Emergency Care for Patients with Hemophilia

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Emergency Care for

Patients with Hemophilia

Slings, splints, immobilizers

p.r.n. for joint bleeds

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.

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

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.

An

instructional manual for

Medical Professionals

Factor Replacement Doses

Hemophilia A or Factor VIII (8)

Severe to moderate deficiency:

Hemophilia A or Factor VIII (8)

Mild deficiency:

Hemophilia B or Factor IX (9) deficiency

Severe/Moderate/Mild:

For mucosal bleeds in all of the above add:

Recombinant Factor VIII concentrate

Dosage 20 - 30 units per kilogram

DDAVP responsive: DDAVP dosage: 0.3 micro-

grams per kilogram (Maximim dose: 20

micrograms)

DDAVP non-responsive: Recombinant Factor VIII

concentrate.

Dosage 20 - 30 units per kilogram

Recombinant Factor IX concentrate

Dosage 35 - 50 units per kilogram

Tranexamic Acid (Cyklokapron) “Topical (mouth-

wash) or systemic” as per bleeding site

(Contraindicated if hematuria)

HemophiliaProgram 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

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

patient’s hemophilia physician before any infusions. Contact information and numbers are

located above.

TREATMENT FOR MINOR BLEEDS

Minor bleeds include: Nose (epistaxis), Mouth (including

gums) Joints (hemarthroses), Abrasions and superficial lacerations

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

Hemophlia B or Factor IX (9)

Severe/Moderate/Mild deficiency

Recombinant Factor VIII concentrate

Dosage 40 - 50 units per kilogram

Recombinant Factor IX concentrate

Dosage 100 - 120 units per kilogram

8

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Compartment syndrome, Iliopsoas

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.

Table of Contents

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Signs & symptoms, Aspiration, Dislocated joints

Gastrointestinal

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urinary tract bleeding

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Abdominal muscle wall, Hematuria

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Anti-fibrinolytics, Epistaxis, Retropharyngeal bleeds

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Mixing, Dosage formula, Factor types

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Site for administration, Prophylaxis, Venous access devices

Invasive procedures / labs / x-ray

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Fractures, Lacerations, LP, Blood gases

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Pain medications, Immunizations

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

injuries, Puncture wounds

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Definition, Treatment

Hemophilia Treatment Centers

1

To The Attending Medical Staff:

This manual is a guide for medical personnel who may be

less familiar with hemophilia treatment. Its content consists

of guidelines, recommendations and suggestions only. The

attending physician has the final responsibility for

appropriate diagnosis and treatment.

Purpose

This manual contributes to hemophilia care by enhancing the emergency

department staff's understanding of hemophilia and its treatment. The goals 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.

tr

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Introduction

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.

ci

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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 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.

Hemophilia basics

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Discharge Instructions

Head injury

First, infuse a major factor dose.

Then perform diagnostic studies,

such as CT scan, neuro exam.

Treat all head injuries with or

without swelling.

Call the hemophilia treatment center or

the patient's hematologist for follow-up

factor doses.

Report any signs or symptoms to the

hemophilia treatment center or the

patient's hematologist.

Head injury instructions for a two

week period (instead of the usual

instructions for 24 - 48 hour period).

*Dose chart inside front cover

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.

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

Early onset joint bleed

- tingling

- pain

- limited range

of motion

Treatment: a routine factor dose.

Joint bleeding

Discharge Instructions

Advanced joint bleed

- heat

- pain

- swelling

- decreased range

of motion

Treatment: a major factor dose.

Remember, toes and fingers

are joints too.

Crutches for weight bearing

joints and crutch instructions.

- RICE (rest, ice, compression

[ace wraps], elevation)

For the next 24 hours:

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

hematologist.

*Dose chart inside front cover

- sling or splinting if support

is needed (i.e. Aircast® for ankles)

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 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

Joint 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

swelling

Iliopsoas bleeds

- flexed hip

- pain/inability to extend

the leg on the affected

side

- major factor dose

Discharge Instructions

- rest

- ice

- non-weight bearing

- follow-up with the hemophilia treatment

center or the patient's hematologist

*Dose chart inside front cover

Muscle / soft tissue bleeding

and hospital admission

9

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 of factor.

