Emergency Care for

Patients with Hemophilia

An instructional manual for Medical Professionals
Third Edition
Written by the Nursing Group of Hemophilia Region VI
Editors: Karen Wulff, R.N.; Susan Zappa 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 injury (pg. 4)
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.

Abdominal bleeds (pg. 10),

Trauma (pg. 23)
Treat with a major factor dose

Slings, splints, immobilizers

p.r.n. for joint bleeds

Avoid intramuscular
injections due to the
possibility of causing a
muscle bleed

Crutches
hip, knee, ankle bleeds

Minor cuts / bruises

no treatment
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)
Severe to moderate deficiency:

Recombinant Factor VIII concentrate

Dosage 20 - 30 units per kilogram

Hemophilia A or Factor VIII (8)

Mild deficiency:

DDAVP responsive: DDAVP dosage: 0.3 micrograms 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

Severe/Moderate/Mild:

Recombinant Factor IX concentrate

Dosage 35 - 50 units per kilogram

For mucosal bleeds in all of the above add:

Tranexamic Acid (Cyklokapron) Topical (mouthwash) 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)
Severe/Moderate/Mild deficiency

Recombinant Factor VIII concentrate

Dosage 40 - 50 units per kilogram

Hemophlia B or Factor IX (9)

Severe/Moderate/Mild deficiency

Recombinant Factor IX concentrate

Dosage 100 - 120 units per kilogram

Hemophilia Program of 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

Dose Chart

Medical Director
Dr. Linda Vickars
Clinical Nurse Specialist
Deb Gue, RN, MSN

dgue@providencehealth.bc.ca

Patient/Nurse Educator
Kam McIntosh, RN, BSN

kmcintosh@providencehealth.bc.ca

Program Secretary
Alex Weatherston

aweatherston@providencehealth.bc.ca

All major bleeding episodes should be considered potentially critical.

The goal is to raise the factor level to 80 - 100%.
Note: For patients with Factor 8 and 9 inhibitors, it is imperative that you contact the
patients hemophilia physician before any infusions. Contact information and numbers are
located above.

C o n t e nt

Table of Contents

Introduction

H emo ph ili a bas ic s

H ead in jur y

Joint bleeding

Signs & symptoms, Aspiration, Dislocated joints

Muscle /soft tissue bleeding

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 formula, Factor types

Venous access

18

Site for administration, Prophylaxis, Venous access devices

Invasive procedures / labs / x-ray

20

Fractures, Lacerations, LP, Blood gases

Other medications

22

Pain medications, Immunizations

Trauma / emergencies

23

Bites, Burns, Falls, Fractures, Gunshot wounds, MVA, MI, Ocular

injuries, Puncture wounds

Inhibitors

24

Definition, Treatment
Copyright 1999, 2010 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, or
registered trademarks of their respecitve owners. These terms are used in this publication only in an editorial
fashion and should not be regarded as affecting their validity.

Phone
List

Hemophilia Treatment Centers

ii
4

Introduction
Intro

Purpose
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.

Basics

Hemophilia basics

Definition
Hemophilia is a genetic disorder characterized by a deficiency or absence of one
of 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.
Hemophilia mostly affects males due to the X-linked inheritance pattern.

Effects of hemophilia
Hemophilia prevents the formation of a firm, fibrin clot and results in a soft,
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
The 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 four times more
common than factor IX (9) deficiency but the clinical presentations and inheritance
patterns are the same.

Severity
The amount of bleeding expected in an individual with hemophilia depends upon
the severity of the deficiency. Normal plasma levels of factor VIII (8) and IX (9)
range from 50-150%.
Those with less than 1% factor VIII (8) and IX (9) are considered to have severe
hemophilia. Frequent bleeding episodes are common, particularly into joints.
Bleeding can occur for no known reason or from trauma.
Persons with factor levels of 1-5% are considered to have moderate hemophilia.
These persons may experience bleeding after minor trauma. After repeated bleeding
into the same joint, persons with moderate hemophilia may experience bleeding in
that joint with minor trauma.
Persons with more than 5% factor activity are considered to have mild
hemophilia and bleed only after significant trauma or with surgery. Some carrier
girls and women (called symptomatic carriers) can have lower than normal plasma
levels of factor VIII (8) or IX (9) and thus may exhibit symptoms of mild
hemophilia.

Hemophilia basics

Serious bleeding sites

Basics

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
Treatment for bleeding involves replacing the deficient factor as the first course of
action. This requires intravenous infusion of commercial factor concentrates. Specific
doses, additional drugs and medical interventions depend upon the site and severity of
bleeding. Once factor replacement therapy has been infused, diagnostic procedures
and examinations can begin.

