Chapter 23: Beta-Thalassemia

Question 1

Which type of beta-thalassemia is also known as Cooley’s anemia and requires regular transfusions?

A. Thalassemia minor
B. Thalassemia intermedia
C. Thalassemia major
D. Alpha-thalassemia trait

Answer 1

B. Thalassemia intermedia

Rationale: Thalassemia major, or Cooley’s anemia, produces severe anemia requiring regular transfusions for management.

Question 2

What is the primary cause of clinical manifestations in thalassemias?

A. Increased platelet production
B. Defective synthesis of hemoglobin and shortened RBC lifespan
C. Overproduction of white blood cells
D. Hypercoagulable state of the blood

Answer 2

B. Defective synthesis of hemoglobin and shortened RBC lifespan

Rationale: Symptoms of thalassemias result from defective hemoglobin synthesis, structurally impaired RBCs, and shortened RBC lifespan.

Question 3

Symptoms of beta-thalassemia major generally appear:

A. At birth
B. During the first 6 months of life
C. During the second 6 months of life
D. After 2 years of age

Answer 3

C. During the second 6 months of life

Rationale: Symptoms of beta-thalassemia major typically manifest during the second 6 months of life as fetal hemoglobin (HbF) production decreases.

Question 4

What is the only cure for thalassemia major?

A. Regular blood transfusions
B. Splenectomy
C. Iron chelation therapy
D. Hematopoietic stem cell transplantation (HSCT)

Answer 4

D. Hematopoietic stem cell transplantation (HSCT)

Rationale: Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for thalassemia major.

Question 5

What is the primary goal of a chronic transfusion program for children with severe thalassemia?

A. Maintain normal hemoglobin levels
B. Reduce splenomegaly
C. Prevent iron overload
D. Increase hematopoietic stem cell production

Answer 5

A. Maintain normal hemoglobin levels

Rationale: The goal of chronic transfusions is to maintain normal hemoglobin levels and prevent complications associated with severe anemia.

Question 6

Which laboratory test is definitive in diagnosing thalassemia?

A. Hemoglobin electrophoresis
B. Complete blood count (CBC)
C. Reticulocyte count
D. Peripheral blood smear

Answer 6

A. Hemoglobin electrophoresis

Rationale: Hemoglobin electrophoresis is definitive in diagnosing thalassemia as it reveals abnormal hemoglobin production.

Question 7

What medication is commonly used to treat iron overload in children undergoing regular blood transfusions for thalassemia?

A. Deferoxamine
B. Erythropoietin
C. Warfarin
D. Folic acid

Answer 7

A. Deferoxamine

Rationale: Deferoxamine is an iron-chelating drug used to treat iron overload, a common side effect of chronic transfusion therapy.

Question 8

Prenatal diagnosis of thalassemia can be achieved through:

A. Ultrasound
B. Amniocentesis or chorionic villi sampling
C. Maternal blood testing
D. Fetal movement monitoring

Answer 8

B. Amniocentesis or chorionic villi sampling

Rationale: Amniocentesis or chorionic villi sampling allows for prenatal diagnosis by identifying genetic mutations associated with thalassemia.

Question 9

Which of the following are clinical manifestations of beta-thalassemia major? (SATA)

A. Pallor
B. Growth retardation
C. Jaundice
D. Polycythemia
E. Hepatomegaly

Answer 9

A. Pallor
B. Growth retardation
C. Jaundice
E. Hepatomegaly

Rationale: Beta-thalassemia major is associated with pallor, growth retardation, jaundice, and hepatomegaly. Polycythemia is not a feature of thalassemia.

Question 10

Which diagnostic findings are consistent with thalassemia? (SATA)

A. Elevated hemoglobin F
B. Increased hematocrit
C. Decreased hemoglobin levels
D. Decreased reticulocyte count
E. Structural impairment of RBCs

Answer 10

A. Elevated hemoglobin F
C. Decreased hemoglobin levels
E. Structural impairment of RBCs

Rationale: Elevated hemoglobin F, decreased hemoglobin levels, and structurally impaired RBCs are diagnostic findings. Hematocrit and reticulocyte counts are typically decreased.

