Chapter 23: Sickle Cell Disease

Question 1

What is the primary pathological process in sickle cell disease (SCD)?

A. Decreased production of red blood cells

B. Abnormal hemoglobin S causing red blood cell deformation

C. Increased destruction of white blood cells

D. Replacement of hemoglobin S with normal hemoglobin

Answer 1

B. Abnormal hemoglobin S causing red blood cell deformation

Rationale: In SCD, normal hemoglobin is replaced by hemoglobin S, which causes red blood cells to become sickle-shaped, leading to occlusion of small blood vessels, ischemia, and organ damage.

Question 2

Which individual is most at risk for experiencing complications associated with sickle cell disease?

A. A person carrying one gene for hemoglobin S

B. A child with sickle cell trait

C. A person with two genes for hemoglobin S

D. An individual with normal hemoglobin levels

Answer 2

C. A person with two genes for hemoglobin S

Rationale: Having two genes for hemoglobin S (homozygous SCD) leads to the disease and is associated with complications such as vaso-occlusion, ischemia, and organ damage.

Question 3

Which complication is most directly caused by occlusion of small blood vessels in sickle cell disease?

A. Severe anemia

B. Ischemia and tissue damage

C. Increased red blood cell production

D. Elevated white blood cell counts

Answer 3

B. Ischemia and tissue damage

Rationale: The occlusion of small blood vessels by sickled cells leads to ischemia, which in turn causes tissue and organ damage.

Question 4

What is the most important health promotion advice for parents of a child with sickle cell trait?

A. Ensure regular hydration and avoid high altitudes.

B. Monitor the child for signs of severe anemia.

C. Limit physical activity to prevent vaso-occlusive crises.

D. Seek genetic counseling for future family planning.

Answer 4

D. Seek genetic counseling for future family planning.

Rationale: Sickle cell trait is generally asymptomatic, but genetic counseling can help parents understand inheritance patterns and the likelihood of future children having sickle cell disease.

Question 5

Which genetic pattern is responsible for the inheritance of sickle cell disease?

A. X-linked dominant
B. Autosomal recessive
C. Autosomal dominant
D. Multifactorial

Answer 5

B. Autosomal recessive

Rationale: Sickle cell disease is inherited in an autosomal recessive pattern, meaning both parents must carry the sickle cell trait for their child to have a 25% chance of inheriting the disease.

Question 6

What causes the sickled shape of red blood cells in sickle cell anemia (HbSS)?

A. Increased levels of fetal hemoglobin
B. Deficiency of red blood cell membrane proteins
C. Prolonged oxygen saturation levels
D. Abnormal hemoglobin S in red blood cells

Answer 6

D. Abnormal hemoglobin S in red blood cells

Rationale: Hemoglobin S in red blood cells leads to deformation of the cells into a sickle shape, especially during periods of low oxygen tension.

Question 7

What is the most common complication of functional asplenia in children with sickle cell disease?

A. Stroke
B. Retinopathy
C. Bacterial infections
D. Gallstone formation

Answer 7

C. Bacterial infections

Rationale: Functional asplenia increases the risk of bacterial infections due to impaired immunity, making it a leading cause of death in young children with sickle cell disease.

Question 8

Which factor is most likely to precipitate a sickle cell crisis?

A. A high-carbohydrate diet
B. Low fetal hemoglobin levels
C. Physical or emotional stress
D. Increased spleen activity

Answer 8

C. Physical or emotional stress

Rationale: Physical or emotional stress can trigger sickling of red blood cells by increasing oxygen demand or altering oxygen transport, precipitating a crisis.

Question 9

What is the primary cause of chronic anemia in sickle cell disease?

A. Bone marrow suppression
B. Impaired oxygen transport
C. Nutritional deficiencies
D. Shortened red blood cell lifespan

Answer 9

D. Shortened red blood cell lifespan

Rationale: In sickle cell disease, red blood cells have a lifespan of 10-20 days instead of the usual 120 days, leading to chronic hemolytic anemia.

