Hemophilia

Question 1

General Info

Answer 1

Usually inherited
18,000 people have this disorder, 400 babies are born annually- is an x-linked disorder (with rare exceptions)
Have little or no clotting factor
Two types-
Hemophilia A- clotting factor 8 deficiency ( 90% have this type)- 70% have severe form
Hemophilia B- clotting factor 9 deficiency

Question 2

Signs & Symptoms

Answer 2

Excessive bleeding after a dental procedure, epistaxis for no reason, excessive bleeding from a minor cut, cuts that quit bleeding then restart
Internal bleeding (hematuria or GI)
Bleeding in the joints- tightness of joint, decreased ROM, swollen, hot, severe pain
Intracranial bleeds after bumps or head trauma- headaches, vomiting, changes in behavior, vision changes, seizures

Question 3

Dx

Answer 3

Mild- 5-30 % of normal clotting factor
Moderate- 1-5% of normal clotting factor
Severe- <1% of normal clotting factor
CBC and bleeding times
Referral, referral, referral

Question 4

Tx

Answer 4

Once established which clotting factor is deficient it can be replaced
Replaced IV in about 15 min at home when necessary
Concentrates of clotting factor made from human blood (Screened carefully for HPV & others) but also now have recombinant clotting factors
Can develop antibodies to the clotting factor

Question 5

Other Tx

Answer 5

DDAVP- stimulates release of stored factor VIII
Not used to treat B or severe A
Usually used prior to dental appts or sporting events that the pt is a participant

Question 6

Summary of Tx
Mild
Moderate
Severe

Answer 6

Mild hemophilia—Replacement therapy usually isn’t needed for mild hemophilia. But DDAVP sometimes is given to raise the body’s level of factor VIII.
Moderate hemophilia—May need replacement therapy only when bleeding occurs or to prevent bleeding that could occur when doing certain activities. May recommend DDAVP prior to having a procedure or doing an activity that increases the risk of bleeding.
Severe hemophilia—Usually need replacement therapy to prevent bleeding that could cause permanent damage to joints, muscles, or other parts of the body. Typically, replacement therapy is given at home two or three times a week. This preventive therapy usually is started in patients at a young age and may need to continue for life.