Question 11

What supportive treatments are commonly used in managing children with thalassemia? (SATA)

A. Folic acid supplementation
B. Chronic transfusion program
C. Antibiotic prophylaxis
D. Iron chelation therapy
E. Platelet transfusions

Answer 11

A. Folic acid supplementation
B. Chronic transfusion program
D. Iron chelation therapy

Rationale: Supportive treatments include folic acid supplementation, a chronic transfusion program, and iron chelation therapy. Platelet transfusions and routine antibiotics are not standard treatments for thalassemia.

Question 12

A 1-year-old child is diagnosed with beta-thalassemia major. The parents are concerned about the long-term treatment plan. Which interventions should the nurse prioritize when educating the family? (SATA)

A. Encourage iron supplementation to address anemia
B. Explain the need for regular blood transfusions
C. Discuss iron chelation therapy to prevent complications
D. Recommend hematopoietic stem cell transplantation as a cure

Answer 12

B. Explain the need for regular blood transfusions
C. Discuss iron chelation therapy to prevent complications
D. Recommend hematopoietic stem cell transplantation as a cure

Rationale: Regular transfusions, iron chelation therapy, and consideration of hematopoietic stem cell transplantation are critical aspects of the treatment plan. Iron supplementation is contraindicated due to the risk of iron overload.

Question 13

What is the primary focus of nursing care for a child with thalassemia?

A. Prevention of dehydration
B. Observation for complications of transfusion therapy
C. Administration of folic acid supplements
D. Restricting physical activity

Answer 13

B. Observation for complications of transfusion therapy

Rationale: Nursing care primarily focuses on monitoring for transfusion therapy complications, such as iron overload or transfusion reactions, while supporting the child and family.

Question 14

Which intervention is essential for parents of a child with thalassemia requiring home therapy with deferoxamine?

A. Instruction on administering oral medications
B. Teaching subcutaneous infusion techniques
C. Demonstrating proper hand hygiene during transfusions
D. Encouraging high iron diets

Answer 14

B. Teaching subcutaneous infusion techniques

Rationale: Parents should be taught the correct technique for subcutaneous infusion of deferoxamine to manage iron overload effectively.

Question 15

What is a common compliance issue for adolescents with thalassemia?

A. Refusal to attend genetic counseling
B. Resistance to deferoxamine therapy
C. Nonadherence to dietary restrictions
D. Avoidance of blood transfusions

Answer 15

D. Avoidance of blood transfusions

Rationale: Adolescents often struggle with compliance to regular transfusion schedules due to the time and lifestyle adjustments required.

Question 16

What should the nurse prioritize when educating parents of a newly diagnosed child with thalassemia?

A. Encouraging physical activity
B. Managing dehydration
C. Genetic counseling and transmission education
D. Avoiding immunizations

Answer 16

C. Genetic counseling and transmission education

Rationale: Education should include the genetic transmission of thalassemia and the implications for other family members.

Question 17

Which expected outcomes indicate effective nursing care for a child with thalassemia? (SATA)

A. Maintenance of normal hemoglobin and hematocrit levels
B. Increased physical activity
C. Safe administration of blood transfusions
D. Maintenance of recommended body iron levels
E. Family understanding of the disease’s genetic transmission

Answer 17

A. Maintenance of normal hemoglobin and hematocrit levels
C. Safe administration of blood transfusions
D. Maintenance of recommended body iron levels
E. Family understanding of the disease’s genetic transmission

Rationale: Nursing care aims to ensure normal hemoglobin levels, safe transfusions, appropriate iron levels, and family education. Increased physical activity is not a primary nursing goal.

Question 18

Which nursing interventions support compliance with transfusion therapy in adolescents with thalassemia? (SATA)

A. Offering flexible transfusion schedules
B. Educating adolescents about the consequences of missed transfusions
C. Avoiding discussions about future complications
D. Involving adolescents in treatment decisions
E. Restricting peer involvement during transfusions

Answer 18

A. Offering flexible transfusion schedules
B. Educating adolescents about the consequences of missed transfusions
D. Involving adolescents in treatment decisions

Rationale: Flexible schedules, education, and involving adolescents in treatment decisions promote compliance. Avoiding discussions and restricting peer involvement are not effective strategies.

