Emergency Care for Patients With Hemophilia FINAL

8/13/2019 Emergency Care for Patients With Hemophilia FINAL

1/28

Slings, splints, immobilizers

p.r.n. for joint bleeds

Head injury(pg. 5)

Always treat immediately with a

major factor dose

Mucous membrane bleeds (pg. 12)

Treat with a routine factor dose

and anti-fibrinolytics

Joint bleeds(pg. 6)

Treat an early onset bleed

with a routine factor dose.

Treat an advanced joint

bleed with a major

factor dose.

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

Hemophilia B or factor IX (9) deficiency

Avoid intramuscular

injections due to the

possibility of causing a

muscle 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

Written by the Nursing Group o Hemophilia Region VI

Editors: Karen Wulff, R.N.;Susan Zappa R.N., C.P.N., C.P.O.N.;Mack Womack R.N.

A n i n s t r u c t i o n a l m a n u a l o r M e d i c a l P r o e s s i o n a l s

Emergency Care forPatients with Hemophilia

www.HemophiliaEmergencyCare.com


2/28

Copyright 1999 Te Nursing Group o Hemophilia Region VI.Updated September, 2011

All rights reserved. Permission granted to photocopy or educational purposes only.All brand names and product names used in this publication are trade names, service marks, trademarks, or registeredtrademarks o their respecitve owners. Tese terms are used in this publication only in an editorial ashion and shouldnot be regarded as affecting their validity.

Factor Replacement Guidelines ...................................................1

Introduction ...................................................................................2

Hemophilia basics .........................................................................3

Head injury.............................................................................5

Joint bleeding .................................................................................6 Signs & symptoms, Aspiration, Dislocated joints

Muscle /sof tissue bleeding .........................................................8 Compartment syndrome, Iliopsoas

Gastrointestinal /urinary tract bleeding ....................................10 Abdominal muscle wall, Hematuria

Mucous membrane bleeding ........................................................12 Anti-fibrinolytics, Epistaxis, Retropharyngeal bleeds

Factor administration ...................................................................16 Mixing, Dosage ormula, Factor types

Venous access .................................................................................18 Site or administration, Prophylaxis, Venous access devices

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

Other medications .........................................................................22 Pain medications, Immunizations

rauma / emergencies ...................................................................23 Bites, Burns, Falls, Fractures, Gunshot wounds, MVA, MI, Ocular

injuries, Puncture wounds

Inhibitors ........................................................................................24

Definition, reatment

Acknowledgements.................................................................26

Emergency Care for Patients with Hemophilia Table of Contents


3/28


Hemophilia B or Factor IX (9) deciency

Routine doseExamples: sutures, early onset jointbleeds, muscle and mouth bleeds.

Hemophilia A or Factor VIII (8) deciency

Major doseExamples: head, GI, prior to invasive proce-dure (ex. LP), and advanced joint bleeds.

Routine doseExamples: sutures, early onset jointbleeds, muscle and mouth bleeds.

Major doseExamples: head, GI, prior to invasive proce-dure (ex. LP), and advanced joint bleeds.

!Imp

ortant

!

NOTE:Please contact the hematologist beore any inusion. For patients with Factor 8 or 9 inhibitors (seepage 24) it is imperative that you contact the patients hemophilia physicianbeore any inusion. Have all patients contact the Hemophilia Center the next day to reporttheir status.

Patient specific treatment guidelines prepared by the patients treatment center and contact

inormation or that center can be ound on this flash drive in the AHNready PersonalHealth Report. **

** Te recommendations in the Personal Health Report are provided by the patients hemophiliatreatment center. Te American Trombosis and Hemostasis Network (AHN) is not responsible forthe patient specific clinical information or treatment guidelines included in the Personal Health Report.

Factor Replacement GuidelinesEmergency Care for Patients with Hemophilia

Each hemophilia treatment center throughout the United States sets its own dosageguidelines. Guidelines rom treatment centers throughout the United States and Canadacan be ound at www.HemophiliaEmergencyCare.com.


4/28


Introduction

To The Attending Medical Staff:

Tis manual is a guide or medical personnel who maybe less amiliar with hemophilia treatment. The contentconsists of guidelines, recommendations andsuggestions only. Te attending physician has the finalresponsibility or appropriate diagnosis and treatment.

