Hemophilia Academy: Hemophilia A Factor VIII Treatment Methods

Question 1

1. Which of the following statements regarding the impact of bleeds in hemophilia A is FALSE?

a. The majority of patients are effectively treated with on-demand therapy, which has been shown to reduce bleeding episodes.
b. If bleeds are not prevented, consequences may include life-threatening bleeds.
c. Bleeds may result in pain and effects on day-to-day activities.
d. The aim of hemophilia treatment should be to prevent and manage bleeding by replacing the deficient clotting factor.

Answer 1

a. The majority of patients are effectively treated with on-demand therapy, which has been shown to reduce bleeding episodes.

Question 2

2. Correctly complete the following sentence. The primary aim of hemophilia care is to prevent and manage bleeding by:

a. Preventing the breakdown of fibrin
b. Replacing the deficient clotting factor
c. Bypassing the need for the deficient clotting factor
d. Replacing the gene for the deficient clotting factor

Answer 2

Replacing the deficient clotting factor

Question 3

3. Correctly complete the following sentence. The optimal treatment protocol for hemophilia A treatment is:

a. On-demand treatment, because it always prevents unnecessary bleeding
b. Prophylaxis, because it reverses joint damage
c. On-demand treatment, because it is the most effective way to reduce bleeding
d. Prophylaxis, because it can help prevent and reduce bleeding, including joint bleeds

Answer 3

Prophylaxis, because it can help prevent and reduce bleeding, including joint bleeds

Question 4

If some bleeds are not prevented, consequences may include which of the following? Select all that apply.

a. Life-threatening bleeds
b. Improved range of motion
c. Pain
d. Effects on day-to-day activities

Answer 4

a. Life-threatening bleeds
c. Pain
d. Effects on day-to-day activities

Question 5

5. Which of the following statements about factor VIII (FVIII) replacement treatment protocols is FALSE?

a. On-demand treatment is when FVIII is given at the time of clinically evident bleeding.
b. Prophylaxis is when FVIII is given regularly to help prevent anticipated bleeding.
c. Standard prophylaxis dosage is based on body mass index (BMI).
d. Pharmacokinetic (PK)-guided prophylaxis dosage is based on the way a patient’s body handles FVIII.

Answer 5

c. Standard prophylaxis dosage is based on body mass index (BMI).

Question 6

6. Correctly complete the following sentence. Primary prophylaxis is:

a. Started after ≥2 large joint bleeds and before the onset of joint disease.
b. Initiated without evidence of joint disease and started before any large joint bleeds and before the age of 1 year.
c. Initiated without evidence of joint disease and started before the second large joint bleed and before the age of 3 years.
d. Started after ≥5 large joint bleeds and after the onset of joint disease.

Answer 6

c. Initiated without evidence of joint disease and started before the second large joint bleed and before the age of 3 years.

Question 7

7. All of the following statements about the benefits of prophylaxis dosing are correct EXCEPT:

a. Prophylaxis can help prevent bleeds.
b. Prophylaxis can help reduce bleeds.
c. Prophylaxis can achieve a lower frequency of joint bleeds.
d. Prophylaxis reverses joint damage.

Answer 7

d. Prophylaxis reverses joint damage.

Question 8

8. Correctly complete the following sentence. The annualized bleeding rate (ABR) can be used:

a. To monitor the effectiveness of on-demand treatment.
b. To quantify primary endpoints in clinical studies.
c. For early detection of hemarthrosis.
d. In the diagnosis of joint disease in hemophilia patients.

Answer 8

b. To quantify primary endpoints in clinical studies.

Question 9

9. According to the Medical and Scientific Advisory Council (MASAC), which of the following is the treatment of choice for hemophilia A patients?
   a. Desmopressin
   b. Fresh frozen plasma
   c. Plasma-derived factor VIII
   d. Recombinant factor VIII

Answer 9

d. Recombinant factor VIII

Question 10

10. Which of the following are methods used to monitor hemophilia treatment? Select all that apply.
    a. Hemophilia Joint Health Score (HJHS)
    b. Monthly blood volume loss
    c. CT scans
    d. Annualized bleeding rate (ABR)

Answer 10

a. Hemophilia Joint Health Score (HJHS)

d. Annualized bleeding rate (ABR)