* Common bleeding sites include the upper arm, forearm, thigh, and calf

muscles. Toddlers frequently have buttock and groin bleeds. Abdominal wall muscles and

iliopsoas muscles are also common bleeding sites. These abdominal muscles generally do not

have observable swelling, yet they may hold a large amount of blood. Patients who complain

of low abdomen or groin pain, especially with signs of nerve compression, are probably

experiencing an iliopsoas bleed. These patients should receive an emergency hematology

consult and possible admission for observation and several major doses of factor coverage.

The hallmark sign of iliopsoas bleeding is spontaneous flexion of the leg on the affected side

with an inability to extend the leg without pain.

Consequences of muscle bleeds

Muscle bleeds can result in serious consequences if not treated promptly. Extensive blood loss

may occur in large muscle groups. Muscle bleeding places pressure on nerves and blood vessels

and, if left untreated, these bleeds may result in permanent disabilities such as foot drop and

Volkmann’s contracture. It is important that the patient's hematologist be consulted before any

surgical consults. Most muscle bleeds respond well to medical management and do not require

fasciotomy. Such an extreme measure will usually generate problems for the patient and

require a tremendous amount of post-surgical factor infusions, more so than if the patient was

treated medically.

Treatment and follow-up care

Most muscle bleeds are treated with a routine dose of factor.

* Large abdominal muscle groups

and iliopsoas bleeds should be treated with a major dose of factor.

* Rest and ice packs are also

helpful. The patient should be referred back to his hemophilia treatment center or to his

hematologist for follow-up as soon as possible. If any suspicion of compartment

syndrome and nerve compression exists, then the patient should have an emergency

hematology consult and should be admitted to the hospital.

Soft tissue and superficial bleeds

Soft tissue bleeds usually do not require aggressive treatment. Superficial hematomas and

bruises may appear anywhere on the body and, if they do not threaten function and mobility,

they do not need to be treated.

*Dose chart inside front cover

Muscle / soft tissue 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 hemophilia

treatment center or

the patient's hematologist

Iliopsoas bleeding

- flexed hip

- pain on extension

- major dose of factor

Discharge Instructions

- report any symptoms

- follow-up with the hemophilia treatment

center or the patient's hematologist

- force fluids for hematuria

- rest

- no weight lifting

Nausea and vomiting may indicate

intracranial hemorrhage as well as

gastrointestinal problems.

*Dose chart inside front cover

Gastrointestinal / urinary tract bleeding

11

*Dose chart inside front cover

U

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.

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.

Gastrointestinal / urinary tract 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.

Discharge Instructions

Patients should follow-up with their

hemophilia treatment center or

hematologist the next day.

Instruct the patient on:

Controlling Epistaxis, Anti-fibrinolytics, Diet

Modification tables (pages 14 - 15) as

needed.

Nose bleeds may respond to

other measures. Refer to

"Controlling Epistaxis" table on

page 14.

*Dose chart inside front cover

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.

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

Controlling Epistaxis

Instruct the patient:

1. To gently blow his nose to remove mucous and unstable clots

which will interfere with hemostasis.

Mucous membrane bleeding

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.

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

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.

*Dose chart inside front cover

olive oil in the nares to keep the membranes soft and

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.

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.

Avoid using a straw. Negative pressure from the sucking action can dislodge

the clot and aggravate the bleeding site.

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

*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.

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.

Factor administration

Factor box top

Factor Reconstitution

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

plasma derived or recombinant

Coagulation factor IX (9)*

plasma derived or recombinant

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.

*Dose chart inside front cover

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.

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)

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

Recombinant

Refers to genetically engineered Factor VIII (8), IX (9) or VII (7) concentrates which are not

derived from plasma.

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.

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.

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.

IMPORTANT!

These are examples. Please

refer to the inside front

cover for specific dose

ranges set by your local

hemophilia treatment

center.

*Dose chart inside front cover

Venous access

Do not use the affected or injured limb

for venous access.

No jugular or femoral sticks except in

life-saving situations.

Use 25g or 23g "winged" needles. After the

needlestick, apply pressure and bandage.

See factor administration (pages 16 - 17)

for further information.

