shematologistforfollow-upcare.

Treatment

WillrequireextensivetreatmentforGastrointestinalbleeding.Contactthepatient'sHemophiliaTreatment Center.

Gastrointestinal / urinary tract bleeding

12

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

- Avoid negative pressure (pacifier, straws). Sippy cup recommended

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

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


Patients should follow-up with their hemophilia treatment center or hematologist the next day.

Instruct the patient on:Controlling Epistaxis, Anti-fibrinolytics, Diet Modification tables (pages 14 - 15) as needed.

Nose bleeds may respond to other measures. Refer to "Controlling Epistaxis" table on page 14.


Muco

us

Mem

br.

Mucous membrane bleeding

Dental Extraction and other oral procedures Obtain verification of oral procedure from dentist/ENT

- requires major factor dose with follow-up factor replacement- Antifibrinolytic therapy- Avoid NSAID's

Retropharyngeal/tongue swelling - consider life-threatening- major dose of factor necessary. - requires days/weeks of factor replacement

13


Mucousmembranebleedingmayrequiremedicalcareintheemergencydepartment.Patientsshouldreceivefactorwho: •areexperiencingprofuseand/orprolongedbleeding •havesustainedaknowninjurytothemouth,tongue,ornose •havesevereswellinginthemouthorthroatarea •areexperiencingrespiratorydistress •havedifficultyswallowing

Thepatientmaynotknowthereasonforthesymptomorbleeding.Itmayhavebeencausedbytrauma,infection,orthebleedmaybespontaneous.If airwayblockageissuspected,thepatientshouldimmediatelyreceiveamajordoseof factor.*Afterthefactorlevelhasbeenraised,furtherinterventionsincludinginvasiveproceduresmaybedone.

Do not make a person with hemophilia wait for factor replacement. The longer he waits,themorebleedingtakesplace.If thebleedisinaclosedspace,theaccumulationof blood will cause surrounding tissue damage, airway obstruction, and enhance pain.

Epistaxis

Apersonwithepistaxiswhoisunabletocontrolthebleedhimself mayneedaroutinedoseof factor*andanti-fibrinolytictreatment(Amicar).Besurethepersonknowshowtocontrolandstopthebleeding.Manytopicalagentsareavailabletocontrolbleeding.

Oral Cavity

Bleeding in the mouth can be hard to control. The patient will probably need factor. A single infusion of a routine dose of factor*maytemporarilystopthebleeding,butclotsbreakdownnormally on days 3-5 and bleeding may start again at that time. An anti-fibrinolytic may be indicatedtomaintainhemostasis.Anti-fibrinolyticsmaybeavailablethroughthepatient'shomehealthcompanyfornextdaydelivery.Amodifieddietshouldbestartedatthesametime as factor therapy.

Bleedingmayoccurwitheruptingorexfoliatingteeth.Itismorecommonwithexfoliatingteeth,especiallyatooththatisveryloose.Adentalconsultmaybeneededtoextractthetoothsinceitwillcontinuetolaceratethetoothsocketaslongasitisinplace.Aroutinedoseof factor*shouldbegivenpriortoextraction.Afrenulumortonguelacerationwillrequirearoutine dose of factor.*Consult Hemophilia Treatment Center - there are many tips in managing oral-mucosal bleeding in young children.

Retropharyngeal

Afterthemajordoseof factor*isgiven,furtherobservation,x-raysandadmissionmayberequired.


Muco

us

Mem

br.

14

Controlling EpistaxisInstruct the patient:

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


2. Tilt his head forward so any blood will come out the nares and not down the back of the throat.

3. Apply continuous firm pressure to the entire 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.

5. If the bleeding continues, reapply pressure for another five minutes.

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/drops 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 nares to keep the membranes soft and moist, and prevent the formation of hard crusts which might crack and restart bleeding.

9. An ENT consult may be required for possible cauterization of a vessel.


Muco

us

Mem

br.

15

Diet ModificationsDirections 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.


Muco

us

Mem

br.


Anti-Fibrinolytics

Anti-fibrinolytics may also be indicated in nasal or oral bleeding. Amicar and Tranexamic Acid 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

Tranexamic acid Recommended dosage: Individual dosing based upon bleed - consult package insert

These medications must be given around the clock to keep blood levels constant.

These medications may be available through the family's home health company, the hemophilia treatment center, or the family may have a supply at home. They 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.

16

Factor administration

Factor box top

Factor Reconstitution for intravenous use

Examples: Antihemophilic factor (AHF) or factor VIII (8)* monoclonal or recombinant

Coagulation factor IX (9)* monoclonal or recombinant

Mixing instructions, rate of administration and use of transfer device are found on the drug 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 records.


