Hemophilia

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 ) v on Willebrand’s Disease Hemophilia C

5. Phathophysiology Hemophilia A Deficiency of coagulation factor VIII Hemophilia B Deficiency of coagulation factor IX v on 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 Fresh frozen plasma (FFP) Replace all clotting factor except platelets For Hemophilia A Human blood serum

15. For Hemophilia B Factor IX concentrate (only when needed as can cause thrombosis) For Hemophilia C Fresh frozen plasma daily 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