Hemophilia

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

Hemophilia

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HEMOPHILIA

Hemophilia refers to a group of bleeding

It is congenital bleeding disorder caused

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

Hemophilia C is an autosomal dominant

Von willebrands disease is a dominant

Hemophilia A (classic hemophilia) due to

Hemophilia A (Christmas hemophilia) due

Hemophilia C due to deficiency of factor IX

Hageman factor deficiency caused by lack

Von willebrand or vascular hemophilia

AHF produced by liver

Formation of thromboplastin for blood

Hemophilic patient have 2 of the 3 factors

They bleed longer period but not at faster

Bleeding into subcetaneous, intramuscular

Prolonged bleeding after minor surgery

Prolonged and frequent bleeding

Bleeding into joints cavities

Subcutaneous and intramuscular hemorrhage

Frequent bleeding in elbows, knees, and

Spontaneous joint bleeding or as a result

Chronic pain due to repeated episodes of

Decreased joint motion

Joint degeneration leading to osteoporosis or

Genetic history, Clinical symptoms

Lab diagnosis CBC, platelet function test,

Prolonged partial thromboplastin time (PTT)

Functional assays measure the amount of

Preventing and controlling bleeding are

Replacement reparation of the factor VIII

One unite of plasma (250ml) provides one

Fresh or frozen plasma can also be used to

Every milliliter of plasma contains 1 unite of

Transfusion have been associated with

NSAIDs for pain caused by synovitis but used

Corticosteroids for hematuria,

Preventing and controlling bleeding

finger nails should be kept short, since a

Diaper pins should not be used or used with

Infant should be provided with soft toys

Use of soft tooth brush to prevent gum

Hard and sharp foods should be avoided

PROVIDING EDUCATION AND

Repeated hospitalization can be prevented

Community support and resources should

Genetic counseling is needed to families

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