Fact

or

Some patients are instructed to bring unmixed factor concentrate with them to the ED to minimize treatment delay and cost. Occasionally, patients will bring prepared factor concentrate after unsuccessful home venipuncture attempts. Please assist with venipuncture and allow the patient or family to infuse the prepared factor concentrate if less than 3 hours have elapsed since reconstitution.

Read package insert before attempting to reconstitute factor. Some patient's are trained in mixing and administering their factor and some infuse themselves.

17

Factor administration

Dosage

Eachbottleof factorconcentrateislabeledwiththeactivityexpressedasInternationalUnits(IU,example:287IU).Thedosagetobeadministeredisbasedonthepatient'sbodyweightinkilograms(kg)*.

Factor VIII (8) is calculated using the formula:1IU/kg=2%riseinFactorVIII(8)activity50IU/kg=100%correction(example)

Factor IX (9) is calculated using the formula:1IU/kg=1%riseinFactorIX(9)activity80IU/kg=80%correction(example)

The ENTIRE contentsof allthevialsreconstitutedforaninfusionshouldbeused,evenif itexceedsthecalculateddosage.Alargerdosewillonlyprolongtheperiodof normalcoagulation.Duetoitsexpense,factor concentrate should never be discarded!

The half-life of factor VIII (8) is 8-12 hours; the half-life of factor IX (9) is 18-24 hours.Noteverypatienthastheexpectedincreaseinfactorlevelandhalf livesverybasedontheindividual,age,factorproductandotherfactors.

Factor types

RecombinantReferstogeneticallyengineeredFactorVIII(8)orIX(9)concentrateswhicharenotderivedfromplasma.

High purity / Monoclonal - plasma productFactorVIII(8)orIX(9)concentratesof highspecificactivityandpurityof thespecificclottingfactorachievedthroughtheuseof anaffinitycolumnmatrix.

Intermediate purity - plasma productReferstoFactorVIII(8)concentratewithlowerspecificactivity.SomecontainvonWillebrandfactorandareusedtotreatvonWillebrandDisease.

FactorIX(9)prothrombincomplexconcentrates(PCCs)-plasmaproductrarelyused.Alsocontainssomeotherclottingfactorsandsmallamountsof activatedcoagulationfactors. Used to treat Hemophilia B and some patients with Factor VIII or IX inhibitors.

DDAVP (Desmopressin Acetate) - Some mild Hemophilia patients may be treated with DDAVP. Consult their Hemophilia Treatment Center.

Some patients may be on extended half-life (Factor VIII or Factor IX) factor-CALL the Hemophilia Treatment Center for dosing guidelines.

IMPORTANT!

Theseareexamples.Pleaserefer to the inside front cover for specific doseranges set by your local hemophilia treatment center.


Factor

18

Venous access

Do not use the affected or injured limb for venous access.

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

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

See factor administration (pages 16 - 17) for further information.

Dorsum of feet(infants/children)

Scalp vein (infants only)

Hickman, Broviac orGroshong catheter

Dorsum of hand

Antecubital fossa(caution in infants)

Percutaneous intravenous central catheter (P.I.C.C.) line

Port (use Huber needle only)

Ven

ous

Acc

ess

19

Venous access

Intravenousaccessforapersonwithhemophiliaisbasicallythesameasforanypatient.Theuseof 23gaugeor25gauge"winged"needlesispreferable,especiallyforchildren.

Sites for access

Sites to consider for a peripheral IV include:

•dorsumof thehands

•antecubitalfossa(cautionininfantsduetotheriskof compartmentsyndrome)

•dorsumof feet(infantsandtoddlers)

•scalpveins(infantsonly)

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

The patient's IV should not bestartedintheaffectedlimb.Theinjuredareashouldbeminimally manipulated, if at all.

Venous access device

Somevenousaccessdevicescurrentlyusedinhemophiliacareinclude:

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

Externalcentralcatheter-Hickman,Broviac,orGroshong;accessperyourinstitution's

procedure

P.I.C.C. line - access per your institution's procedure

Heparin flush

Itisrecommendedtodoafinalflushwithheparinforanyvenousaccessdevice(exceptforGroshong'swhichareonlyflushedwithnormalsaline).Checkwiththeparentorthepatient'sinstitutionfortheamountof heparinflushtouse.If theseoptionsarenotavailable,useyourinstitution's procedure for the amount and concentration of heparin. This small amount of heparinwillnotharmthepatient(remember-you'vejustgivenhimfactor).Theaccessdeviceneeds to stay patent; this is accomplished with the heparin.