Purpose

Tis manual contributes to hemophilia care by enhancing the emergency department staff s understanding ohemophilia and its treatment. Te goals o this manual are to:

- promote understanding o the complexities o hemophilia treatment

- provide a reerence or the emergency center staff

- promote a consultative dialogue with the emergency department, hemophilia treatmentcenter, and patient/amily

Use

Tis manual provides a standardized ormat or evaluation and treatment o hemophilia emergencies. Tecontent is segmented by systems and complications o hemophilia. urn to an area o interest. Te illustrationsprovide inormation points or quick review. Te text gives urther detail o bleeding presentations, their possiblecomplications, and treatment.

It is suggested that the patients hemophilia treatment center or hematologist be consulted or anything other thanroutine bleeding episodes.

Emergency Care for Patients with Hemophilia


5/28


Hemophilia is a genetic disorder characterized by a deficiency or absence o one o the clotting proteins in plasma.Te result is delayed clotting. Deficiencies o actor VIII (8) [Hemophilia A or Classic Hemophilia] and actor IX (9)[Hemophilia B or Christmas Disease] are the most common and reerred to as hemophilia. Hemophilia mostly affectsmales due to the X-linked inheritance pattern.

Effects of hemophilia

Hemophilia prevents the ormation o a firm, fibrin clot and results in a sof, unstable clot. Persons withhemophilia do not bleed aster than others; rather the bleeding is continuous. Significant blood loss can occur itreatment is delayed.

Incidence

Te incidence worldwide is estimated to occur in 1:7,500 live male births; all races and ethnic groups are affected.

Factor VIII (8) deficiency is our times more common than actor IX (9) deficiency but the clinical presentationsand inheritance patterns are the same.

Severity

Te amount o bleeding expected in an individual with hemophilia depends upon the severity o the deficiency.Normal plasma levels o actor VIII (8) and IX (9) range rom 50-150%.

Tose with less than 1% actor VIII (8) and IX (9) are considered to have severe hemophilia. Frequent bleedingepisodes are common, particularly into joints. Bleeding can occur spontaneously or rom trauma.

Persons with actor levels o 1-5% are considered to have moderate hemophilia. Tese persons may experiencebleeding afer minor trauma but should not bleed spontaneously. Afer repeated bleeding into the same joint,persons with moderate hemophilia may experience spontaneous bleeding in that joint.

Persons with more than 5% actor activity are considered to have mild hemophilia and bleed only afersignificant trauma or with surgery. Some carrier girls and women (called symptomatic carriers) can have lowerthan normal plasma levels o actor VIII (8) or IX (9) and thus may exhibit symptoms o mild hemophilia.

BasicsEmergency Care for Patients with Hemophilia


6/28


Serious bleeding sites

The six major sites of serious bleeding which threaten life, limb, or function are:- intracranial- spinal cord- throat- intra-abdominal- limb compartments- ocular

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

reatment or bleeding involves replacing the deficient actor as the rst course o action. Tis requiresintravenous inusion o commercial actor concentrates. Specific doses, additional drugs and medical interventionsdepend upon the site and severity o bleeding. Once actor replacement therapy has been inused, diagnosticprocedures and examinations can begin.

Family

Parents and persons with hemophilia are knowledgeable about the management o the disorder and their inputshould be sought and heeded. Most hemophilia amilies are medically sophisticated and should not be dismissed

as novices.

Interview the amily about whether actor concentrate has been administered prior to arriving at the ER; i so,determine when and at what dose. Additional actor may be required, depending upon the time lag and severityo the bleed. Establish who the treating hematologist or hemophilia treatment center is and contact them or otherthan routine bleeding.

BasicsEmergency Care for Patients with Hemophilia


7/28


Head injur

Intracranial hemorrhage (ICH) is the leading cause o death rom bleeding in all age groups. Without earlyrecognition and treatment, death or severe neurologic impairment can occur. ICH may be spontaneous, withouthistory o injury. Early neurologic symptoms may not be evident due to the slow, oozing nature o hemophiliableeding.

Treatment

All significant head trauma, with or without hematoma, must be treated promptly with the major dose o actorreplacement* beore any diagnostic tests.

Diagnostic imaging

Obtain an emergency C scan to rule out ICH afer the major actor dose* has been given. Notiy the patientshematologist or hemophilia treatment center o the ED admission and the diagnostic findings.

Possible admission

Te patient should be admitted to the hospital or observation i he suffered a severe blow to the head or i heexhibits any neurologic symptoms such as headache with increased severity, irritability, vomiting, seizures,vision problems, ocal neurologic deficits, stiff neck, or changes in level o consciousness. Patients with apast history of ICH are at increased risk of repeated head bleeds.

