Chapter 44 Hematological Disorders

PRACTICE QUESTIONS
1. A clinic nurse instructs the mother of a child with sickle cell anemia
about the precipitating factors related to pain crisis. Which of the
following, if identified by the mother as a precipitating factor,
indicates the need for further instructions?
1. Stress
2. Trauma
3. Infection
4. Fluid overload

2. A 10-year-old child with hemophilia A has slipped on the ice and

bumped his knee. The nurse should prepare to administer an:
1. Injection of factor X
2. Intravenous infusion of factor VIII
3. Intravenous infusion of cryoprecipitate
4. Intravenous infusion of desmopressin (DDAVP)

3. Laboratory studies are performed for a child suspected of having

iron
deficiency anemia. The nurse reviews the laboratory results, knowing
that which of the following results would indicate this type of anemia?
1. An elevated hemoglobin level
2. A decreased reticulocyte count
3. An elevated red blood cell count
4. Red blood cells that are microcytic and hypochromic

4. A home care nurse is instructing the parents of a child with iron

deficiency anemia regarding the administration of a liquid oral iron
supplement. The nurse tells the mother to:
1. Administer the iron at mealtimes.
2. Administer the iron through a straw.
3. Mix the iron with cereal to administer.
4. Add the iron to formula for easy administration.

5. A nurse analyzes the laboratory results of a child with hemophilia.

The
nurse understands that which of the following would most likely be
abnormal in this child?
1. Platelet count
2. Hematocrit level
3. Hemoglobin level
4. Partial thromboplastin time

1
6. A nurse is providing home care instructions to the mother of a
10-year-old child with hemophilia. Which of the following activities
should the nurse suggest that the child could participate safely with
peers?
1. Soccer
2. Basketball
3. Swimming
4. Field hockey

7. A nursing student is presenting a clinical conference and discusses

the
cause of b-thalassemia. The nursing student informs the group that the
child at greatest risk of developing this disorder is:
1. A child of Mexican descent
2. A child of Mediterranean descent
3. A child whose intake of iron is extremely poor
4. A breast-fed child of a mother with chronic anemia

8. A child with b-thalassemia is receiving long-term blood transfusion

therapy for the treatment of this disorder. Chelation therapy is
prescribed to prevent organ damage from the presence of too much
iron in the body as a result of the transfusions. Which of the following
medications would the nurse anticipate to be prescribed in chelation
therapy?
1. Meropenem (Merrem)
2. Metoprolol (Toprol-XL)
3. Deferoxamine (Desferal)
4. Dalteparin sodium (Fragmin)

9. A pediatric nursing instructor asks a nursing student to describe the

cause of the clinical manifestations that occur in sickle cell anemia.
The student responds correctly by telling the instructor that:
1. Bone marrow depression occurs because of the development of
sickled cells
2. Sickled cells increase blood flow through the body and cause a
great deal of pain
3. Sickled cells mix with the unsickled cells and cause the immune
system to become depressed
4. Sickled cells are unable to flow easily through the
microvasculature and their clumping obstructs blood flow

ALTERNATE ITEM FORMAT: MULTIPLE RESPONSE (1)

A nurse is reviewing a physician's orders for a child with sickle cell
anemia
who was admitted to the hospital for the treatment of vaso-occlusive
crisis.

2
Which orders documented in the child's record should the nurse
question?
□ 1. Restrict fluid intake.
□ 2. Position for comfort.
□ 3. Avoid strain on painful joints.
□ 4. Apply nasal oxygen at 2 L/min
□ 5. Provide a high-calorie, high-protein diet.
□ 6. Give meperidine (Demerol), 25 mg IV, every 4 hours for pain.
ALTERNATE ITEM FORMAT: MULTIPLE RESPONSE (2)
Which of the following are characteristics of von Willebrand disease?
Select
all that apply.
□ 1. Gum bleeding occurs.
□ 2. Easy bruising occurs.
□ 3. It is a hereditary bleeding disorder.
□ 4. It is characterized by extremely high creatinine levels.
□ 5. The disorder causes platelets to adhere to damaged endothelium.
□ 6. Treatment and care are similar to those implemented for
hemophilia.

ANSWERS
1. 4 Rationale: Pain crisis may be precipitated by infection,
dehydration,
hypoxia, trauma, or physical or emotional stress. The mother of a
child with sickle cell disease should encourage fluid intake of 1½ to 2
times the daily requirement to prevent dehydration.

2. 2 Rationale: Hemophilia refers to a group of bleeding disorders

resulting from a deficiency of specific coagulation proteins. The
primary treatment is replacement of the missing clotting factor;
additional medications, such as those to relieve pain, may be
prescribed depending on the source of bleeding from the disorder. A
child with hemophilia A will be at risk for joint bleeding after a fall.
Factor VIII will be prescribed intravenously to replace the missing
clotting factor and minimize the bleeding. Desmopressin (DDAVP) is
used to stimulate production of factor VIII, but it is not given
intravenously. Factor X and cryoprecipitate are not used for clients
with hemophilia A.

