MEDINA COLLEGE

Ozamiz City

College of Nursing

In Partial Fulfillment Of
The Requirements in
Pediatric nursing ll

Submitted by:

Madrio, Stephanne S.
Lungay, Ginalyn B.
BSN-II/BLOCK A

Submitted to:
Mr. Sherwin L. Cabanlet, RN, MN
CLINICAL INSTRUCTOR

Date Submitted: February 25, 2010

 HEMOPHILIA
DESCRIPTION

Hemophilia is a rare bleeding disorder in which your blood doesn't clot

normally.
There are two main types of hemophilia – Hemophilia A (due to factor VIII
deficiency) and Hemophilia B (due to factor IX deficiency). They are clinically
almost identical and are associated with spontaneous bleeding into joints
and muscles and internal or external bleeding after injury or surgery.

Other Types:

In hemophilia, certain clotting factors are either decreased in quantity, absent, or

improperly formed. Because the clotting cascade uses amplification to
rapidly plug up a bleeding area, absence or inactivity of just one clotting
factor can greatly increase bleeding time.
Hemophilia A is the most common type of bleeding disorder and involves
decreased activity of factor VIII. There are three levels of factor VIII
deficiency: severe, moderate, and mild. This classification is based on the
percentage of normal factor VIII activity present:

• severe hemophilia Individuals with less than 1% of normal factor VIII activity level. Half of
all people with hemophilia A fall into this category. Such individuals frequently experience
spontaneous bleeding, most frequently into their joints, skin, and muscles. Surgery or trauma
can result in life-threatening hemorrhage, and must be carefully managed.
• moderate hemophilia Individuals with 1–5% of normal factor VIII activity level, and are at
risk for heavy bleeding after seemingly minor traumatic injury.
• mild hemophilia Individuals with 5–40% of normal factor VIII activity level, and must
prepare carefully for any surgery or dental procedures.

Individuals with hemophilia B have symptoms very similar to those of hemophilia A, but the deficient
factor is factor IX. This type of hemophilia is also known as Christmas disease.

Hemophilia C is very rare, and much more mild than hemophilia A or B; it involves factor XI.

Hemophilia A affects between one in 5,000 to one in 10,000 males in most populations.

ASSESSMENT

Prolonged Bleeding
Hemorrhage from any trauma

-loss of decidous teeth, circumcision, cuts, epistaxis, injections

 Excessive Bruising
Subcutaneous and Intramuscular Hemorrhages
Hemarthrosis especially the knees, ankles and elbows
Hematomas –pain , swelling and limited motion
Spontaneous hematuria
Bleeding in other mucous membranes such as nasal passages.

PATHOPHYSIOLOGY
Definition

Any of several hereditary blood-coagulation disorders in which the blood fails to

clot normally because of a deficiency or abnormality of one of the clotting
factors

Etiology: Hemphilia is an X-linked recessive disorder transmitted by

females and found predominantly in males. It may also caused by gene
mutation.

Risk Factors

• Family members with hemophilia

• Family history of bleeding disorders
• Sex: male
• Race Hemophilia affects all racial groups.

C. Schematic Diagram

X-linked recessive disorder transmitted by females

deficiency of, or a defect in, factor VIII (antihemophilic factor

[AHF])

or defect or deficiency of factor IX.

Clotting factor malfunction Sx: bleeding

HEMOPHILIA (A OR B)
MEDICAL MANAGEMENT
Lab/ Diagnostic Test

blood tests  to determine if clotting factors are missing or at low levels, and
which ones are causing the problem.
Genetic Tests  Analyzing DNA to look for a genetic alteration that may indicate an
increased risk for developing a specific disease or disorder.
Prenatal testing - if a pregnant woman has a history of hemophilia, a hemophilia
gene test can be done during pregnancy. A sample of placenta is removed
from the uterus and tested. This test is known as a CVS (chorionic villus
sampling) test.

Drug Regimen

Acetaminophen  sold under the brand names Tempra and Tylenol, is

recommended as a safe pain reliever for people with hemophilia.

Note: Never take any product with aspirin, or acetylsalicylic

acid, in it like Alka-Seltzer, Ibuprofen, such as Advil, Aleve and
Motrin . ‫ﻆ‬Aspirin can interfere with clotting

Antifibrinolytic medicines (including tranexamic acid and aminocaproic acid)

may be used with replacement therapy. They're usually given as a pill, and they
help keep blood clots from breaking down.
Desmopressin (DDAVP) Mild hemophilia may be treated with infusion of
these medications. They cause the release of blood factor concentrate. The
factor is stored within the body on the lining of blood vessels.
Immunization: The hepatitis B vaccine is important. There is an increased
risk of exposure to hepatitis with frequent infusions of blood products.
Estrogen-progesterone  in high doses (10 mg/day), they may raise plasma
levels of factor VIII and IX after several weeks and also reduce endometrial
proliferation. They are given to women with factor VIII deficiency to treat
menorrhagia or to raise factor levels before surgery.

Surgical Management

Certain types of surgery may become necessary, including:

• Joint replacement
• Removal of an uncontrollable, expanding hematoma (partially
clotted blood under the skin that resembles a bruise)
NURSING MANAGEMENT
Nursing Diagnosis

1. Parental health-seeking behaviors related to strategies for protecting

the child from injury.

Help parents set appropriate limits like infants should have his crib sides
padded, all toys need to be inspected for sharp edges or parts.

R: to prevent from injury.

Parents and the child as soon as he is approximately 10 yrs old, can be taught
to administer a replacement factor intravenously combined with
immobilization of the injured extremity and an ice pack applied locally. R: to
prevent bleeding immediately after injury.
Pressure shoud be applied to a laceration. R: to halt bleeding directly. Note:
suturing of lacerations is avoided whenever possible because the sutures
make additional puncture sites that may bleed.

2. Ineffective Protection related to abnormal blood profile.

Maintain a safe environment. Keep all necessary object within patients reach
and keep bed in low position. R: reduces accidental injury which could result
in bleeding
Avoid injections, rectal temperatures or rectal tubes. R: protect patient from
procedures related causes of bleeding
Avoid use of aspirin products or NSAIDs. R: these medication reduces platelet
aggregation.

3. Risk for interrupted family processes related to fears regarding childs

prognosis and long-term nature of illness.

Give them a chance to talk about how the bleeding began. R: for parents to
allow the child to lead a normal life
Remind them that it is impossible to prevent all injuries
Assist them with measures that offer them a sense of control over the situation.

HEALTH TEACHINGS

Identify signs of hemarthrosis and teach parents how to immobilize the joint,
pack it in ice, and administer replacement factor.
Assess home safety and teach about injury prevention. Consider the child’s
developmental level to ask specific safety questions.
Recommend using a soft toothbrush and point out the need for regular dental
checkups
Provide family about diet information of the patient because weight increase
can impose further stress on joints.
Teach the child and family that superficial injuries are treated with ice and
pressure.

PROGNOSIS

Good. Near normal lifestyle with treatment, but with need to

avoid injury. Advances in treatment over the last three decades
 have permitted a near-normal lifestyle and life-span for many
individuals with hemophilia

REFERENCES:

Essentials of Pediatric Nursing-wong

Medical Surgical Book
www.medicalnewstoday.com
www.ucsfhealth.org
nursingcrib.com