Family
Parents and persons with hemophilia are knowledgeable about the management of the
disorder and their input should be sought and heeded. Most hemophilia families are
medically sophisticated and should not be dismissed as novices.
Interview the family about whether factor concentrate has been administered prior to
arriving at the ED; if so, determine when and at what dose. Additional factor may be
required, depending upon the time lag and severity of the bleed. Establish who the
treating hematologist or hemophilia treatment center is and contact them for other
than routine bleeding.

Head injury

Head

Treat all head injuries with or

without swelling.

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.
Head

Treatment
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

Joint

Joint bleeding

Advanced joint bleed

- heat
- pain
- swelling
- decreased 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
Treatment: a routine factor dose.*

Remember, toes and fingers

are joints too.

Discharge Instructions
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 patient's

hematologist.

*Dose chart inside front cover

Joint bleeding
The hallmark of hemophilia is joint and muscle bleeding. Spontaneous joint and muscle
bleeding can occur without a definite history of trauma. The patient may not be able to
identify a specific event that resulted in bleeding.

Joint

While persons with hemophilia may bleed into any joint space, the joints which most
frequently bleed are the ankles, knees, and elbows. Other possible bleeding joint sites include
the shoulders and hips. As repeated bleeding occurs, the synovial tissue thickens and develops
even more friable blood vessels. A vicious cycle of bleeding and rebleeding may set in and the
affected joint is referred to as a "target joint." Eventually, repeated bleeding into joints leads to
a form of chronic arthritis with destruction of cartilage and the eventual destruction of bone
resulting in decreased joint mobility and function.

Signs and symptoms

Outward signs of joint bleeding include restriction of movement, swelling, heat, and
erythema on and around the joint. The patient may report symptoms of a bubbling or
tingling sensation with no physical signs. Later symptoms include a feeling of fullness within
the joint and moderate to severe pain as the bleed worsens.

Treatment
Some patients may present for treatment with no other outward signs of bleeding than
decreased range of motion and a complaint of pain or tingling. This is indicative of an early
onset joint bleed and is the optimal time to treat. The patient should be infused as quickly
as possible with a routine dose of factor* in order to minimize pain and joint destruction.
Extreme pain, swelling, heat, and immobility are signs and symptoms of an advanced joint
bleed which occurs only after blood has filled the joint space. Symptoms suggestive of an
advanced joint bleed require a major factor dose.*
Infuse before any diagnostic procedures such as x-ray. If a joint bleed is treated early before
obvious outward signs occur, then the need for expensive follow-up infusions may be lessened
or avoided altogether. Before dislocated joints are reduced, infuse with a major factor
dose.*

Joint aspiration: Caution!

Joint bleeds in hemophilia are generally not treated by aspirating the joint. This procedure can
make matters worse by creating another site from which the patient may bleed. If joint
aspiration is deemed necessary, such as in suspected joint sepsis, then the joint should be
aspirated by an orthopedic surgeon associated with a hemophilia treatment center. The
patient's hematologist must be involved in order to arrange for factor coverage before and
after 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 wrapping with an ace bandage. For ankle bleeds, an Aircast
may be a useful splinting device. Crutches are useful to help individuals when they have lower
extremity joint bleeds and need to be non-weight bearing. Follow-up should be made to the
local hemophilia treatment center or to the patient's hematologist as soon as possible.
*Dose chart inside front cover

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
Iliopsoas bleeds
- flexed hip
- pain/inability to extend
the ll[(s)]TJ[(p)]TJ0.9526 0 Td* [(e)]TJ0.514 0 Td[(c)]TJ0.4612 0 Td[(t)]TJ[(e)]TJ0w981.4 0 Td(*)9.e

Deltoid / forearm bleed

- routine factor dose*
- major factor dose* if a
compartment syndrome
is suspected

Buttock bleeds
- pain
- with/without swelling
- routine dose of factor*
- major dose of factor* if
the leg on the affected
side exhibits tingling or
swelling
Thigh/calf bleed
- pain
- with/without swelling
- impaired mobility
- routine factor dose*
- major factor dose* if a
compartment syndrome
is suspected

Gastrointestinal / urinary tract bleeding

GI/GU

Nausea and vomiting may indicate

intracranial hemorrhage as well as
gastrointestinal problems.

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
- rest
- no weight lifting

10

*Dose chart inside front cover

- report any symptoms

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

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.
GI/GU

A history of lifting heavy objects, weight lifting, falling on bicycle handlebars or stretching
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.

Mucous
Membr.