Instructions

I the patient is discharged home, instruct the amily to monitor the patient or signs and symptoms oneurologic deterioration and report any abnormalities to the hematologist. Consult the hematologist or ollow-up actor replacement doses i the patient is discharged home rom the emergency department.

* See page 1 for factor dosage guidelines.


Call the hemophilia treatment center or the patients

hematologist for follow-up factor doses.*

Report any signs or symptoms to the hemophilia

treatment center or to the patients hematologist.

Head injury instructions for a two week period

(instead of the usual instructions for 24- 48 hour

period.)

Discharge Instructions

Treat all head injuries, with or without swelling, by infusing

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


8/28


Advanced joint bleed- heat

- pain

- swelling

- decreased range

of motion

Treatment: a major factor dose.*

Early onset joint bleed

- tingling

- pain

- limited range

of motion

Treatment: a routine factor dose.*

Remember, toes and

fingers are joints too.

Crutches for weightbearing joints and

crutch instructions.

Joint bleeding

The hallmark o hemophilia is joint and muscle bleeding. Spontaneous joint and muscle bleeding can occur without adefinite history o trauma. Te patient may not be able to identiy a specific event that resulted in bleeding.

While persons with hemophilia may bleed into any joint space, the joints which most requently bleed are the elbows,knees, and ankles. Other possible bleeding joint sites include the shoulders and hips. As repeated bleeding occurs, thesynovial tissue thickens and develops even more riable blood vessels. A vicious cycle o bleeding and rebleeding mayset in and the affected joint is reerred to as a target joint. Eventually, repeated bleeding into joints leads to a ormo chronic arthritis with destruction o cartilage and the eventual destruction o bone resulting in decreased jointmobility and unction.



For the next 24 hours:

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

- sling or splinting if support is needed (i.e. Aircast for ankles)

Follow-up with the hemophilia treatment center or with the patients hematologist


Signs and symptoms


9/28


Treatment

Some patients may present or treatment with noother outward signs o bleeding than decreased rangeo motion and a complaint o pain or tingling. Tis isindicative o an early onset joint bleed and is theoptimal time to treat. Te patient should be inused asquickly as possible with a routine dose o actor* in orderto minimize pain and joint destruction.

Extreme pain, swelling, heat, and immobility are signsand symptoms o an advanced joint bleed which occursonly afer blood has filled the joint space. Symptomssuggestive o an advanced joint bleed require a majoractor dose.*

Inuse beore any diagnostic procedures such as x-ray.I a joint bleed is treated early beore obvious outwardsigns occur, then the need or expensive ollow-upinusions may be lessened or avoided altogether. Beforedislocated joints are reduced, infuse with a major

Joint bleeding

factor dose.*

Joint aspiration: Caution!

Joint bleeds in hemophilia are generally not treated by aspirating the joint. Tis procedure can make mattersworse by creating another site rom which the patient may bleed. I joint aspiration is deemed necessary, such as insuspected joint sepsis, then the joint should be aspirated by an orthopedic surgeon associated with a hemophiliatreatment center. Te patients hematologist must be involved in order to arrange or actor coverage beore andafer the procedure.

Discharge and follow-up care

Upon discharge, the hemophilia patient with a joint bleed should be instructed to keep the affected joint at rest,elevate the affected limb, and apply ice packs. Additional support to the affected joint may be applied by wrappingwith an ace bandage. For ankle bleeds, an Aircast may be a useul splinting device. Crutches are useul to helpindividuals when they have lower extremity joint bleeds and need to be non-weight bearing. Follow-up should bemade to the local hemophilia treatment center or to the patients hematologist as soon as possible.

Swelling and a major

decrease in range of

motion are signs of an

advanced joint bleed.

A tingling sensation and

a limited or small decrease

in range of motion are

signs of an early onset

joint bleed.

Advanced or Early onset joint bleed?


Outward signs o joint bleeding include restriction o movement, swelling, heat, and erythema on and around thejoint. Te patient may report symptoms o a bubbling or tingling sensation with no physical signs. Later symptomsinclude a eeling o ullness within the joint and moderate to severe pain as the bleed worsens.