Question 10

Which organ is most commonly affected by infarction in children with sickle cell disease?

A. Lungs
B. Liver
C. Kidneys
D. Spleen

Answer 10

D. Spleen

Rationale: The spleen is commonly affected by infarction, leading to functional asplenia and an increased risk of infections in children with sickle cell disease.

Question 11

Which condition is characterized by blood pooling in the spleen and can be life-threatening in children with sickle cell disease?

A. Splenic sequestration
B. Acute chest syndrome
C. Priapism
D. Hemolytic crisis

Answer 11

A. Splenic sequestration

Rationale: Splenic sequestration occurs when blood becomes trapped in the spleen, leading to hypovolemic shock and life-threatening complications.

Question 12

What is the primary cause of acute chest syndrome in children with sickle cell disease?

A. Pulmonary infiltrates and infection
B. Increased red blood cell production
C. Severe dehydration
D. Functional asplenia

Answer 12

A. Pulmonary infiltrates and infection

Rationale: Acute chest syndrome is caused by pulmonary infiltrates and infection, which result in respiratory distress.

Question 13

Which factor is least likely to precipitate a sickle cell crisis?

A. Dehydration
B. High altitudes
C. Infection
D. Vitamin deficiency

Answer 13

D. Vitamin deficiency

Rationale: Sickle cell crises are triggered by factors such as dehydration, hypoxia, and infection, but not directly by vitamin deficiencies.

Question 14

What is the approximate lifespan of sickled red blood cells compared to normal red blood cells?

A. 10-20 days vs. 120 days
B. 30-40 days vs. 90 days
C. 60-70 days vs. 100 days
D. 80-90 days vs. 110 days

Answer 14

A. 10-20 days vs. 120 days

Rationale: Sickled red blood cells have a significantly shortened lifespan of 10-20 days compared to the normal lifespan of 120 days.

Question 15

Why does hypoxia exacerbate sickling in red blood cells?

A. It increases cell rigidity.
B. It promotes hemolysis.
C. It leads to vascular occlusion.
D. It decreases hemoglobin oxygenation.

Answer 15

D. It decreases hemoglobin oxygenation.

Rationale: Hypoxia decreases the oxygenation of hemoglobin, leading to a conformational change in hemoglobin S and promoting red blood cell sickling.

Question 16

Which clinical manifestation indicates a life-threatening complication in a child with sickle cell disease?

A. Fatigue and pallor
B. Persistent fever
C. Severe abdominal pain with splenomegaly
D. Elevated bilirubin levels

Answer 16

C. Severe abdominal pain with splenomegaly

Rationale: Severe abdominal pain with splenomegaly may indicate splenic sequestration, a life-threatening complication that requires immediate intervention.

Question 17

Which treatment strategy is most effective in reversing sickling during a crisis?

A. Rehydration and reoxygenation
B. Blood transfusion
C. Pain management with opioids
D. Immunosuppressive therapy

Answer 17

A. Rehydration and reoxygenation

Rationale: Rehydration and reoxygenation help restore red blood cells to their normal shape and improve blood flow during a sickle cell crisis.

Question 18

Why are children with sickle cell disease asymptomatic until 4 to 6 months of age?

A. Immature immune system
B. High levels of fetal hemoglobin
C. Fully functional spleen
D. Low oxygen demands during infancy

Answer 18

B. High levels of fetal hemoglobin

Rationale: Fetal hemoglobin (HbF) inhibits the sickling of red blood cells. As HbF levels decline around 4 to 6 months, symptoms of sickle cell disease begin to appear.

Question 19

Which is the most common reason for emergency department visits in children with sickle cell disease?

A. Splenic sequestration crisis
B. Stroke symptoms
C. Acute pain crisis
D. Acute chest syndrome

Answer 19

C. Acute pain crisis

Rationale: Acute pain crisis, caused by vaso-occlusion and subsequent tissue ischemia, is the leading reason for emergency department visits in children with sickle cell disease.

