Question 1

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?&#10;A) Advate 400 units IV over 10 minutes&#10;B) Benefix 800 units slow IV push&#10;C) Desmopressin 6 mcg IV over 15 to 30 minutes&#10;D) Tranexamic acid (Cyklokapron) 200 mg IV

Accepted Answer

Advate is a third-generation product for factor VIII replacement, which this child needs. Third-generation products are safer, because they are not exposed to BSA or HAS during production; therefore, this is the product of choice. Benefix is a factor IX replacement product and is not indicated for hemophilia A. Desmopressin is used to stop bleeding in patients with mild hemophilia. Tranexamic acid is used as adjunctive therapy.

Question 2

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

Port infection

Accepted Answer

Central venous access devices are used to facilitate frequent IV administration of factor replacement but pose the risks of infection and thrombosis. This patient has a fever, which is a symptom of infection. Anaphylactic reactions to factor replacement can occur but are characterized by wheezing and shortness of breath, along with swelling in the face. Viral contamination of factor replacement is rare with factor VIII. Thrombolytic events would have symptoms associated with clot formation in certain organs and are not associated with fever.

Question 3

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?&#10;A) Tranexamic acid (Cyklokapron)&#10;B) Aminocaproic acid (Amicar)&#10;C) Desmopressin (Stimate)&#10;D) Factor VIII

Accepted Answer

The cornerstone of treatment for hemophilia A is replacement therapy with factor VIII. Tranexamic acid and aminocaproic acid are antifibrinolytic agents that act primarily by preventing the formation of plasmin from its precursor. Desmopressin promotes the release of factor VIII from the vascular endothelium and has the advantage of being cheaper than factor VIII. Also, it can be administered by nasal spray or by IV infusion. However, repeated use of desmopressin can deplete stored factor VIII.

Question 4

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

Accepted Answer

Children who weigh less than 50 kg should use 1 spray in one nostril for a dose of 150 mcg. Each spray delivers 150 mcg, so 1 spray in both nostrils would give a dose of 300 mcg. Using 2 sprays in one nostril would give a dose of 300 mcg; using 2 sprays in both nostrils would give a dose of 600 mcg.

Question 5

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

Accepted Answer

Antifibrinolytic drugs, such as tranexamic acid, which prevent the breakdown of fibrin, are used as adjuncts to replacement factor in special cases in which bleeding is likely. Desmopressin is used in patients with mild hemophilia A to help control bleeding episodes. Hemophilia B is a deficiency of factor IX, so giving factor VIII would not be appropriate. Ibuprofen would only reduce clotting and should not be used.

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.)&#10;A) Male gender&#10;B) Female gender&#10;C) Mother is a carrier.&#10;D) All races and ethnicities are susceptible.&#10;E) Both parents are carriers.

Accepted Answer

Hemophilia is more common in males. The female carries this sex-linked recessive gene. The risk of acquiring hemophilia is shared by all races and ethnic groups. Females typically are carriers but do not have hemophilia. Both parents do not have to carry the gene for their children to inherit the disease.

Question 7

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?&#10;A) Tranexamic acid (Cyklokapron)&#10;B) Acetaminophen (Tylenol)&#10;C) Desmopressin (Stimate)&#10;D) Vitamin K

Accepted Answer

Antifibrinolytic drugs (e.g., aminocaproic acid and tranexamic acid) can be used as adjuncts to factor VIII and factor IX in special situations, such as a tooth extraction. Acetaminophen is indicated for mild pain. Desmopressin is used as replacement therapy but is not used specifically for tooth extractions. Vitamin K is not indicated in this situation.

Question 8

A nurse is caring for a child with 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.

Accepted Answer

To minimize the risk of hepatitis, all patients with hemophilia should be fully vaccinated, and all newly diagnosed patients should receive both the hepatitis A and hepatitis B vaccines to minimize the risk of hepatitis from infusions. Clinicians should inject vaccines intramuscularly, taking precautions to prevent excessive bleeding, because subcutaneous administration does not guarantee efficacy. There is no reason to withhold any vaccine in children with hemophilia.

Question 9

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 pathologic developments in the joints.
D) It prevents the development of inhibitors.

Accepted Answer

Primary prophylaxis, especially in young children, minimizes bleeding episodes and long-term damage to joints. Ongoing treatment does not reverse the progression of the disease. Ongoing therapy is not less expensive, nor does it prevent the development of inhibitors.

Question 10

A nurse is caring for a patient with 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."

Accepted Answer

When patients develop antibodies against factor VIII, activated factor VII is used, because it has the same actions as factor VIII. Factor VIIa is not stronger than factor VIII. It is not used for prophylaxis only. Immune tolerance therapy describes the use of repeated administration of factor replacement products to eliminate inhibitor production.

Deck 54: Drugs for Hemophilia