10/28


Muscle / soft tissue bleeding


Deltoid / forearm bleed

- routine factor dose*

- major factor dose*if a

compartment syndrome

is suspected

Thigh/calf bleed

- pain

- with/without swelling

- impaired mobility

- routine factor dose*

- major factor dose*if a

compartment syndrome

is suspected

Neck swelling: EMERGENCY- potential airway

compromise

- major dose of factor*

Soft tissue bleeds

and bruising

- no functional

impairment

- tenderness, but

no severe pain

- no factor needed

Buttock bleeds

- pain

- with/without swelling

- routine dose of factor*

- major dose of factor*if

the leg on the affected

side exhibits tingling or

swellingIliopsoas bleeds

- flexed hip

- pain/inability to extendthe leg on the affected

side

- major factor dose*


- rest

- ice

- non-weight bearing

- Follow-up with the hemophilia treatment center or with the patients hematologist



11/28


Muscle / soft tissue bleeding



Signs, symptoms, and sites

Muscle bleeding is common in persons with hemophilia. Any muscle group may be subject to bleeding.Muscles that exhibit warmth, pain, and swelling should be treated with a routine dose o actor.* Common

bleeding sites include the upper arm, orearm, thigh, and cal muscles. oddlers requently have buttockand groin bleeds. Abdominal wall muscles and iliopsoas muscles are also common bleeding sites. Teseabdominal muscles generally do not have observable swelling, yet they may hold a large amount o blood.Patients who complain o low abdomen or groin pain, especially with signs o nerve compression, areprobably experiencing an iliopsoas bleed. Tese patients should receive an emergency hematology consultand possible admission or observation and several major doses o actor coverage.* Te hallmark sign oiliopsoas bleeding is spontaneous flexion o the leg on the affected side with an inability to extend the legwithout pain.

Consequences of muscle bleeds

Muscle bleeds can result in serious consequences i not treated promptly. Extensive blood loss may occur inlarge muscle groups. Muscle bleeding places pressure on nerves and blood vessels and, i lef untreated, thesebleeds may result in permanent disabilities such as oot drop and wrist contracture. It is important that thepatients hematologist be consulted beore any surgical consults. Most muscle bleeds respond well to medicalmanagement and do not require asciotomy. Such an extreme measure will usually generate problems or thepatient and require a tremendous amount o post-surgical actor inusions, more so than i the patient wastreated medically.

Treatment and follow-up care

Most muscle bleeds are treated with a routine dose o actor.* Large abdominal muscle groups and iliopsoasbleeds should be treated with a major dose o actor.* Rest and ice packs are also helpul. Te patient shouldbe reerred back to his hemophilia treatment center or to his hematologist or ollow-up as soon as possible. Ifany suspicion of compartment syndrome and nerve compression exists, then the patient shouldhave an emergency hematology consult and should be admitted to the hospital.

Soft tissue and supercial bleeds

Sof tissue bleeds usually do not require aggressive treatment. Superficial hematomas and bruises may appearanywhere on the body and i they do not threaten unction and mobility, they do not need to be treated.


12/28


Gastrointestinal / urinary tract bleeding

Abdominal pain

Treat immediately with a

major dose of factor* for:

- flank pain

- melena

- vomiting blood

Hematuria

- bed rest for 24 hours

- force fluids

- consult the hemophiliatreatment center or

the patients hematologist

Iliopsoas bleeding

- flexed hip

- pain on extension

- major dose of factor*

Nausea and vomiting may indicate intracranial hemorrhage as well as gastrointestinal

problems.



- force fluids for hematuria

- rest

- no weight bearing

- report any symptoms

- follow-up with the hemophilia treatment center or with the patients hematologist



13/28


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 o kidney stones. Notiy the hemophilia treatment center or thepatients hematologist.

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

Abdominal trauma and benign events such as orceul coughing or vomiting can precipitate an abdominalbleed. Blood loss can be significant beore outward signs and symptoms appear. Inants can have bleeds withgastroenteritis, intussusception or Meckels Diverticulum.

A history o lifing heavy objects, weight lifing, alling on bicycle handlebars or stretching the groin canprecipitate abdominal wall, iliopsoas (see pages 8 and 9), or retroperitoneal bleeding.

Symptoms

Symptoms o 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 or days with asteadily dropping hemoglobin. Rupture o the liver, spleen, or pancreas should be considered when thehemoglobin alls dramatically ollowing trauma.

For nausea and vomiting without an obvious cause, consider that these may be symptoms o intracranialbleeding. Inquire about head injury, mental status changes, and other neurologic signs and symptoms, andconsider C scan o the head.

