It is a congenital coagulation disorder leading to severe bleeding.
Types of hemophilia:
- Hemophilia A due to deficiency of factor 8.
- Hemophilia B due to deficiency of factor 9.
Mode of inheritance: as sex linked recessive disorder, however spontaneous mutation may have occurred.
In children bleeding frequently occur into the joint(haemoarthrosis) which is
extremely painful and its management include:
- Sedation or narcotic.
- Immobilization with splint or traction.
- Application of cold sponge to the affected part.
- Physiotherapy with careful handling to prevent bleeding.
- Replacement therapy to increase the level of factor 8,plasma can be given also or blood transfusion.
Responsibility of the nurse:
- Teach the parents and patient how to administer factor 8 at home.
- Health education about the disease including:
methods of preventing injuries that cause bleeding.
careful handling of the child is necessary.
medication is given orally, however if injection is necessary the site is carefully chosen and rotated, the medication is injected slowly and pressure is applied for at least 5 minutes.
if blood is to be drawn superficial veins are used.
the child’s toys must be soft.
the child must be protected from falling.
if removal of teeth becomes necessary the child is hospitalized for the procedure.