Dorsum of feet

(infants/children)

Scalp vein (infants only)

Hickman, Broviac or

Groshong catheter

Dorsum of hand

Antecubital fossa

(caution in infants)

Percutaneous intravenous central

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

Port

(use non-coring needle only)

Venous access

Intravenous access for a person with hemophilia is basically the same as for any patient. The

use of 23 gauge or 25 gauge "winged" needles is preferable, especially for children.

Sites for access

Sites to consider for a peripheral IV include:

•

dorsum of the hands

•

antecubital fossa (caution in infants due to the risk of compartment syndrome)

•

dorsum of feet (infants and children)

•

scalp veins (infants only)

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

The patient's IV should not be started in the affected limb. The injured area should be

minimally manipulated, if at all.

Venous access device

Some venous access devices currently used in hemophilia care include:

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

External central catheter - Hickman, Broviac, or Groshong; access per your institution's

procedure

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

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.

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).

Sutures: Remind the patient he will

need factor for suture removal.

Head injury

(see page 5)

Fracture

X-rays and lab are not indicated

for a joint or muscle bleed.

First give a major

dose of factor

* . . .

. . . then apply

the cast.

First give a major

dose of factor

* . . .

. . . then perform

a CT scan.

*Dose chart inside front cover

-R

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

*

Fractures

Give a major dose of factor

replacement, then x-ray and set the bone.

Lacerations and sutures

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.

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

R-

Invasive procedures, labs, x-rays

factor dose.

Other medications

Pressure and ice pack

after injections

Routine medications

Patients with hemophilia can receive routine medications (e.g. pain medications, antibiotics,

etc.) that do not interfere with clotting function. Avoid non-steroidal anti-inflammatories

(NSAIDS), ASA and any product with aspirin-related ingredients (e.g. Pepto-Bismol, Excedrin,

Percodan).

Medications for fever or pain

Acetaminophen can be given for fever or pain. Narcotics/opioids are often recommended to

control the pain experienced by a patient with hemophilia. Avoid giving intramuscular injections

of antibiotics, pain medications, or immunizations because of the possibility of causing a muscle

bleed.

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.

23

Trauma / emergencies

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

The more common are:

•

Animal bites

•

Motor vehicle accidents

•

Burns

•

Myocardial infarctions

•

Falls

•

Ocular injuries

•

Fractures (see page 21)

•

Puncture wounds

•

Gunshot wounds

•

Dislocated joints

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

Trauma / emergencies

Inhibitors

High Responding Inhibitors

Products used to treat bleeding:

Factor IX complex concentrates (PCC)

Used for Factor VIII (8) or IX (9)

patient e.g. Profilnine, Bebulin

Activated PCC

e.g. FEIBA

Recombinant F VIIa (7) (activated)

e.g. NovoSeven

Low Responding Inhibitors

Factor product and dose is variable.

Dose may be 2-3x routine range.

Contact the hemophilia treatment center

or the patient's hematologist due to the

complexity of managing inhibitors.

*Dose chart inside front cover

oti

25

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

h

ib

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:

Nurses from Region VI Hemophilia Treatment Centers:

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

Gulf States Hemophilia Diagnostic and Treatment Center, Houston, TX

Hemophilia Center of Arkansas, Little Rock, AR

Louisiana Comprehensive Hemophilia Care Center, New Orleans, LA

North Texas Comprehensive Hemophilia Center,

Adult Program, Dallas, TX

North Texas Comprehensive Hemophilia Center,

Pediatric Program, Dallas, TX

The Oklahoma Center for Bleeding Disorders, Oklahoma City, OK

South Texas Comprehensive Hemophilia Center, San Antonio, TX

Reviewers:

Howard Britton, M.D.

Medical Director, South Texas Comprehensive

Hemophilia Center, San Antonio, TX

Cindy Leissinger, M.D.

Medical Director, Louisiana Comprehensive

Hemophilia Care Center, New Orleans, LA

Arlo Weltge, M.D.

Associate Professor of Surgery/ER Medicine,

The University of Texas Medical School, Houston, TX

Rita Gonzales

consumer parent and past President, Lone Star

Hemophilia Chapter, Houston, TX