Question 20

Which clinical manifestation is associated with chronic ischemia of bones in sickle cell disease?

A. Aplastic crisis
B. Osteomyelitis
C. Peripheral neuropathy
D. Priapism

Answer 20

B. Osteomyelitis

Rationale: Chronic ischemia of bones increases susceptibility to infection, leading to complications such as osteomyelitis.

Question 21

Which organ is most affected by infarction, resulting in increased infection rates in children with sickle cell disease?

A. Liver
B. Brain
C. Spleen
D. Kidneys

Answer 21

C. Spleen

Rationale: Infarction of the spleen leads to fibrosis and functional asplenia, significantly increasing the risk of infection.

Question 22

What is the primary cause of pain during a vaso-occlusive crisis?

A. Hemolytic anemia
B. Retinal detachment
C. Hepatomegaly
D. Tissue ischemia

Answer 22

D. Tissue ischemia

Rationale: Pain during a vaso-occlusive crisis results from tissue ischemia caused by restricted blood flow due to occlusion of blood vessels by sickled cells.

Question 23

Which complication of sickle cell disease is characterized by fever, chest pain, tachypnea, and pulmonary infiltrates?

A. Acute chest syndrome
B. Splenic sequestration crisis
C. Aplastic crisis
D. Hemolytic crisis

Answer 23

A. Acute chest syndrome

Rationale: Acute chest syndrome is a life-threatening complication of sickle cell disease characterized by fever, chest pain, tachypnea, coughing, wheezing, and pulmonary infiltrates visible on chest radiographs.

Question 24

Which clinical manifestation may indicate chronic ischemia of the kidneys in children with sickle cell disease?

A. Hematuria
B. Priapism
C. Retinopathy
D. Aphasia

Answer 24

A. Hematuria

Rationale: Chronic ischemia of the kidneys can cause hematuria, enuresis, and the inability to concentrate urine.

Question 25

What symptom might a child with sickle cell disease exhibit if they are experiencing a stroke?

A. Arthralgia
B. Aphasia
C. Peripheral neuropathy
D. Osteoporosis

Answer 25

B. Aphasia

Rationale: Aphasia, along with headache, convulsions, or visual changes, may indicate a stroke, a serious complication of sickle cell disease.

Question 26

Which clinical manifestation may suggest peripheral neuropathy in a child with sickle cell disease?

A. Ulcerations
B. Hematuria
C. Weakness in extremities
D. Visual changes

Answer 26

C. Weakness in extremities

Rationale: Vaso-occlusion and chronic ischemia can result in peripheral neuropathy, which may manifest as weakness in the extremities.

Question 27

What is a potential complication of vaso-occlusion in the penis in boys with sickle cell disease?

A. Hematuria
B. Priapism
C. Hepatomegaly
D. Retinal detachment

Answer 27

B. Priapism

Rationale: Priapism, or sustained painful penile erection, occurs due to microcirculatory obstruction and engorgement in the penis.

Question 28

Which manifestation indicates decreased peripheral circulation in a child with sickle cell disease?

A. Aphasia
B. Ulcerations
C. Priapism
D. Spinal deformities

Answer 28

B. Ulcerations

Rationale: Decreased peripheral circulation caused by vaso-occlusion may result in skin ulcerations.

Question 29

What is the first step in confirming a diagnosis of sickle cell disease in a newborn with a positive screening test?

A. Hemoglobin electrophoresis
B. Genetic testing
C. Blood smear analysis
D. Complete blood count (CBC)

Answer 29

A. Hemoglobin electrophoresis

Rationale: Hemoglobin electrophoresis is the confirmatory test for diagnosing sickle cell disease after a positive newborn screening test.

Question 30

What is the rationale for administering intravenous fluids during a sickle cell crisis?

A. To prevent infection
B. To reduce blood viscosity
C. To enhance oxygenation
D. To decrease energy expenditure

Answer 30

B. To reduce blood viscosity

Rationale: Intravenous fluids promote hydration, which reduces blood viscosity and decreases the risk of further vaso-occlusion.

