C HAPTER 12: S ICKLE C ELL A NEMIA
1. How should the nurse respond when asked by the mother of a child with beta-
thalassemia why the child is receiving deferoxamine?
a. To improve the anemia.
b. To decrease hepatosplenomegaly.
c. To prevent organ damage.
d. To prepare your child for a bone marrow transplant.
ANS: C
Multiple transfusions result in hemosiderosis. Deferoxamine is given to
chelate iron and prevent organ damage and complications from repeated
transfusions. Preparation for a bone marrow transplant would not include
administration of deferoxamine.
2. The nurse is caring for a child with aplastic anemia. Which of the following
nursing diagnoses would be appropriate? Select all that apply.
a. Acute pain related to vaso-occlusion
b. Risk for infection related to inadequate secondary defenses or
immunosuppression
c. Ineffective protection related to thrombocytopenia
d. Ineffective tissue perfusion related to anemia
ANS: B, C, D
Risk for infection, ineffective protection, and ineffective tissue perfusion are
appropriate nursing diagnoses for the nurse planning care for a child with
aplastic anemia. Aplastic anemia is a condition in which the bone marrow
, ceases production of the cells it normally manufactures, resulting in
pancytopenia. The child will have varying degrees of the disease depending
on how low the values are for absolute neutrophil count (affecting the bodys
response to infection), platelet count (putting the child at risk for bleeding),
and absolute reticulocyte count (causing the child to have anemia). Acute pain
related to vaso-occlusion is an appropriate nursing diagnosis for sickl e cell
anemia for the child in vaso-occlusive crisis, but it is not applicable to a child
with aplastic anemia.
3. A nurse is teaching home care instructions to parents of a child with sickle cell
disease. Which instructions should the nurse include? Select all that apply.
a. Limit fluid intake.
b. Administer aspirin for fever.
c. Administer penicillin as ordered.
d. Avoid cold and extreme heat.
e. Provide for adequate rest periods.
ANS: C, D, E
Parents should be taught to avoid cold, which can increase sickling, and
extreme heat, which can cause dehydration. Adequate rest periods should be
provided. Penicillin should be administered daily as ordered. The use of
aspirin should be avoided; acetaminophen or ibuprofen should be used as an
alternative. Fluids should be encouraged and an increase in fluid intake is
encouraged in hot weather or when there are other risks for dehydration.
4. Which is true about the genetic transmission of sickle cell disease?
a. Both parents must carry the sickle cell trait.
b. Both parents must have sickle cell disease.
c. One parent must have the sickle cell trait.
d. Sickle cell disease has no known pattern of inheritance.
1. How should the nurse respond when asked by the mother of a child with beta-
thalassemia why the child is receiving deferoxamine?
a. To improve the anemia.
b. To decrease hepatosplenomegaly.
c. To prevent organ damage.
d. To prepare your child for a bone marrow transplant.
ANS: C
Multiple transfusions result in hemosiderosis. Deferoxamine is given to
chelate iron and prevent organ damage and complications from repeated
transfusions. Preparation for a bone marrow transplant would not include
administration of deferoxamine.
2. The nurse is caring for a child with aplastic anemia. Which of the following
nursing diagnoses would be appropriate? Select all that apply.
a. Acute pain related to vaso-occlusion
b. Risk for infection related to inadequate secondary defenses or
immunosuppression
c. Ineffective protection related to thrombocytopenia
d. Ineffective tissue perfusion related to anemia
ANS: B, C, D
Risk for infection, ineffective protection, and ineffective tissue perfusion are
appropriate nursing diagnoses for the nurse planning care for a child with
aplastic anemia. Aplastic anemia is a condition in which the bone marrow
, ceases production of the cells it normally manufactures, resulting in
pancytopenia. The child will have varying degrees of the disease depending
on how low the values are for absolute neutrophil count (affecting the bodys
response to infection), platelet count (putting the child at risk for bleeding),
and absolute reticulocyte count (causing the child to have anemia). Acute pain
related to vaso-occlusion is an appropriate nursing diagnosis for sickl e cell
anemia for the child in vaso-occlusive crisis, but it is not applicable to a child
with aplastic anemia.
3. A nurse is teaching home care instructions to parents of a child with sickle cell
disease. Which instructions should the nurse include? Select all that apply.
a. Limit fluid intake.
b. Administer aspirin for fever.
c. Administer penicillin as ordered.
d. Avoid cold and extreme heat.
e. Provide for adequate rest periods.
ANS: C, D, E
Parents should be taught to avoid cold, which can increase sickling, and
extreme heat, which can cause dehydration. Adequate rest periods should be
provided. Penicillin should be administered daily as ordered. The use of
aspirin should be avoided; acetaminophen or ibuprofen should be used as an
alternative. Fluids should be encouraged and an increase in fluid intake is
encouraged in hot weather or when there are other risks for dehydration.
4. Which is true about the genetic transmission of sickle cell disease?
a. Both parents must carry the sickle cell trait.
b. Both parents must have sickle cell disease.
c. One parent must have the sickle cell trait.
d. Sickle cell disease has no known pattern of inheritance.
