Hemophilia

Hemophilia

1. Hemophilia

2. Introduction haemophilia is a disease that causes problems with blood clotting.it makes people's blood clot much more slowly than usual . when blood does not clot properly it can lead to bleeding inside the body too-For instance following a fall or a crush injury

3. Definition haemophilia is a inherited disorder in which blood does not clot normally

4. Incidence Haemophilia A affect 1in 5,000 male births . About 400 babies are born with haemophilia A each year . haemophilia A is about 4 times as common as haemophilia B . The number of people with haemophilia in the United States is estimated to be about 20,000 individual.

5. types Hemophilia A Hemophilia B Hemophilia C

6. CONTI… Haemophilia A caused by a lack of the blood clotting factor III .Approximately 80%of haemophilia have A type

7. CONTI… Haemophilia B caused by a deficiency of clotting factor IX

8. CONTI… Haemophilia C caused by lack of clotting factor XI

9. Causes mutation or change in one of the genes that provides instructions for making the clotting factor proteins needed to form a blood clot.

10. Risk factor family history male sex malignancy pregnancy auto immune disorder (rheumatoid arthritis ) infections ( hepatitis,AIDS ) drugs dermatological conditions (psoriasis)

11. Pathophysiology

12. Clinical Manifestation large or deep bruises , joint pain & swelling, blood in urine blood in stool nose bleeds heavy or prolonged periods , internal bleeding

13. Diagnostic Evaluation screening tests, complete blood test (CBC), Activated Partial Thromboplastin Time Test(APTT ), Prothrombin Time test(PT ), fibrinogen test

14. CONTI… Activated Partial Thromboplastin Test This test measures how long it takes for blood to clot . The result of this will show a longer clotting time among people with haemophilia A or B

15. CONTI… Prothrombin Time Test This test also measures the time it takes for blood to clot. The results of this test will be normal among most people with haemophilia A or B.

16. CONTI… Fibrinogen Test This test also helps doctors assess a patients ability to form a blood clot

17. MANAGEMENT Pharmacological management plasma derived factor concentrates , Recombinant factor concentrates , Emici zuman , desmopressin , acetate , amicar , cryoprecipitate

18. CONTI… Non pharmacological management clot-preserving medication fibrin sealants physical therapy first aid for minor cuts vaccination

19. Surgical management this can be 2types of surgeries ; minor major surgeries

20. CONTI… 1. Major joint replacement ,spinal , cardio vascular, abdominal and intra cranial surgery 2. Minor dental procedure, Endoscopy/colonoscopy, removal of skin lesion, Arthroscopy

21. Nursing management Nursing assessment history, physical examination

22. CONTI… Nursing diagnosis- acute pain related to traumatic injury to the muscle , impaired physical injury related to pain & discomfort , risk for bleeding related to decreased concentration of clotting factor circulation of in the blood , risk for injury related to decreased clotting factor

23. CONTI… Nursing intervention relieve pain, maintain optimal physical mobility assist in the coping of the family prevent bleeding , present injury

24. Treatment The best way to treat haemophilia is to replace the missing blood clotting factor ,so that the blood can clot properly . This is typically done by injecting treatment products called clotting factor concentrates into a person's vein.

25. Patient Teaching exercise regularly , avoid certain pain medication , avoid blood -thinning medication , practice good dental hygiene .

26.   Complications deep internal bleeding damage to joint infection adverse reaction to clotting factor treatment

27. CONCLUSION Haemophilia should be suspected in patients with a history of easy bruising in early childhood, spontaneous bleeding, particularly into the joints, muscles, and soft tissues, or prolonged bleeding following trauma or surgery .