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 Disease
...
Phathophysiology
 Hemophilia A
 Deficiency of coagulation factor VIII
 Hemophilia B
 Deficiency of coagulation factor ...
Clinical Manifestation
 Hemarthrosis
 Easy bruising and cutaneous hematoma
formation with minor trauma
 Bleeding from g...
 Spontaneous hematuria or epistaxis
 Pain or paralysis due to pressure
hematomas on nerves
 Intracranial hemorrhage
Investigation
 1) Blood Test
 Help the doctor to check for certain disease and
condition.
 It is also help to check the...
2) Coagulations Study
 (A) Partial Thromboplastin Time (PTT)
 PTT is the blood test that looks how long it takes for
blo...
 (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 sa...
 It is the test to measure how many platelet you
have in your blood. Platelet also can help the
blood clot.
 The size is...
 Factor viii deficiency or extrinsic ( protein ) is an
inherited disorder in which a lack of plasma protein.
 Factor vii...
 The procedure that use to diagnose fetal defects
in the early second trimester of pregnancy
 It is surrounds a fetus of...
Medical Treatment
1. Fresh frozen plasma (FFP)
 Replace all clotting factor except platelets
2. For Hemophilia A
 Human ...
3. For Hemophilia B
 Factor IX concentrate (only when needed as can
cause thrombosis)
4. For Hemophilia C
 Fresh frozen ...
Nursing Care Plan 1
Nursing Diagnosis
 Ineffective body protection related to lack of
clotting factor
Goal
 Increasing p...
 Asses patient body protection by taking CBC to
evaluate patient condition
 Instruct patient on bleeding precaution to p...
Nursing Care Plan 2
Nursing Diagnosis
 Risk of aspiration related to uncontrolled nose
bleeding.
Goal
 Reduce risk of as...
Nursing Intervention
 Asses patient nose bleeding to evaluate
patient condition
 Apply cold compress to reduce nose blee...
Nursing Care Plan 3
Nursing diagnosis
 Pain related bleeding into tissue
Goal
 Patient will verbalize that pain is relie...
 Asses patient pain by report the location,
intensity, and rate of pain (pain scale) to provide
caregiver with data for t...
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Hemophilia

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Hemophilia

  1. 1. HEMOPHILIA
  2. 2. Presenter
  3. 3. A group of inherited bleeding disorders in which ability of blood to clot is impaired. Definition
  4. 4. Type of Hemophilia  Hemophilia A ( Classic hemophilia )  Hemophilia B ( Christmas disease )  von Willebrand’s Disease  Hemophilia C
  5. 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. 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. 7.  Spontaneous hematuria or epistaxis  Pain or paralysis due to pressure hematomas on nerves  Intracranial hemorrhage
  8. 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. 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. 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. 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. 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. 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. 14. Medical Treatment 1. Fresh frozen plasma (FFP)  Replace all clotting factor except platelets 2. For Hemophilia A  Human blood serum
  15. 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. 16. Nursing Care Plan 1 Nursing Diagnosis  Ineffective body protection related to lack of clotting factor Goal  Increasing patient body protection
  17. 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. 18. Nursing Care Plan 2 Nursing Diagnosis  Risk of aspiration related to uncontrolled nose bleeding. Goal  Reduce risk of aspiration  Control nose bleeding
  19. 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. 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. 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

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