Emergency Care for Patients with Hemophilia: An instructional manual for Medical Professionals

Emergency Care for
Patients with Hemophilia
An instructional manual for Medical Professionals
www.HemophiliaEmergencyCare.com
Determine if the patient has:
Hemophilia A or factor VIII (8) deficiency
Hemophilia B or factor IX (9) deficiency
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
Slings, splints, immobilizers
p.r.n. for joint bleeds
Joint bleeds (pg. 6)
Treat an early onset bleed with a
routine factor dose. Treat an
advanced joint bleed with
a major factor dose.
Avoid intramuscular
injections due to the
possibility of causing
a muscle bleed
Abdominal bleeds (pg. 10),
Trauma (pg. 23)
Treat with a major factor dose
Crutches
hip, knee, ankle bleeds
Minor cuts / bruises
no treatment
Ice pack / Ace wrap
joint bleeds
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.

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Emergency Care for Patients with Hemophilia
Table of Contents
Factor Replacement Guidelines ........................................ 1
Introduction ......................................................................... 2
Hemophilia basics .............................................................. 3
Head injury .......................................................................... 5
Joint bleeding....................................................................... 6
Signs & symptoms, Aspiration, Dislocated joints
Muscle /soft 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 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
Acknowledgements ............................................................ 26
Copyright © 1999 The Nursing Group of Hemophilia Region VI.
Updated September, 2003
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.

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Emergency Care for Patients with Hemophilia
Factor Replacement Guidelines
Quebec Reference Centre for the Treatment of Patients with Inhibitors
Centre d'hematologie - 1er Videotron, Hopital Ste-Justine
3175, chemin de la Cote Ste-Catherine, Montreal, Quebec H3T 1C5
(514) 345-2360
Hemophilia A or Factor VIII (8) deficiency
Hemophilia B or Factor IX (9) deficiency
Routine dose = 15 - 25 units x kg.
Routine dose
use recombinant factor VIII concentrate
<15 yrs = 50 - 70 IU/kg
Examples: sutures, early onset joint
>15 yrs = 35 - 50 IU/kg
bleeds, muscle and mouth bleeds.
use recombinant factor IX concentrate
Examples: sutures, early onset joint
NOTE: Patients with mild Hemophilia A
bleeds, muscle and mouth bleeds.
who are in need of a routine dose can be
treated with DDAVP - 0.3 mcg/kg (max. 20
mcg/kg.). Contraindicated in children<2
Major dose
yrs due to risk of seizures.

<15 yrs = 135 - 160 IU/kg
>15 yrs = 100 - 120 IU/kg
Major dose = 40 - 50 units x kg.

use recombinant factor IX concentrate
use recombinant factor VIII concentrate
Examples: head and neck, GI, chest,
Examples: head and neck, GI, chest,
pelivs and spine, extremity muscle
pelivs and spine, extremity muscle
compartments, iliopsoas muscle and
compartments, iliopsoas muscle and
hip, prior to invasive procedure and
hip, prior to invasive procedure and
advanced joint bleeds.
advanced joint bleeds.
NOTE: For patients with Factor 8 or 9 inhibitors (pages 24 -25), it is imperative you contact the
patient's hemophilia physician before any infusions. Contact information and numbers located
below.
Hematologists:
Dr. Georges Etienne Rivard
(514) 345-2360
Dr. Jean St-Louis
(514) 345-2360
Hemophilia Nurse:
Sylvie Lacroix
(514) 345-2360
Night/Weekend:
Page hematologist on-call
(514) 345-2360
www.HemophiliaEmergencyCare.com 1

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Emergency Care for Patients with Hemophilia
Introduction
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
illustrations provide information points for quick review. The text 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. Contact information can be found on page 1.
To The Attending Medical Staff:
This manual is a guide for medical personnel who
may be less familiar with hemophilia treatment. The
content consists of guidelines, recommendations
and suggestions only. The attending physician has
the final responsibility for appropriate diagnosis
and treatment.
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Emergency Care for Patients with Hemophilia
Basics
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 spontaneously or from
trauma.
Persons with factor levels of 1-5% are considered to have moderate hemophilia. These persons may
experience bleeding after minor trauma but should not bleed spontaneously. After repeated bleeding
into the same joint, persons with moderate hemophilia may experience spontaneous bleeding in that
joint.
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.
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Emergency Care for Patients with Hemophilia
Basics
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
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
ER; 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.
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Emergency Care for Patients with Hemophilia
Head injury
Treat all head injuries, with or without swelling,
by infusing 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 to the patient’s hematologist.
Head injury instructions for a two week
period (instead of the usual instructions for 24- 48
hour period.)
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.
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.
* See page 1 for factor dosage guidelines.
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Emergency Care for Patients with Hemophilia
Joint bleeding
Crutches for weight
Advanced joint bleed
bearing joints and
- heat
crutch instructions.
- pain
- swelling
- decreased range
of motion
Treatment: a major factor dose.*
Remember, toes and
Early onset joint bleed
fingers are joints too.
- tingling
- pain
- limited range
of motion
Treatment: a routine factor dose.*
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
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.
While persons with hemophilia may bleed into any joint space, the joints which most frequently bleed are
the elbows, knees, and ankles. 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.
* See page 1 for factor dosage guidelines.
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Emergency Care for Patients with Hemophilia
Joint bleeding
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
Advanced or Early onset joint bleed?
Some patients may present for treatment with no
other outward signs of bleeding than decreased
range of motion and a complaint of pain or
Swelling and a major
tingling. This is indicative of an early onset joint
decrease in range of
bleed and is the optimal time to treat. The patient
motion are signs of an
should be infused as quickly as possible with a
advanced joint bleed.
routine dose of factor* in order to minimize pain
and joint destruction.
Extreme pain, swelling, heat, and immobility are
A 'tingling' sensation and
signs and symptoms of an advanced joint bleed
a limited or small decrease
which occurs only after blood has filled the joint
in range of motion are
space. Symptoms suggestive of an advanced joint
signs of an early onset
bleed require a major factor dose.*
joint bleed.
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.
* See page 1 for factor dosage guidelines.
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Emergency Care for Patients with Hemophilia
Muscle / soft tissue bleeding
Deltoid / forearm bleed
- routine factor dose*
Neck swelling: EMERGENCY
- major factor dose* if a
- potential airway
compartment syndrome
compromise
is suspected
- major dose of factor*
Soft tissue bleeds
and bruising
- no functional
impairment
- tenderness, but
no severe pain
Buttock bleeds
- no factor needed
- pain
- with/without swelling
- routine dose of factor*
- major dose of factor* if
the leg on the affected
side exhibits tingling or
Iliopsoas bleeds
swelling
- flexed hip
- pain/inability to extend
the leg on the affected
side
Thigh/calf bleed
- major factor dose*
- pain
- with/without swelling
- impaired mobility
- routine factor dose*
- major factor dose* if a
compartment syndrome
is suspected
Discharge Instructions
- rest
- ice
- non-weight bearing
- Follow-up with the hemophilia treatment center or with the patient’s hematologist
* See page 1 for factor dosage guidelines.
www.HemophiliaEmergencyCare.com 8

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