Question 31

Which medication is commonly used for pain management in a child with a sickle cell crisis?

A. Acetaminophen
B. Ibuprofen
C. Morphine
D. Aspirin

Answer 31

C. Morphine

Rationale: Morphine is a parenteral narcotic commonly used to manage severe pain in children with sickle cell crisis.

Question 32

Why is penicillin prophylaxis initiated in children with sickle cell disease?

A. To prevent infection from Streptococcus pneumoniae
B. To reduce the risk of vaso-occlusive crises
C. To improve hemoglobin concentration
D. To decrease iron overload

Answer 32

A. To prevent infection from Streptococcus pneumonia

Rationale: Penicillin prophylaxis is used to prevent life-threatening bacterial infections, particularly those caused by Streptococcus pneumoniae.

Question 33

Which vaccination is recommended for children with sickle cell disease beginning at 2 months of age if they have functional asplenia?

A. Hepatitis B vaccine
B. Influenza vaccine
C. Varicella vaccine
D. Meningococcal vaccine

Answer 33

D. Meningococcal vaccine

Rationale: Children with functional asplenia should receive the meningococcal vaccine starting at 2 months of age to prevent meningococcal infections.

Question 34

What is the primary benefit of hydroxyurea therapy in children with sickle cell disease?

A. Reducing iron overload
B. Increasing fetal hemoglobin levels
C. Preventing infections
D. Enhancing oxygen saturation

Answer 34

B. Increasing fetal hemoglobin levels

Rationale: Hydroxyurea increases fetal hemoglobin levels, which reduces red blood cell sickling and the frequency of vaso-occlusive crises.

Question 35

Which complication is a potential risk of frequent blood transfusions in children with sickle cell disease?

A. Hemolysis
B. Dehydration
C. Hemosiderosis
D. Hypertension

Answer 35

C. Hemosiderosis

Rationale: Frequent transfusions can result in hemosiderosis, or iron overload, due to the body’s inability to excrete excess iron.

Question 36

Which medication is used to chelate excess iron in children with hemosiderosis caused by frequent transfusions?

A. Hydroxyurea
B. Ibuprofen
C. Penicillin
D. Deferoxamine

Answer 36

D. Deferoxamine

Rationale: Deferoxamine is an iron-chelating drug that binds excess iron, allowing it to be excreted by the kidneys.

Question 37

What is the purpose of annual transcranial Doppler (TCD) testing in children with sickle cell disease?

A. Detecting retinopathy
B. Identifying risk of stroke
C. Monitoring liver function
D. Diagnosing infection

Answer 37

B. Identifying risk of stroke

Rationale: Annual TCD testing is performed in children aged 2 to 16 to assess blood flow and identify those at risk for stroke.

Question 38

Why are blood cultures obtained immediately when infection is suspected in a child with sickle cell disease?

A. To identify the source of pain
B. To determine the causative organism
C. To prevent dehydration
D. To confirm sickle cell disease

Answer 38

B. To determine the causative organism

Rationale: Blood cultures are essential to identify the source of infection and guide appropriate antibiotic therapy.

Question 39

At what age should children with sickle cell disease receive the 23-valent pneumococcal vaccine (Pneumovax)?

A. 6 months
B. 1 year
C. 2 years
D. 12 years

Answer 39

C. 2 years

Rationale: Children with sickle cell disease should receive the 23-valent pneumococcal vaccine at age 2 and age 5, with booster doses every 5 years as adults.

Question 40

What is the only known cure for sickle cell disease?

A. Hematopoietic stem cell transplantation
B. Hydroxyurea therapy
C. Prophylactic blood transfusions
D. Iron-chelating therapy

Answer 40

A. Hematopoietic stem cell transplantation

Rationale: Hematopoietic stem cell transplantation (HSCT) is the only known cure for sickle cell disease, though its use is limited due to donor compatibility.

Question 41

Which of the following immunizations should a child with sickle cell disease receive annually?