Nose bleeds may respond to

other measures. Refer to
"Controlling Epistaxis" table on
page 14.

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
hemophilia treatment center or
hematologist the next day.

12

*Dose chart inside front cover

Instruct the patient on:

Controlling Epistaxis, Anti-fibrinolytics, Diet
Modification tables (pages 14 - 15) as
needed.

Mucous membrane bleeding

Mucous membrane bleeding may require medical care in the emergency department. Patients
should receive factor who:
are experiencing profuse and/or prolonged bleeding
have sustained a known injury to the mouth, tongue, or nose
have severe swelling in the mouth or naso-pharynx area
are experiencing respiratory distress
have difficulty swallowing
The patient may not know the reason for the symptom or bleeding. It may have been caused
by trauma, infection, or the bleed may be spontaneous. If airway blockage is suspected, the
patient should immediately receive a major dose of factor.* After the factor level has been
raised, further interventions including invasive procedures may be done.
Do not make a person with hemophilia wait for factor replacement. The longer he
waits, the more bleeding takes place. If the bleed is in a closed space, the accumulation of
blood will cause surrounding tissue damage, airway obstruction, and enhance pain.

Mucous
Membr.

Epistaxis
A person with epistaxis who is unable to control the bleed himself may need a routine dose
of factor* and anti-fibrinolytic treatment (Amicar). Be sure the person knows how to control
and stop the bleeding. (See table on page 14.)

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* may temporarily stop the bleeding, but clots break down
normally on days 3-5 and bleeding may start again at that time. An anti-fibrinolytic may be
indicated to maintain hemostasis. (See table on page 15.) Anti-fibrinolytics may be available
through the patient's home health company for next day delivery. A modified diet should be
started at the same time as factor therapy. (See table on page 15.)
Bleeding may occur with erupting or exfoliating teeth. It is more common with exfoliating
teeth, especially a tooth that is very loose. A dental consult may be needed to extract the tooth
since it will continue to lacerate the tooth socket as long as it is in place. A major or routine dose of
factor* should be given prior to extraction. A frenulum or tongue laceration will require a
major or routine dose of factor.*

Retropharyngeal
After the major dose of factor* is given, further observation, x-rays and admission may be
required.

*Dose chart inside front cover

13

Mucous membrane bleeding

Controlling Epistaxis
Instruct 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 firm pressure to the entire side of the nose that is bleeding for 15 minutes.

Mucous
Membr.

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.

14

*Dose chart inside front cover

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.
Mucous
Membr.

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.

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

Factor

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

plasma derived or recombinant
Coagulation factor IX (9)*
plasma derived or recombinant

Factor box top

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.
Patients may request the box top for their records.
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.

16

*Dose chart inside front cover

Factor administration

Dosage
Each bottle of factor concentrate is labeled with the activity expressed as International Units
(IU, example: 287 IU). The dosage to be administered is based on the patient's body weight
in kilograms (kg)*.

IMPORTANT!
These are examples. Please
refer to the inside front
cover for specific dose
ranges set by your local
hemophilia treatment
center.

Factor VIII (8) is calculated using the formula:

1 IU/kg = 2% rise in Factor VIII (8) activity
50 IU/kg = 100% correction (example)
Factor IX (9) is calculated using the formula:
1 IU/kg = 1% rise in Factor IX (9) activity
80 IU/kg = 80% correction (example)

The ENTIRE contents of all the vials reconstituted for an infusion should be used, even if it
exceeds the calculated dosage. A larger dose will only prolong the period of normal
coagulation. Due to its expense, 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.

Factor types

Plasma derived product

Factor VIII (8) or IX (9) concentrates of high specific activity and purity of the specific
clotting factor achieved through the use of an affinity column matrix. Some contain von
Willebrand factor and are used to treat von Willebrand Disease.
Activated prothrombin complex concentrates - plasma product
Prothrombin complex concentrates purposely "activated" so they contain some Factor IX
(9), Factor X (10), etc. in the active form for use with Factor VIII (8) inhibitor patients
only. The brand is FEIBA. Dose is 75 IU/kg.
Factor IX (9) prothrombin complex concentrates (PCCs) - plasma product
Also contains some other clotting factors and small amounts of activated coagulation
factors. Used to treat Hemophilia B and some patients with Factor VIII or IX inhibitors.

*Dose chart inside front cover

17

Factor

Recombinant
Refers to genetically engineered Factor VIII (8), IX (9) or VII (7) concentrates which are not
derived from plasma.

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.