The patient on "prophylaxis"

Somepatientswithhemophiliareceive"prophylactic"dosesof factorreplacementonaregularschedulethatisdeterminedbythehalf-lifeof thefactortheyarereceiving.Thiscouldbeseveraltimesaweek,onceaweekoreveneveryotherweek.Thepatientshouldbeaskedabouttheirschedule.Somepatientsonprophylaxismayhaveavenousaccessdevicefortheirinfusions.So,alwayschecktoseeif thepatienthasavenousaccessdevicebeforestartingaperipheral infusion.

Ven

ous

Access

20


Patient should follow-up with the hemophilia treatment center or his hematologist the next day.

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

Sutures: Remind the patient he will need factor for suture removal.

Head injury (see page 5)

Fracture

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

First give a major dose of factor* . . .

. . . then apply the cast.

First give a major dose of factor* . . .

. . . then performa CT scan.


Labs

X-R

ay

Invasive procedures, labs, x-rays

21

Ingeneral,patientswithhemophiliawhoareexperiencinganacutebleedingepisodeneedfactoronly. Other procedures should not be done unless there is another clinical indication for the study. In any situation, the infusion of factor should never be delayed if any bleeding is suspected. Delaying the infusion simply increases bleeding that will result in greater morbidity.

Laboratory studiesIf theonlycomplaintisanacutejointormusclebleed,nolaboratorystudiesarenecessary.If GI,abdominal,largemuscle,ororalcavitybleedingissuspectedandhaspotentiallybeenextensive,aCBCmaybeindicatedtodetermineif theindividualisanemic.Factorlevelsandinhibitorlevelsarenotnecessary for treatment in an acute emergency setting. Factor should not be delayed for laboratory studies to be drawn or completed.

X-rays and other radiological studiesGive factor first,thendecideif aradiologicalstudyisindicated.Rememberthataswollenjointorextremityisusuallytheresultof internalbleeding,notafracture.X-raysof thejointcanbeusedtodocumentajointbleed,butaregenerallynotusefulindetectingearlyonsetbleeds(andthatiswhentreatmentisoptimal).Thepatientwillbeawareof jointbleedingbeforeradiologicalchangesareevident.

A CT of the head (see page 5) is necessary when dealing with a potential head bleed. It can document locationandextentof bleedingandhelpdirectfurthertreatment.Firstgiveamajorfactordose.*

Fractures

Giveamajordoseof factor*replacement,thenx-rayandsetthebone.

Lacerations and suturesSutures and staples should be used as on any other patient. If the laceration is significant enough to requiresutures,thepatientshouldfirstreceivearoutinedoseof factor* then the procedure. Contact thepatient'shematologistforfollow-upfactorinfusioninstructions.Forremovalof sutures,aroutinedose of factor* is usually needed.

Invasive proceduresInvasiveproceduresshouldbeperformedasclinicallyindicated,i.e.,lumbarpuncturewithsymptomsof meningitis.However,amajordoseof factor*mustbegivenbefore the procedure begins.

Arterial sticks and venipuncturesDonotdoarterialsticksunlessnootheroptionisavailable.If anarterialstickmustbedone,thenamajorfactordose* must begivenfirst.

Venipuncture(seepage19)maybedoneatanylocation;handsaregenerallyexcellentandnofactortreatmentisnecessary.Avoid"digging"fordeepveins.Applypressureforseveralminutesafterthepuncture.


Labs

X-R

ay

Invasive procedures, labs, x-rays

22

Other medications

Pressure and ice pack after injections

Routine medications

Patientswithhemophiliacanreceiveroutinemedications(e.g.painmedications,antibiotics,etc.)thatdonotinterferewithclottingfunction.Avoidnon-steroidalanti-inflammatories(NSAIDS),ASAandanyproductwithaspirin-relatedingredients(e.g.Pepto-Bismol,Excedrin,Percodan).

Medications for fever or pain

Acetaminophencanbegivenforfeverorpain.Narcotics/opioidsareoftenrecommendedtocontrolthepainexperiencedbyapatientwithhemophilia.Avoid giving intramuscular injections of antibiotics, pain medications, or immunizations because of the possibility of causing a muscle bleed.

Routes of administration

MedicationswhichcanbegivenPO,SC,orIVarepreferred.Routineimmunizationsandtetanustoxoidmay be given subcutaneously.If therabiesvaccinationseriesisneeded,anexperiencedhematologist(preferablythepatient's)shouldbecontactedtoarrangefactorinfusionspriortoandaftertheinjectionsinordertopreventinternalbleeding.

