Chapter 54: Drugs for Hemophilia

Chapter 54: Drugs for Hemophilia

 

Complete Chapter Questions With Answers

 

Sample Questions Are Posted Below

 

MULTIPLE CHOICE

 

1. 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

 

 

ANS:  D

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.

 

PTS:   1                    DIF:    Cognitive Level: Application           REF:   pp. 633-634

TOP:   Nursing Process: Planning

MSC:  NCLEX Client Needs Category: Physiologic Integrity: Pharmacologic and Parenteral Therapies

 

2. 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.

 

 

ANS:  C

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.

 

PTS:   1                    DIF:    Cognitive Level: Application           REF:   pp. 633-634

TOP:   Nursing Process: Implementation

MSC:  NCLEX Client Needs Category: Physiologic Integrity: Pharmacologic and Parenteral Therapies

 

3. 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

 

 

ANS:  D

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.

 

PTS:   1                    DIF:    Cognitive Level: Application           REF:   pp. 633-634

TOP:   Nursing Process: Planning

MSC:  NCLEX Client Needs Category: Physiologic Integrity: Reduction of Risk Potential

 

4. 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.

 

 

ANS:  A

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.

 

PTS:   1                    DIF:    Cognitive Level: Application           REF:   p. 634

TOP:   Nursing Process: Planning

MSC:  NCLEX Client Needs Category: Physiologic Integrity: Reduction of Risk Potential