Genitourinary bleeding

Hematuria is ofen rightening to the patient but not a serious event. Instruct the patient to remain at bedrest and orce fluids the next 24 hours. Protracted hematuria may require a routine dose o actor coverage.*Anti-brinolytics are contraindicated with hematuria. Contact the hematologist.

Scrotal bleeding may occur afer trauma, especially in toddlers. Inuse with a routine actor dose* and havethe amily contact the patients hematologist or ollow-up care.

Gastrointestinal / urinary tract bleeding




14/28


Mucous membrane bleeding


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.

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

Nose bleeds may respond to other

measures.Refer to Controlling

Epistaxis table on page 14.


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.



15/28


Mucous membrane bleeding may require medical care in the emergency department. Patients should receiveactor who: - are experiencing prouse and/or prolonged bleeding - have sustained a known injury to the mouth, tongue, or nose - have severe swelling in the mouth or throat area

- are experiencing respiratory distress - have difficulty swallowing

Te patient may not know the reason or the symptom or bleeding. It may have been caused by trauma, inection,or the bleed may be spontaneous. I airway blockage is suspected, the patient should immediately receive a majordose o actor.* Afer the actor level has been raised, urther interventions including invasive procedures may bedone.

Do not make a person with hemophilia wait for factor replacement. Te longer he waits, the morebleeding takes place. I the bleed is in a closed space, the accumulation o blood will cause surrounding tissuedamage, airway obstruction, and enhance pain.

Epistaxis

A person with epistaxis who is unable to control the bleed himsel may need a routine dose o actor* andanti-fibrinolytic treatment (Amicar). Be sure the person knows how to control and stop the bleeding. (Seetable on page 14.)

Oral Cavity

Bleeding in the mouth can be hard to control. Te patient will probably need actor. A single inusion o aroutine dose o actor* may temporarily stop the bleeding, but clots break down normally on days 3-5 andbleeding may start again at that time. An anti-fibrinolytic may be indicated to maintain hemostasis. (Seetable on page 15.) Anti-fibrinolytics may be available through the patients home health company or next daydelivery. A modified diet should be started at the same time as actor therapy. (See table on page 15.)

Bleeding may occur with erupting or exoliating teeth. It is more common with exoliating teeth, especially atooth that is very loose. A dental consult may be needed to extract the tooth since it will continue to laceratethe tooth socket as long as it is in place. A routine dose o actor* should be given prior to extraction. Arenulum or tongue laceration will require a routine dose o actor.*

Retropharyngeal

Afer the major dose o actor* is given, urther observation, x-rays and admission may be required.





16/28


Instruct the patient:

1. o gently blow his nose to remove mucous and unstable clotswhich will interere with hemostasis.

2. ilt his head orward so any blood will come out the nares and not down the back o the throat.

3. Apply firm pressure to the entire side o the nose that is bleeding or 15 minutes.

4. Release the pressure to see i bleeding has stopped, blow out any sof clots.

5. I the bleeding continues, reapply pressure or another five minutes.

6. Factor replacement* at a routine dose and/or anti-fibrinolytic agents (see next page) may beneeded.

7. During active bleeding, or when the bleeding has stopped, spray or apply two drops ooxymetazoline (ex. NeoSynephrine, Dristan, or Arin) nasal spray/drops to the side that wasbleeding. Tese can be used at home PRN or epistaxis.

8. Instruct the patient to use Vaseline in the nares to keep the membranes sof and moist, andprevent the ormation o hard crusts which might crack and restart bleeding.

9. An EN consult may be required or possible cauterization o a vessel.


Controlling Epistaxis




17/28



Diet Modifications

Directions or the patient:

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

2. Milk products may cause nausea and vomiting i the patient has swallowed blood.

3. Avoid using a straw. Negative pressure rom the sucking action can dislodge the clotand aggravate the bleeding site.

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

Anti-Fibrinolytics

Anti-fibrinolytics may also be indicated in nasal or oral bleeding. Amicar andCyklokapron are both anti-fibrinolytic agents. Either may be prescribed or mucous

Anti-fibrinolytics may also be indicated in nasal or oral bleeding. Amicar andCyklokapron are both anti-fibrinolytic agents. Either may be prescribed or mucous

membrane bleeding to promote clot adhesion in conjunction with actor replacement at a routine dose.*In some cases they may be prescribed without actor replacement.