3. 4 Rationale: The results of a complete blood cell count in children

with
iron deficiency anemia will show decreased hemoglobin levels and
microcytic and hypochromic red blood cells. The red blood cell count
is decreased. The reticulocyte count is usually normal or slightly
elevated.

3
4. 2 Rationale: An oral iron supplement should be administered
through a
straw or medicine dropper placed at the back of the mouth because
the
iron will stain the teeth. The parents should be instructed to brush or
wipe the child's teeth after administration. Iron is administered
between meals because absorption is decreased if there is food in the
stomach. Iron requires an acid environment to facilitate its absorption
in the duodenum. Iron is not added to formula or mixed with cereal or
other food items.

5. 4 Rationale: Hemophilia refers to a group of bleeding disorders

resulting from a deficiency of specific coagulation proteins. Results of
tests that measure platelet function are normal; results of tests that
measure clotting factor function may be abnormal. Therefore,
abnormal laboratory results in hemophilia indicate a prolonged partial
thromboplastin time. The platelet count, hemoglobin level, and
hematocrit level are normal in hemophilia.

Rationale: Hemophilia refers to a group of bleeding disorders

resulting from a deficiency of specific coagulation proteins. Children
with hemophilia need to avoid contact sports and to take precautions
such as wearing elbow and knee pads and helmets with other sports.
The safest activity for them is swimming.

7. 2 Rationale: b-Thalassemia is an autosomal recessive disorder

characterized by the reduced production of one of the globin chains in
the synthesis of hemoglobin (both parents must be carriers to produce
a child with b-thalassemia major). This disorder is found primarily in
individuals of Mediterranean descent. Options 1, 3, and 4 are incorrect.

8. 3 Rationale: b-Thalassemia is an autosomal recessive disorder

characterized by the reduced production of one of the globin chains in
the synthesis of hemoglobin (both parents must be carriers to produce
a child with b-thalassemia major). The major complication of chronic
transfusion therapy is hemosiderosis. To prevent organ damage from
too much iron in the blood, chelation drug therapy with deferoxamine
(Desferal) is used. Deferoxamine is classified as an antidote for acute
iron toxicity. Dalteparin is an anticoagulant used as prophylaxis for
postoperative deep vein thrombosis. Meropenem is an antibiotic.
Metoprolol is a b blocker used to treat hypertension.

9. 4 Rationale: Sickle cell anemia is a group of diseases termed

4
hemoglobinopathies, in which hemoglobin A is partly or completely
replaced by abnormal sickle hemoglobin S. It is caused by the
inheritance of a gene for a structurally abnormal portion of the
hemoglobin chain. Hemoglobin S is sensitive to changes in the
oxygen content of the red blood cell; insufficient oxygen causes the
cells to assume a sickle shape and the cells become rigid and clumped
together, obstructing capillary blood flow. All the clinical
manifestations of sickle cell anemia result from the sickled cells being
unable to flow easily through the microvasculature, and their
clumping obstructs blood flow. With reoxygenation, most of the
sickled red blood cells resume their normal shape. Options 1, 2, and 3
are incorrect statements.

ALTERNATE ITEM FORMAT: MULTIPLE RESPONSE (1)

Answer: 1, 6
Rationale: Sickle cell anemia is a group of diseases termed
hemoglobinopathies, in which hemoglobin A is partly or completely
replaced by abnormal sickle hemoglobin S. It is caused by the
inheritance of a gene for a structurally abnormal portion of the
hemoglobin chain. Hemoglobin S is sensitive to changes in the oxygen
content of the red blood cell; insufficient oxygen causes the cells to
assume a sickle shape and the cells become rigid and clumped
together, obstructing capillary blood flow. Therefore, oral and
intravenous fluids are an important part of treatment. Meperidine
(Demerol) is not recommended for the child with sickle cell disease
because of the risk for normeperidine-induced seizures.
Normeperidine, a metabolite of meperidine, is a central nervous
system
stimulant that produces anxiety, tremors, myoclonus, and generalized
seizures when it accumulates with repetitive dosing. The nurse would
thus question the order for restricted fluids and meperidine for pain
control. Positioning for comfort, avoiding strain in painful joints,
oxygen, and a high-calorie, high-protein diet are also important parts
of the treatment plan.

ALTERNATE ITEM FORMAT: MULTIPLE RESPONSE (2)

Answer: 1, 2, 3, 5, 6
Rationale: von Willebrand disease is a hereditary bleeding disorder
characterized by a deficiency of or a defect in a protein termed von
Willebrand factor (vWF). The disorder causes platelets to adhere to
damaged endothelium. It is characterized by an increased tendency to
bleed from mucous membranes. Assessment findings include epistaxis,
gum bleeding, easy bruising, and excessive menstrual bleeding. An
elevated creatinine level is not associated with this disorder.

5
6