Hemophilia is a group of inherited bleeding disorders caused by deficiencies in clotting factors, leading to impaired blood clotting. There are several types of hemophilia including Hemophilia A, Hemophilia B, and von Willebrand's disease. Symptoms include bleeding into joints, easy bruising, and prolonged bleeding from minor injuries. Treatment involves replacing the deficient clotting factor through products like fresh frozen plasma or specific clotting factor concentrates. Nursing care focuses on preventing and treating bleeding episodes, avoiding risk factors, managing pain, and providing patient education.

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