Amicar - aminocaproic acid Recommended dosage: child: oral dose 50-100 mg/kg (not to exceed 4 Gms) every 6 hours or 3 - 10 days adult: oral dose 3-5 Gms every 6 hours or 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 or 3 - 8 days

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

Tese medications may be available through the amilys home health company, the hemophiliatreatment center, or the amily may have a supply at home. Tey are difficult to obtain rom mostpharmacies.

Follow-up through the hemophilia treatment center or patients hematologist.

opical agents such as opical Trombin and Geloam may also be used to help control mucous

membrane bleeding.

Antibiotics and pain medications may also be indicated.




18/28


Factor Administration

Factor Reconstitution

Filter needleTransfer needle

attach

syringe

Note: Draw the

reconstituted factor into a

syringe. Discard the filter

needle after withdrawing

the factor.

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

monoclonal or recombinant

Coagulation factor IX (9)*

monoclonal or recombinant

The volume will

vary by product.

Mixing instructions and the rate of administration are found on the drug insert.

Document the lot number(s), expiration date(s), factor concentrate trade name

and total number of units infused.

Note: Patients may request

the box top for their records.



Note: This illustration shows one way to reconstitute factor. Each product has

its own transfer system. Please follow directions on the drug insert.

What if the patient brings his own factor?

Some patients are instructed to

bring unmixed factor concentrate

with them to the ED to minimizetreatment 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.


19/28


Factor types

RecombinantReers to genetically engineered Factor VIII (8) or IX (9) concentrates which are not derived rom plasma.

A recombinant actor VII activated product (NovoSeven) is available or the treatment o inhibitors to FactorVIII (8) or IX (9) and actor VII (7) deficiency.

High purity / Monoclonal - plasma productFactor VIII (8) or IX (9) concentrates o high specific activity and purity o the specific clotting actor achievedthrough the use o an affinity column matrix.

Intermediate purity - plasma productReers to Factor VIII (8) concentrate with lower specific activity. Some contain von Willebrand actor and areused to treat von Willebrand Disease.

Factor IX (9) prothrombin complex concentrates (PCCs) - plasma productAlso contains some other clotting actors and small amounts o activated coagulation actors. Used to treatHemophilia B and some patients with Factor VIII or IX inhibitors. When used to treat Factor VIII (8)

inhibitor, the dose is 75 IU/kg or all situations. I a patient brings PCC product to the ED, veriy whether hisdiagnosis is Factor IX deficiency or Factor VIII inhibitors as the dosage is different.

Activated prothrombin complex concentrates - plasma productProthrombin complex concentrates purposely activated so they contain some Factor IX (9), Factor X (10),etc. in the active orm or use with Factor VIII (8) inhibitor patients only. Te brands are Autoplex and FEIBA.

Factor AdministrationEmergency Care for Patients with Hemophilia

Dosage

Each bottle o actor concentrate is labeled with the activity expressed as International Units (IU, example: 287 IU).Te dosage to be administered is based on the patients body weight in kilograms (kg)*.

Te ENTIRE contents o all the vials reconstituted or an inusion should be used, even i it exceeds the calculateddosage. A larger dose will only prolong the period o normal coagulation. Due to its expense, factor concentrateshould never be discarded!

Te hal-lie o actor VIII (8) is 8-12 hours; the hal-lie o actor IX (9) is 18-24 hours.

Factor VIII (8) is calculated using the ormula: 1 IU/kg = 2% rise in Factor VIII (8) activity 50 IU/kg = 100% correction (example)

Factor IX (9) is calculated using the ormula: 1 IU/kg = 1% rise in Factor IX (9) activity

80 IU/kg = 80% correction (example)

IMPORTANT!

hes e a re exa mp l es .Please reer to page 1 orspecific dose ranges set bythe patients hemophiliatreatment center.


20/28


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.

Scalp vein (infants only)

Hickman, Broviac or

Groshong catheter

Antecubital fossa

(caution in infants)

Dorsum of feet

(infants/children)

Dorsum of hand

Percutaneous intravenous

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

Port(use Huber needle only)



21/28


Intravenous access or a person with hemophilia is basically the same as or any patient. Te use o 23 gauge or 25gauge winged needles is preerable, especially or children.

