Telling you about hemophilia

1. HEMOPHILIA

2. Presenter

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

4. Type of Hemophilia
 Hemophilia A ( Classic hemophilia )
 Hemophilia B ( Christmas disease )
 von Willebrand’s Disease
 Hemophilia C

5. Phathophysiology
 Hemophilia A
 Deficiency of coagulation factor VIII
 Hemophilia B
 Deficiency of coagulation factor IX
 von Willebrand Disease
 Deficiency of von Willebrand protein
 Hemophilia C
 After 3 coagulation factor ( hemophilia A and B, von
Willebrand )

6. Clinical Manifestation
 Hemarthrosis
 Easy bruising and cutaneous hematoma
formation with minor trauma
 Bleeding from gums and prolonged bleeding
following minor injuries or cuts
 GIT bleeding with hematemesis, occult blood in
stools, gastric pain or abdominal pain

7.  Spontaneous hematuria or epistaxis
 Pain or paralysis due to pressure
hematomas on nerves
 Intracranial hemorrhage

8. 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.

9. 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.

10.  (B)Thrombin Time
 TT 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.

11.  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

12.  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

13.  The procedure that use to diagnose fetal defects
in the early second trimester of pregnancy
 It is surrounds a fetus of womb
 Performed on fetal cells found in the sample can
reveal the presence of many type of genetic
disorders.
5) Amniocentesis

14. Medical Treatment
1. Fresh frozen plasma (FFP)
 Replace all clotting factor except platelets
2. For Hemophilia A
 Human blood serum

15. 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

16. Nursing Care Plan 1
Nursing Diagnosis
 Ineffective body protection related to lack of
clotting factor
Goal
 Increasing patient body protection

17.  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

18. Nursing Care Plan 2
Nursing Diagnosis
 Risk of aspiration related to uncontrolled nose
bleeding.
Goal
 Reduce risk of aspiration
 Control nose bleeding

19. Nursing Intervention
 Asses patient nose bleeding to evaluate
patient condition
 Apply cold compress to reduce nose bleeding
 Avoid patient from expose with high
temperature to avoid nose bleeding
 Avoid 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.

20. Nursing Care Plan 3
Nursing diagnosis
 Pain related bleeding into tissue
Goal
 Patient will verbalize that pain is relieved to a
satisfactory level

21.  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