Foranyneedlestick,pressureandanicepackafterwardwillminimizesofttissueormusclebleeding.

Caution

Somepatientswithhemophiliamayhaveliverdiseasefromhepatitis.Usecautionwhenprescribingdrugsthatmaycauselivertoxicity.Otherpatientsmaybeonothertherapiesforhemophilia-related complications such as HIV or hepatitis. Be aware of potential serious drug interactions.

Med

s/Tr

aum

a

23

Trauma / emergencies

AccessemergencyservicesandcontactyourHemophiliaTreatmentCenter.Manydifferentemergencies/traumasmayoccurtopersonswithhemophilia,justastoothers.The more common are:

• Animalbites • Motorvehicleaccidents• Burns • Myocardialinfarctions• Falls • Ocularinjuries• Fractures(seepage21) • Puncturewounds• Gunshotwounds • Dislocatedjoints

Treatment

Amajordoseof factor*shouldbeinfusedassoonaspossible(beforeanytest,x-rays,debriding, sutures, etc.).


Med

s / Traum

a

Trauma / emergencies

24

Inhibitors

High Responding InhibitorsProducts used to treat bleeding:

Activated PCCe.g. FEIBA, Autoplex

recombinant F Vlla (7) (activated)e.g. NovoSeven

Low Responding Inhibitors

Factor product and dose is variable. Dose may be 2-3x routine range.*

Contact the hemophilia treatment center or the patient's hematologist due to the complexity of managing inhibitors.


Inh

ibit

ors

For patients diagnosed with Hemophilia A (Factor VIII deficiency) with or without an inhibitorandtakingHEMLIBRA®(emicizumab-kxwh):

Call hematologist for all episodes requiring treatment

***If it’s been determined the non-inhibitor hemophilia ApatientrequiresfactorVIIItostopinternalorexternalbleeding, the patient CANreceiveanappropriatedoseof factorVIII,asdeterminedbythebleedingepisode,withoutanycomplicationsoccurringfromtheemicizumab.***

Emicizumab(HEMLIBRA®)isabispecificfactorIXa-andfactorX-directedantibodyindicatedforroutineprophylaxistopreventorreducethefrequencyof bleedingepisodesinadultandpediatricpatientsagesnewbornandolderwithhemophilia A (congenital factor VIII deficiency) with or without factor VIII inhibitors.

EmicizumabshouldNOT be used to treat acute bleeding. Due to the potential complications of treating bleeds while on emicizumab,pleaseconsultwithhematologypriortorecommendingtreatment.

Generalapproachtotrauma/bleeding:itisrecommendedthatpatientswhoareonemicizumabcontact the hemophilia treatment center/provider if they exhibit clinical manifestations of a bleed.Emicizumabislikelytotransformthebleedingphenotypeof patientstoamilderphenotype.Givenimprovedbaselinehemostasisinpatientsonemicizumabprophylaxis,thecurrentparadigmof treatingatthefirstsignsandsymptomsof bleedinginsomecasesshouldchange.Significant and serious or life-threatening bleeding should continue to be treated promptly with factor VIII, emicizumab does not interfere with factor VIII.However,thereshouldbeadditionalevaluationof muscleandjointcomplaintspriortotreatmentwithanadditionalhemostaticagent.Forequivocalsignsorsymptomsof minorbleeds,thepatientshouldcontacttheirproviderbeforeinitiatingtreatment.

***If it’s been determined the non-inhibitor hemophilia ApatientrequiresfactorVIIItostopinternalorexternalbleeding, the patient CANreceiveanappropriatedoseof factorVIII,asdeterminedbythebleedingepisode,withoutanycomplicationsoccurringfromtheemicizumab.***

Important Laboratory Considerations: these laboratory tests should NOTbeobtainedfromthepatientonemicizumabasthe results will not be correct.

Emicizumabinterfereswith-

*activatedclottingtime(ACT), *one-stageaPTT-basedsingle-factorassays,

*activatedpartialthromboplastintime(aPTT), *aPTT-basedActivatedProteinCResistance(APC-R),

* coagulation laboratory tests based on aPTT * Bethesda assays (clotting-based) for factor VIII (examplefactorVIIIactivity) (FVIII)inhibitortiters

Intrinsic pathway clotting-based laboratory tests should not be used.

REPORTEDADVERSEEVENTSWITHHEMLIBRA–INHIBITOR PATIENTS TAKING FEIBA®

Thromboticmicroangiopathy(TMA)andthromboticeventshavebeenobservedwhilereceivingHEMLIBRA®prophylaxiswhenonaverageacumulativeamountof >100U/kg/24hoursof aPCC(FEIBA®) for 24 hours or more were

administered.

