Hemophilia is a rare bleeding disorder
In minor disease factor level should be raised to 20 % - 30% .
In severe bleeding , it raised at least 50% . For the major surgery , it raised to 100 % . preoperatively and maintained above 50% until healing .
To prevent secondary hemorrhage , maintain 10 -25% of normal by 10 -5 ml/ kg / 12 h .
Intravenous infusion of factor VIII concentrate /FFP / cryoprecipitate .
- T/2 is about 12 h ( at least twice daily )
> 30 ml / kg within 24 hrs course circulatory overload .
- 1ml of plasma contain 1u of factor VIII
- stored in domestic refrigerator at 4 *c
- immediately after bleeding starts to infuse
To severely affected patients, give prophylaxis 3/52 time
Recombinant factor VIII concentrated
- reduced risk of transmission of infection
- safe - but limited supply
- effective - high cost
- - similar incidence of antibodies development
Synthetic vasopressin ( DDAVP ) IV , S.C. or intra nasal
- It avoid complication associated with blood products
- to mild cases
- as prophylaxis before miner surgery
Never give aspirin to the patient
physiotherapy give to pressure muscle strength and avoid damage from immobilization
Social and psychological support .
Good dental care
Avoid IM injection
Each person with hemophilia carries a medical card which gives details of the disorder and its treatment .
Factor viii 1u /kg increase 2 % of factor level
15u/kg increase 30% ( haemostasis in joint bleeding )
30u/kg increase 60% ( required in ICH and to cover the surgery )
tranexamic acid is given in mucosal bleeding .
should not used in haematuria .