HEMOPHILIA

Presenter

A group of inherited bleeding disorders in which ability of blood to clot is impaired.

Definition

Type of Hemophilia

Hemophilia A ( Classic hemophilia )Hemophilia B ( Christmas disease )

von Willebrand’s DiseaseHemophilia C

Phathophysiology Hemophilia A

Deficiency of coagulation factor VIII

Hemophilia BDeficiency of coagulation factor IX

von Willebrand DiseaseDeficiency of von Willebrand protein

Hemophilia CAfter 3 coagulation factor ( hemophilia A and

B, von Willebrand )

Clinical ManifestationHemarthrosisEasy bruising and cutaneous hematoma

formation with minor traumaBleeding from gums and prolonged bleeding

following minor injuries or cutsGIT bleeding with hematemesis, occult blood in

stools, gastric pain or abdominal pain

Spontaneous hematuria or epistaxisPain or paralysis due to pressure

hematomas on nervesIntracranial hemorrhage

Investigation 1) Blood Test

Help the doctor to check for certain disease and condition.

It is also help to check the function of the organs and show how the treatment are working.

2) Coagulations Study

(A) Partial Thromboplastin Time (PTT)PTT is the blood test that looks how long it takes

for blood clot. It can help to tell if you have bleeding or clotting problem.

If the patient in heparin medication, you will be watched for sign of bleeding.

(B)Thrombin TimeTT is the blood test which measures the time it take for a

clot to form in the plasma from a blood sample in anticoagulant which had added an excess of thrombin.

This test repeated with pooled plasma from normal patient. Different in time between the test and the normal indicates an abnormality in the conversion of fibrogen.

It is the test to measure how many platelet you have in your blood. Platelet also can help the blood clot.

The size is smaller than red and white blood cell.

Normal blood platelet counts should be between 150-200000.

3) Serum Platelet Level

Factor viii deficiency or extrinsic ( protein ) is an inherited disorder in which a lack of plasma protein.

Factor viii leads to abnormal bleeding and it occurs when the body does not have enough of factor vii.

It is very important blood clotting protein.

4) Factor Assay

The procedure that use to diagnose fetal defects in the early second trimester of pregnancy

It is surrounds a fetus of wombPerformed on fetal cells found in the sample can

reveal the presence of many type of genetic disorders.

5) Amniocentesis

Medical Treatment1. Fresh frozen plasma (FFP)

Replace all clotting factor except platelets

2. For Hemophilia A Human blood serum

3. For Hemophilia B Factor IX concentrate (only when needed as can

cause thrombosis)

4. For Hemophilia C Fresh frozen plasma daily

5. For Von Willebrand’s disease Factor VIII concentrate

Aspirin is avoided in Hemophilia

Nursing Care Plan 1

Nursing DiagnosisIneffective body protection related to lack

of clotting factor

GoalIncreasing patient body protection

Asses patient body protection by taking CBC to evaluate patient condition

Instruct patient on bleeding precaution to promote early intervention to prevent injury

Assist with administration of factor concentration, fresh frozen plasma, cryoprecipitate or blood to treat acute of bleeding.

If bleeding, apply cold compress at bleeding site to help slow bleeding

Avoid any route of injection ( IM, IV, Subcutaneous ) or rectal medication that cause bleeding into tissue

Nursing Care Plan 2

Nursing DiagnosisRisk of aspiration related to uncontrolled

nose bleeding.

GoalReduce risk of aspirationControl nose bleeding

Nursing InterventionAsses patient nose bleeding to evaluate

patient conditionApply cold compress to reduce nose

bleedingAvoid patient from expose with high

temperature to avoid nose bleedingAvoid patient in doing major surgery to

avoid excessive bleeding (aspiration)Replace clotting factor and blood

product to increase patient blood clotting.

Avoid all anticoagulant medication ( Heparin, Aspirin )to control excessive bleeding.

Nursing Care Plan 3

Nursing diagnosisPain related bleeding into tissue

GoalPatient will verbalize that pain is relieved to

a satisfactory level

Asses patient pain by report the location, intensity, and rate of pain (pain scale) to provide caregiver with data for treatment plan.

Administer opiod (morphine) as prescribe to control pain from severe to moderate.

Avoid IM injection because the risk of bleeding into the muscle which can cause more pain

Reassess the level of pain within 1 hour after administer opiod to determine the effectiveness of treatment ordered.

Monitor sedation and respiratory status of the patient receiving opiod of pain because opiod can cause depress respiratory center of the brain