Question 19

What should a nurse include when teaching parents about managing thalassemia at home? (SATA)

A. Techniques for administering subcutaneous deferoxamine
B. Signs of transfusion reactions
C. Encouraging a high-iron diet
D. Monitoring for symptoms of anemia
E. Genetic counseling referrals

Answer 19

A. Techniques for administering subcutaneous deferoxamine
B. Signs of transfusion reactions
D. Monitoring for symptoms of anemia
E. Genetic counseling referrals

Rationale: Teaching includes deferoxamine administration, recognizing transfusion reactions, monitoring for anemia, and providing resources for genetic counseling. A high-iron diet is contraindicated.

Question 20

A 14-year-old adolescent with thalassemia frequently misses scheduled transfusions due to school and extracurricular activities. The nurse plans an intervention to improve compliance. Which action is most appropriate?

A. Schedule transfusions during after-school hours to accommodate the adolescent’s schedule

B. Instruct the family to prioritize transfusions over the adolescent’s activities

C. Refer the adolescent to a counselor to address noncompliance

D. Limit the adolescent’s extracurricular activities

Answer 20

A. Schedule transfusions during after-school hours to accommodate the adolescent’s schedule

Rationale: Adjusting the transfusion schedule to fit the adolescent’s lifestyle promotes compliance while supporting normal development and participation in activities.

Question 21

What is the underlying cause of anemia in thalassemia?

A. Increased hemoglobin synthesis
B. Structural impairment and shortened lifespan of RBCs
C. Autoimmune destruction of red blood cells
D. Inability to absorb dietary iron

Answer 21

B. Structural impairment and shortened lifespan of RBCs

Rationale: Thalassemia anemia is caused by defective hemoglobin synthesis, structurally impaired RBCs, and their shortened lifespan.

Question 22

What is the only curative treatment for thalassemia major?

A. Routine blood transfusions
B. Hematopoietic stem cell transplant
C. Iron-chelating therapy
D. Splenectomy

Answer 22

B. Hematopoietic stem cell transplant

Rationale: Hematopoietic stem cell transplant is the only definitive cure for thalassemia major.

Question 23

At what age do clinical manifestations of thalassemia major typically develop?

A. Birth
B. 3 months of age
C. Between 6 months and 1 year
D. After 2 years of age

Answer 23

C. Between 6 months and 1 year

Rationale: Symptoms of thalassemia major usually develop between 6 months and 1 year of age as fetal hemoglobin production decreases.

Question 24

What condition is characterized by abnormal accumulation of fluid in two or more fetal compartments, including ascites and pleural effusion?

A. Hydrops fetalis
B. Hemarthrosis
C. Cooley’s anemia
D. Thalassemia minor

Answer 24

A. Hydrops fetalis

Rationale: Hydrops fetalis is a severe fetal condition involving abnormal fluid accumulation in multiple compartments.

Question 25

Which diagnostic test confirms thalassemia?

A. Complete blood count (CBC)
B. Peripheral blood smear
C. Hemoglobin electrophoresis
D. Bone marrow biopsy

Answer 25

C. Hemoglobin electrophoresis

Rationale: Hemoglobin electrophoresis identifies abnormal globin chain synthesis and is diagnostic for thalassemia.

Question 26

Which of the following is a clinical manifestation of thalassemia major?

A. Hypertension
B. Cyanosis
C. Bradycardia
D. Hepatomegaly and splenomegaly

Answer 26

D. Hepatomegaly and splenomegaly

Rationale: Hepatomegaly and splenomegaly are common findings due to increased erythropoiesis and destruction of RBCs in thalassemia major.

Question 27

Which thalassemia subtype typically causes mild anemia and rarely requires transfusion therapy?

A. Thalassemia minor
B. Thalassemia intermedia
C. Thalassemia major
D. Alpha-thalassemia major

Answer 27

A. Thalassemia minor

Rationale: Thalassemia minor results in mild anemia and generally does not require transfusions.

Question 28

What complication commonly arises as children with thalassemia reach adolescence?