Patients taking HEMLIBRA® (emicizumab-kxwh)

25

Definition

AninhibitorisanantibodythatsomeindividualswithhemophiliadevelopagainstfactorVIII(8)orIX(9).Theseantibodiesneutralizethefactorprocoagulantactivity,thuscounteractingthe desired effect of an infusion of factor concentrate.

How inhibitors are measured

LabsintheU.S.expressthepresenceof aninhibitorintermsof Bethesdaunits(BU).OneBethesdaunitistheamountof antibodythatdestroyshalf of thefactorVIIIinanequalmixtureof normalandpatientplasmaintwohours.

Low responding inhibitor

Measures less then 5 BU.

High responding inhibitor

Measures 5 or greater BU. An infusion of factor concentrate further stimulates the inhibitor antibodies, causing a rise in BUs.

When to suspect an inhibitor

Suspectaninhibitorif bleedingdoesn'tstopafterseveralinfusionsof factorconcentrates.

Callthepatient'shematologistif theinhibitorisknownorsuspectedbeforeattemptingtreatment.Besuretoalsoaskthepatientandfamilyif theyhavebeentoldthepatienthasaninhibitor.

Treatment of inhibitors

Inhibitormanagementisdifficultfortheexperiencedhematologist.Contactthepatient'shematologist or hemophilia treatment center when these patients present in the emergency departmentfortreatment.Bleedinginaninhibitorpatientcanquicklyleadtoseriouslife-orlimb-threateningcomplicationswithoutexpertmanagement.

Inhibitors

Inh

ibito

rs

26

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 groupwishes to thank the following for their contributions:

Nurses from Region VI Hemophilia Treatment Centers:

Cook Children's Medical Center, Ft. Worth, TX

Gulf States Hemophilia Diagnostic and Treatment Center, Houston, TX

Hemophilia Center of Arkansas, Little Rock, AR

Louisiana Comprehensive Hemophilia Care Center, New Orleans, LA

North Texas Comprehensive Hemophilia Center,

Adult Program, Dallas, TX

North Texas Comprehensive Hemophilia Center,

Pediatric Program, Dallas, TX

The Oklahoma Center for Bleeding Disorders, Oklahoma City, OKSouth Texas Comprehensive Hemophilia Center, San Antonio, TX

Reviewers:Howard Britton, M.D.

Medical Director, South Texas Comprehensive

Hemophilia Center, San Antonio, TX

Cindy Leissinger, M.D.Medical Director, Louisiana Comprehensive

Hemophilia Care Center, New Orleans, LA

Arlo Weltge, M.D.Associate Professor of Surgery/ER Medicine,

The University of Texas Medical School, Houston, TX

Rita Gonzalesconsumer parent and past President, Lone StarHemophilia Chapter, Houston, TX

Production Team - Project Manager: Art Gardner Illustrations: Mark Gilmore Colorizing: Heather Swaim Design andLayout: Art Gardner, Mark Gilmore Additional illustrations: Bob Aul Printer: Clarence Printing 716-983-0166

Reviewers for 2016/2017:

Susan C. Zappa, RN-BC, CPNEditor

Linda Belling, RN-BC, MS, CRRNClinical Program CoordinatorHemophilia Center of Western New YorkBuffalo, NY

Renee DeRider, BSN, RN-BCVice President, Clinical ProgramsMary M. Gooley Hemophilia CenterRochester, NY

John Drake, MSN, RNRegional CoordinatorGreat Plains Regional Hemophilia Network

Sarah M. Hawk, BS, PA-COklahoma Center for Bleeding DisordersOklahoma City, OK

Laurel McKernan, MSN, RNHemophilia Clinical Specialist/Prg CrdComprehensive Hemophilia & Thrombosis CenterDartmouth-Hitchcock Medical CenterLebanon, NH

Caitlin Montcrieff, MSN, CPNP, CPHONAssociate Director, Pediatric Nurse PractitionerRhode Island Hemostasis & Thrombosis CenterProvidence, RI

Ellen White, RN, MSNComprehensive Hemophilia CenterNewark Beth Israel Medical Center and Children’s Hospital of New JerseyNewark, NJ

Yasmin Zambrano Ortiz, BSN, RNNurse CoordinatorGulf States Hemophilia & Thrombophilia CenterHouston, TX

Documents

Emergency Care for Patients with Hemophilia · Hemophilia basics Definition Hemophilia is a genetic disorder characterized by a deficiency or absence of one of the clotting proteins