Deck 54: Drugs for Hemophilia

Question 1

A nurse is caring for a child who has hemophilia. While reviewing this child's immunization records,the nurse notes that the child needs the tetanus and reduced diphtheria toxoids and acellular pertussis (Tdap)vaccine and the hepatitis A vaccine. The nurse should notify the provider and obtain an order to give:

A) both vaccines intramuscularly.
B) both vaccines subcutaneously.
C) the hepatitis A vaccine only subcutaneously.
D) the Tdap vaccine only intramuscularly.

Answer 1

both vaccines intramuscularly.

Question 2

A child with hemophilia A who weighs 30 kg receives factor VIII on an ongoing basis as prophylaxis. The provider orders desmopressin [Stimate] to be used for nosebleeds. The nurse will instruct the child's parents to give the medication as ____ nostril as needed.

A) 1 spray in each
B) 1 spray in one
C) 2 sprays in each
D) 2 sprays in one

Answer 2

1 spray in one

Question 3

A nurse is caring for an African American patient with severe hemophilia A who has been admitted for bleeding into the knee joint. The prescriber has ordered intravenous factor VIIa [NovoSeven RT]. A nursing student wants to know why this patient is not receiving factor VIII. Which statement by the nurse is correct?

A) "Factor VIIa is stronger than factor VIII and will work faster."
B) "Factor VIII is used for prophylaxis, and factor VIIa is used for acute episodes."
C) "Factor VIIa is used when patients develop antibodies against factor VIII."
D) "Factor VIIa provides immune tolerance therapy so that factor VIII will be more effective."

Answer 3

"Factor VIIa is used when patients develop antibodies against factor VIII."

Question 4

A patient with hemophilia B is admitted to the hospital for surgery. The patient's history reveals long-term use of factor replacement as prophylaxis for bleeding episodes. The nurse anticipates that the provider will order which medication to prevent excessive perioperative bleeding?

A) Desmopressin
B) Factor VIII
C) Ibuprofen
D) Tranexamic acid

Question 5

A patient who is receiving a factor VIII concentrate to treat hemophilia A develops hives,a low-grade temperature elevation,and a stuffy nose. The nurse will prepare to:

A) administer subcutaneous epinephrine.
B) give an antipyretic medication.
C) give oral diphenhydramine.
D) provide respiratory support.

Question 6

The nurse is conducting a nursing education class on hemophilia. The nurse correctly states that which characteristics are most likely to be seen in a patient with hemophilia? (Select all that apply.)

A) Male gender
B) Female gender
C) Mother is a carrier.
D) All races and ethnicities are susceptible.
E) Both parents are carriers.

Question 7

A patient with epistaxis and a history of hemophilia A is admitted to the unit and is scheduled for replacement therapy. The nurse should prepare to administer which medication?

A) Tranexamic acid [Cyklokapron]
B) Aminocaproic acid [Amicar]
C) Desmopressin [Stimate]
D) Factor VIII

Question 8

A child with severe hemophilia A receives factor VIII. The nurse expects this drug to be given:

A) on demand when bleeding occurs.
B) on demand when plasma factor VIII activity is less than 1% above normal.
C) prophylactically once weekly to maintain normal factor VIII activity.
D) prophylactically three times weekly to maintain factor VIII activity above 1% of normal.

Question 9

A patient with hemophilia A is undergoing a tooth extraction at the dentist's office. As an adjunct to factor VIII,what other drug should the nurse anticipate being ordered for this patient?

A) Tranexamic acid [Cyklokapron]
B) Acetaminophen [Tylenol]
C) Desmopressin [Stimate]
D) Vitamin K

Question 10

A child with moderate hemophilia A who weighs 20 kg is admitted to the hospital after a knee injury with bleeding into the joint. The nurse will expect the provider to order which medication?

A) Advate 400 units IV over 10 minutes
B) Benefix 800 units slow IV push
C) Desmopressin 6 mcg IV over 15 to 30 minutes
D) Tranexamic acid [Cyklokapron] 200 mg IV

Question 11

A patient with hemophilia is hospitalized for infusion of factor VIII replacement through a venous port. While giving the drug,the nurse notes that the patient's temperature is 101.5°F. The nurse will contact the provider to report which possibility?

A) Anaphylactic reaction
B) Contamination of factor replacement
C) Port infection
D) Thrombolytic event

Question 12

The parents of a boy with hemophilia A want to know why their son will receive factor replacement on an ongoing basis rather than when needed for bleeding episodes. They tell the nurse that the boy's grandfather,who had the same disease,received the drug only when he had bleeding. The nurse will give the parents which information about ongoing therapy?

A) It can reverse progression of the disease.
B) It is cheaper, because the amounts of the drug used are less.
C) It is used to minimize long-term damage to the joints.
D) It prevents the development of inhibitors.