Port
(use non-coring needle only)

Scalp vein (infants only)

Hickman, Broviac or
Groshong catheter

Venous
Access

Antecubital fossa
(caution in infants)

Percutaneous intravenous central

catheter (P.I.C.C.) line
Dorsum of hand

Dorsum of feet
(infants/children)

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:

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

Venous
Access

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

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

dose of factor* . . .

. . . then perform
a CT scan.

Fracture

Labs
X-Ray

First give a major

dose of factor* . . .

. . . then apply
the cast.

Discharge Instructions
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).

20

*Dose chart inside front cover

Sutures: Remind the patient he will

need factor for suture removal.

Invasive procedures, labs, x-rays

In general, patients with hemophilia who are experiencing an acute bleeding episode need factor only.
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 studies
If the only complaint is an acute joint or muscle bleed, no laboratory studies are necessary. If GI,
abdominal, large muscle, or oral cavity bleeding is suspected and has potentially been extensive, a CBC
may be indicated to determine if the individual is anemic. Factor levels and inhibitor levels are not
necessary 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 studies

Give factor first, then decide if a radiological study is indicated. Remember that a swollen joint or
extremity is usually the result of internal bleeding, not a fracture. X-rays of the joint can be used to
document a joint bleed, but are generally not useful in detecting early onset bleeds (and that is when
treatment is optimal). The patient will be aware of joint bleeding before radiological changes are
evident.
A CT of the head (see page 5) is necessary when dealing with a potential intracranial hemorrhage. It
can document location and extent of bleeding and help direct further treatment. First give a major
factor dose. *

Fractures
Give a major dose of factor* replacement, then x-ray and set the bone.

Lacerations and sutures

Invasive procedures
Invasive procedures should be performed as clinically indicated, i.e., lumbar puncture with symptoms
of meningitis. However, a major dose of factor* should be given before the procedure begins.

Arterial sticks and venipunctures

Do not do arterial sticks unless no other option is available. If an arterial stick must be done, then a
major factor dose* must be given first.
Venipuncture (see page 19) may be done at any location; hands are generally excellent and no factor
treatment is necessary. Avoid "digging" for deep veins. Apply pressure for several minutes after the
puncture.
*Dose chart inside front cover

21

Labs
X - Ra y

Sutures and staples should be used as on any other patient. If the laceration is significant enough to
require sutures, the patient should first receive a routine dose of factor* then the procedure. Contact
the patient's hematologist for follow-up factor infusion instructions. For removal of sutures, a routine
dose of factor* is usually needed.

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

Meds/
Trauma

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.

Routes of administration
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
Burns
Falls
Fractures (see page 21)
Gunshot wounds

Motor vehicle accidents

Myocardial infarctions
Ocular injuries
Puncture wounds
Dislocated joints

Meds / Trauma

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

High Responding Inhibitors

Products used to treat 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

Inhibitors

Factor product and dose is variable.

Dose may be 2-3x routine range.*

24

*Dose chart inside front cover

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.
Inhibitors

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

Hemophilia Center, San Antonio, TX

Gulf States Hemophilia Diagnostic and Treatment Center, Houston, TX

Hemophilia Center of Arkansas, Little Rock, AR

Cindy Leissinger, M.D.

Louisiana Comprehensive Hemophilia Care Center, New Orleans, LA

Medical Director, Louisiana Comprehensive

Hemophilia Care Center, New Orleans, LA

North Texas Comprehensive Hemophilia Center,

Arlo Weltge, M.D.

Associate Professor of Surgery/ER Medicine,
The University of Texas Medical School, Houston, TX

Adult Program, Dallas, TX

North Texas Comprehensive Hemophilia Center,

Rita Gonzales

Pediatric Program, Dallas, TX

consumer parent and past President, Lone Star

Hemophilia Chapter, Houston, TX

The Oklahoma Center for Bleeding Disorders, Oklahoma City, OK

South Texas Comprehensive Hemophilia Center, San Antonio, TX

Sponsors

1-800-727-6500
www.novonordisk.com

The Nursing Group of

Hemophilia Region VI also
wishes to thank the following
sponsors whose generous
educational grant made
possible Emergency Care for
Patients with Hemophilias
second printing.

1-800-288-8371 www.bayerusa.com

1-800-423-2090 www.hemophiliagalaxy.com

1-888-440-8100 www.genetics.com

1-713-500-8360 www.hemophiliaregion6.org

1-800-292-6118 www.alphather.com

1-202-639-3520
www.redcross.org

1-210-704-2187 www.uthscsa.edu/pedionc

1-800-340-7502 www.aventis.com

1-800-456-7322
www.hyate.com

1-800-327-7106
www.nabi.com

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