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
1.Hemophilia A
2.Hemophilia B
3.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;
1.minor
2.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-
1.acute pain related to traumatic injury to the muscle,
2.impaired physical injury related to pain & discomfort,
3.risk for bleeding related to decreased concentration of clotting
factor circulation of in the blood,
4.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.