A. Decreased appetite
B. Noncompliance with transfusion therapy
C. Onset of autoimmune disorders
D. Increased risk of infections

Answer 28

B. Noncompliance with transfusion therapy

Rationale: Adolescents with thalassemia often struggle with compliance to regular transfusion therapy due to lifestyle changes and peer pressures.

Question 29

Which clinical manifestations are associated with thalassemia major? (SATA)

A. Pallor
B. Jaundice
C. Hemarthrosis
D. Hepatomegaly
E. Growth retardation

Answer 29

A. Pallor
B. Jaundice
D. Hepatomegaly
E. Growth retardation

Rationale: Pallor, jaundice, hepatomegaly, and growth retardation are common manifestations of thalassemia major. Hemarthrosis is not typically seen in thalassemia.

Question 30

What are the goals of managing thalassemia major? (SATA)

A. Maintaining normal hemoglobin levels
B. Preventing iron overload
C. Eliminating the disease with transfusions
D. Supporting normal growth and development
E. Preventing thrombus formation

Answer 30

A. Maintaining normal hemoglobin levels
B. Preventing iron overload
D. Supporting normal growth and development

Rationale: Management focuses on maintaining hemoglobin levels, preventing complications like iron overload, and supporting normal growth and development. Transfusions do not cure the disease, and thrombus prevention is not a primary concern.

Question 31

Which findings on a hemoglobin electrophoresis suggest thalassemia? (SATA)

A. Decreased production of a globin chain
B. Elevated hemoglobin F
C. Decreased hemoglobin A
D. Increased platelet count
E. Decreased white blood cell count

Answer 31

A. Decreased production of a globin chain
B. Elevated hemoglobin F
C. Decreased hemoglobin A

Rationale: Thalassemia is confirmed by findings such as decreased production of globin chains, elevated hemoglobin F, and decreased hemoglobin A. Platelets and WBCs are unaffected.

Question 32

Which complications are associated with untreated thalassemia major? (SATA)

A. Chronic hypoxia
B. Iron overload
C. Intracranial hemorrhage
D. Growth retardation
E. Hepatomegaly

Answer 32

A. Chronic hypoxia
B. Iron overload
D. Growth retardation
E. Hepatomegaly

Rationale: Untreated thalassemia major can lead to chronic hypoxia, iron overload from frequent transfusions, growth retardation, and hepatomegaly. Intracranial hemorrhage is not a direct complication.

Question 33

A 9-month-old infant presents with pallor, irritability, and hepatosplenomegaly. Hemoglobin electrophoresis reveals decreased hemoglobin A and elevated hemoglobin F. Which diagnosis is most likely?

A. Sickle cell anemia
B. Thalassemia major
C. Thalassemia minor
D. Iron-deficiency anemia

Answer 33

B. Thalassemia major

Rationale: The clinical presentation and diagnostic findings are consistent with thalassemia major, which presents in infancy with severe anemia and abnormal hemoglobin production.

Question 34

A pregnant woman undergoes prenatal testing via chorionic villus sampling, which reveals the fetus has alpha-thalassemia major. What intervention should the nurse anticipate?

A. Immediate induction of labor
B. Administration of iron supplements to the mother
C. Genetic counseling referral postpartum
D. Intrauterine transfusion

Answer 34

D. Intrauterine transfusion

Rationale: Intrauterine transfusion may be required to manage hydrops fetalis associated with alpha-thalassemia major.

Question 35

A child who has beta-thalassemia is receiving numerous blood transfusions. The child is also receiving deferoxamine (Desferal) therapy. The parents ask how the deferoxamine will help their child. Which rationale does the nurse use when responding to the parents?

1. It prevents blood transfusion reactions.
2. It stimulates RBC production.
3. It provides vitamin supplementation.
4. It prevents iron overload.

Answer 35

4. It prevents iron overload.

Rationale: Iron overload can be a side effect of a hypertransfusion therapy. Deferoxamine (Desferal) is an iron-chelating drug, which binds excess iron so it can be excreted by the kidneys. It does not prevent blood-transfusion reactions, stimulate RBC production, or provide vitamin supplementation.