Sites for access

Sites to consider or a peripheral IV include:

- dorsum o the hands - antecubital ossa (caution in inants

due to the risk o compartment syndrome) - dorsum o eet (inants and children)

- scalp veins (inants only)

The patients IV should not be started in the affected limb. The injured area should beminimally 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 institutions procedure

External central catheter - Hickman, Broviac, or Groshong; access per your institutions procedure

P.I.C.C. line - access per your institutions procedure

Heparin ush

It is recommended to do a final flush with heparin or any venous access device (except or Groshongs whichare only flushed with normal saline). Check with the parent or the patients institution or the amount oheparin flush to use. I these options are not available, use your institutions procedure or the amount andconcentration o heparin. Tis small amount o heparin will not harm the patient (remember - youve justgiven him actor). Te access device needs to stay patent; this is accomplished with the heparin.

The patient on prophylaxis

Some patients with hemophilia now receive prophylactic doses o actor replacement on an every-other-dayor 2-3 times a week routine schedule. Tis should be asked o the patient. Tese patients may have venousaccess devices or inusions. Always check to see i the patient has a venous access device beore starting aperipheral inusion.

Venous accessEmergency Care for Patients with Hemophilia

NOTE: Groin and neckveins are contraindicatedexcept in life-threateningsituations.


22/28


In any situation, the inusion ofactor should never be delayed i any bleeding is suspected. Delaying theinusion simply increases bleeding that will result in greater morbidity. Diagnostic work-ups are not indicated orpatients with an established hemophilia diagnosis. In general, patients with hemophilia who are experiencing anacute bleeding episode first need a actor inusion. Other procedures should not be done unless there is anotherclinical indication or the study.

Invasive procedures, labs, x-rays


Head injury

(see page 5)

Fracture

First give a major

dose of factor*. . .

. . . then apply

the cast.

First give a major

dose of factor*. . .. . . then perform

a CT scan.


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

day.

If the patient received sutures, remind the patient he will need factor for suture removal.

If the patient received a head injury, discharge with routine post head injury instructions

(patient to follow-up for two weeks instead of 48 hours - see page 5).



23/28


Laboratory studies

I the only complaint is an acute joint or muscle bleed, no laboratory studies are necessary. I GI, abdominal,large muscle, or oral cavity bleeding is suspected and has potentially been extensive, a CBC may be indicatedto determine i the individual is anemic. Factor levels and inhibitor levels are not necessary or treatmentin an acute emergency setting. Factor should not be delayed for laboratory studies to be drawn or

completed.

X-rays and other radiological studies

Give factor rst, then decide i a radiological study is indicated. Remember that a swollen joint or extremity isusually the result o internal bleeding, not a racture. X-rays o the joint can be used to document a joint bleed, butare generally not useul in detecting early onset bleeds (and that is when treatment is optimal). Te patient will beaware o joint bleeding beore radiological changes are evident.

A C o the head (see page 5) is necessary when dealing with a potential head bleed. It can document location and

extent o bleeding and help direct urther treatment. First give a major actor dose.*

Fractures

Give a major dose o actor* replacement, then x-ray and set the bone.

Lacerations and sutures

Sutures and staples should be used as on any other patient. I the laceration is significant enough to require sutures,the patient should first receive a routine dose o actor* then the procedure. Contact the patients hematologist or

ollow-up actor inusion instructions. For removal o sutures, a routine dose o actor* is usually needed.

Invasive procedures

Invasive procedures should be perormed as clinically indicated, i.e., lumbar puncture with symptoms omeningitis. However, a major dose o actor* should be given before the procedure begins.

Arterial sticks and venipunctures

Do not do arterial sticks unless no other option is available. I an arterial stick must be done, then a major actordose* must be given first.

Venipuncture (see page 19) may be done at any location; hands are generally excellent and no actor treatment isnecessary. Avoid digging or deep veins. Apply pressure or several minutes afer the puncture.

Invasive procedures, labs, x-rays




24/28


Routine medications

Patients with hemophilia can receive routine medications (e.g. pain medications, antibiotics, etc.) that do notinterere with clotting unction. Avoid non-steroidal anti-inflammatories (NSAIDS), ASA and any product withaspirin-related ingredients (e.g. Pepto-Bismol, Excedrin, Percodan).

Medications for fever or pain

Acetaminophen can be given or ever or pain. Narcotics/opioids are ofen recommended to control the painexperienced by a patient with hemophilia.Avoid giving intramuscular injections of antibiotics, painmedications, or immunizations because of the possibility of causing a muscle bleed.

