HEMOPHILIA

Hemophilia refers to a group of bleeding

disorders in which there is a deficiency of
factors necessary for coagulation of blood

It is congenital bleeding disorder caused

by genetic lack of at least one coagulating
factor (x-Linked Receive)

CAUSES

Hemophilia A and B are inherited as sexlinked recessive traits and diagnosed in

males

Hemophilia C is an autosomal dominant

or a complete recessive trait

CAUSES

Hageman
factor
deficiency
homozygous autosomal trait

is

Von willebrands disease is a dominant

trait

CLASSIFICATION

Hemophilia A (classic hemophilia) due to

deficiency of factor VIII

Hemophilia A (Christmas hemophilia) due

to deficiency of factor XI

Hemophilia C due to deficiency of factor IX

CLASSIFICATION

Hageman factor deficiency caused by lack

of XII

Von willebrand or vascular hemophilia

caused by decreased levels of factor VIII

PATHOPHYSIOLOGY
Deficiency of factor VIII In hemophilia(AHF)

AHF produced by liver

Formation of thromboplastin for blood

coagulation
Less AHF in blood, more severe disease

Hemophilic patient have 2 of the 3 factors

required for coagulation

They bleed longer period but not at faster

rate

Bleeding into subcetaneous, intramuscular

tissues, hemarthrosis, bony changes, and
crippling deformities occurs after repeated
bleeding

CLINICAL MANIFESTATION

Bruise easily

Epistaxis

Gum bleeding

Prolonged bleeding after minor surgery

such as tooth extraction

CLINICAL MANIFESTATION

Prolonged and frequent bleeding

Bleeding into joints cavities

Excessive
bleeding
circumcision

Subcutaneous and intramuscular hemorrhage

after

injection

or

ASSESSMENT

Frequent bleeding in elbows, knees, and

ankles

Spontaneous joint bleeding or as a result

of trauma

Chronic pain due to repeated episodes of

hemarthrosis

ASSESSMENT

Swelling

Decreased joint motion

Joint degeneration leading to osteoporosis or

muscle atrophy

Hematuria

Intracranial bleeding

DIAGNOSTIC EVALUATION

Genetic history, Clinical symptoms

Lab diagnosis CBC, platelet function test,

clotting studies

Prolonged partial thromboplastin time (PTT)

Functional assays measure the amount of

each coagulating factor in blood plasma

MANAGEMENT

Preventing and controlling bleeding are

the integral aspect of the day to day care
of a child with hemophilia

Replacement reparation of the factor VIII

and IX are available in the form of
cryoprecipitate made from fresh plasma

MANAGEMENT

One unite of plasma (250ml) provides one

bag of mm (75-125u) of coagulation factor

Fresh or frozen plasma can also be used to

correct factor deficiency

Every milliliter of plasma contains 1 unite of

factor VIII and slightly less than 1 unite of
factor IX

MANAGEMENT

Transfusion have been associated with

hepatitis and AIDS, so careful screening
of blood is very necessary

DDAVP
(I-deamino-8-d-arginine
vasopressin) a synthetic form of
vasopressin that increase plasma factor
VIII and VWF level

MANAGEMENT

NSAIDs for pain caused by synovitis but used

with caution because they inhibit platelet
function

Corticosteroids for hematuria,

hemarthrosis and chronic synovitis

acute

NURSING MANAGEMENT

1.

Preventing and controlling bleeding

If bleeding tendency is recognized at birth,
infant should not be circumcised

2.

finger nails should be kept short, since a

small scratch may cause bleeding

3.

Diaper pins should not be used or used with

caution

NURSING MANAGEMENT
4.

Infant should be provided with soft toys

without sharp corners and a hazards free
environment

5.

Use of soft tooth brush to prevent gum

trauma

6.

Hard and sharp foods should be avoided

NURSING MANAGEMENT

1.

2.

PROVIDING EDUCATION AND

SUPPORT FOR FAMILY MEMBERS
Many parents feel guilty about their
childs disease
Parent education and support can help
them provide as normal life as possible
for their affected child

NURSING MANAGEMENT
4.

Repeated hospitalization can be prevented

if parents can provide factor replacement at
home

5.

Community support and resources should

be discussed with families of affected child

6.

Genetic counseling is needed to families