Routes of administration

Medications which can be given PO, SC, or IV are preerred. Routine immunizations and tetanus toxoid maybe given subcutaneously. I the rabies vaccination series is needed, an experienced hematologist (preerablythe patients) should be contacted to arrange actor inusions prior to and afer the injections in order to preventinternal bleeding.

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

Caution

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

Other medications

Pressure and ice pack after injections



25/28


Trauma / Emergencies

Trauma / Emergencies

Many different emergencies/traumas may occur to persons with hemophilia, just as to others. Te more commonare:

- Animal bites - Motor vehicle accidents

- Burns - Myocardial inarctions

- Falls - Ocular injuries

- Fractures (see page 21) - Puncture wounds

- Gunshot wounds - Dislocated joints

Treatment

A major dose o actor* should be inused as soon as possible (beore any test, x-rays, debriding, sutures, etc.).




26/28


Inhibitors

How inhibitors are measured

Labs express the presence o an inhibitor in terms o Bethesda units (BU). One Bethesda unit is the amount oantibody that destroys hal o the actor VIII in an equal mixture o normal and patient plasma in two hours.

Low responding inhibitor measures less then 10 BU. High responding inhibitor measures greater than 10 BU. Aninusion o actor concentrate urther stimulates the inhibitor antibodies, causing a rise in BUs.

When to suspect an inhibitor

Suspect an inhibitor i bleeding doesnt stop afer several inusions o actor concentrates.

Call the patients hematologist i the inhibitor is known or suspected beore attempting treatment. Be sure to alsoask the patient and amily i they have been told the patient has an inhibitor.

Treatment of inhibitors

Inhibitor management is difficult or the experienced hematologist. Contact the patients hematologistor hemophilia treatment center when these patients present in the emergency department fortreatment. Bleeding in an inhibitor patient can quickly lead to serious lie- or limb-threatening complicationswithout expert management.

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 aninfusion of factor concentrate.

Contact the hemophilia treatment center or the

patients hematologist due to the complexity of

managing inhibitors.



27/28


Low Responding Inhibitors:

May be able to overwhelm the inhibitor by dosing 2-3x the routine range.*Call the hemophilia treatment center or the patients hematologist orguidance.

High Responding Inhibitors:

Recombinant Factor VIIa (activated)

e.g. NovoSeven

Activated Prothrombin Complex Concentrates (aPCC)

e.g. FEIBA, Autoplex

Prothrombin Complex Concentrates (PCC)

e.g. Bebulin, Proplex, Profilnine

Porcine Factor VIII Concentrate

e.g. Hyate:C

Due to the complexity o managing inhibitors, call the hemophilia treatment

center or patients hematologist or guidance when using these products.

Inhibitors

Products used to treat bleeding in patients with inhibitors



28/28

Emergency Care or Patients with Hemophilia was written and prepared by the Nursing Group o HemophiliaRegion VI (AR, LA, OK, X) in order to help acilitate prompt care o patients through their emergencydepartments. Te group wishes to thank the ollowing or their contributions:

Reviewers:

Howard Britton, M.D.

Medical Director, South exas ComprehensiveHemophilia Center, San Antonio, X

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

Hemophilia Care Center, New Orleans, LA

Arlo Weltge, M.D. Associate Proessor o Surgery/ER Medicine,

Te University o exas Medical School, Houston, X

Rita Gonzales consumer parent and past President, Lone Star

Hemophilia Chapter, Houston, X

Acknowledgements

Nurses from Region VI Hemophilia Treatment Centers:

Cook Childrens Medical Center, Ft. Worth, X

Gul States Hemophilia and Trombophilia Center, Houston, X

Hemophilia Center o Arkansas, Little Rock, AR

Louisiana Comprehensive Hemophilia Care Center, New Orleans, LA

North exas Comprehensive Hemophilia Center,

Adult Program, Dallas, X

North exas Comprehensive Hemophilia Center,

Pediatric Program, Dallas, X

Te Oklahoma Center or Bleeding Disorders, Oklahoma City, OK

South exas Comprehensive Hemophilia Center, San Antonio, X

The Nursing Group o Hemophilia Region VI also wishes to thank Novo Nordiskwhose generous educational grant made possible this printed material as well as theweb site www.HemophiliaEmergencyCare.com

Production eam - Project Manager / Layout: Art Gardner, Illustrations: Mark Gilmore and Heather Swaim


Emergency Care for Patients With Hemophilia FINAL

Documents

Transcript of Emergency Care for Patients With Hemophilia FINAL