A. Varicella vaccine
B. Hepatitis B vaccine
C. Influenza vaccine
D. Pneumococcal vaccine

Answer 41

C. Influenza vaccine

Rationale: Annual influenza vaccination is recommended for children with sickle cell disease to prevent complications from the flu.

Question 42

Which adjunctive therapy is used for pain relief during a sickle cell crisis?

A. Parenteral morphine
B. Oral ibuprofen
C. Subcutaneous insulin
D. Intramuscular penicillin

Answer 42

B. Oral ibuprofen

Rationale: Ibuprofen or ketorolac is often used as adjunctive therapy to parenteral narcotics for pain management.

Question 43

Why might penicillin prophylaxis be continued beyond 5 years of age in a child with sickle cell disease?

A. If the child has functional asplenia
B. If the child is receiving hydroxyurea
C. If the child has abnormal TCD results
D. If the child is iron deficient

Answer 43

A. If the child has functional asplenia

Rationale: Penicillin prophylaxis may be continued beyond age 5 if the child has functional asplenia or a history of severe pneumococcal sepsis.

Question 44

Which complication does hydroxyurea primarily help prevent?

A. Hemosiderosis
B. Stroke
C. Vaso-occlusive crises
D. Iron overload

Answer 44

C. Vaso-occlusive crises

Rationale: Hydroxyurea reduces the frequency of vaso-occlusive crises by increasing fetal hemoglobin levels.

Question 45

What is the primary reason for prophylactic blood transfusions in children with abnormal TCD results?

A. To prevent stroke
B. To treat anemia
C. To reduce pain
D. To prevent infection

Answer 45

A. To prevent stroke

Rationale: Prophylactic transfusions reduce the risk of stroke in children with abnormal transcranial Doppler results.

Question 46

A 4-year-old child with sickle cell disease (SCD) is admitted to the hospital for a painful crisis. The child is experiencing severe pain in the arms and legs and is visibly distressed. Vital signs are within normal limits. The physician orders intravenous fluids and analgesics.
What is the priority nursing action for this child?

A) Administer blood transfusions and oxygen
B) Monitor for transfusion reactions

C) Provide analgesics as ordered and ensure hydration

D) Schedule caregiving activities to reduce energy expenditure

Answer 46

C) Provide analgesics as ordered and ensure hydration

Rationale: The priority in a sickle cell crisis is to control pain and ensure adequate hydration. Both of these are essential to improving tissue perfusion and reducing the severity of the crisis.

Question 47

A 6-year-old child with sickle cell disease is being discharged after a hospital stay for a crisis. The child’s mother is concerned about managing the child’s nutrition at home.
Which of the following instructions should the nurse provide regarding nutrition for the child with sickle cell disease?

A) Emphasize a low-protein, low-calorie diet to avoid complications.
B) Encourage the child to eat a high-protein, high-calorie diet.
C) Suggest that the child avoid vitamin C supplements.
D) Advise the child to avoid any supplements as they are unnecessary.

Answer 47

B) Encourage the child to eat a high-protein, high-calorie diet.

Rationale: A high-protein, high-calorie diet is essential for children with sickle cell disease to promote growth and maintain adequate nutritional status.

Question 48

A 7-year-old child with sickle cell disease presents with fever, cough, and difficulty breathing. The nurse suspects acute chest syndrome.
What is the most appropriate nursing intervention for this child?

A) Administer a blood transfusion immediately
B) Withhold antibiotics until the culture results are available
C) Encourage the child to increase fluid intake and rest
D) Monitor the child’s vital signs and respiratory status

Answer 48

D) Monitor the child’s vital signs and respiratory status

Rationale: Acute chest syndrome requires close monitoring of vital signs and respiratory status. Early intervention with oxygen and possible antibiotics is essential, but first, the nurse should assess the child’s clinical condition.

Question 49

A 10-year-old child with sickle cell disease presents with swelling of the abdomen and pain in the lower left quadrant. The physician suspects splenic sequestration.
What is the most important nursing intervention in this case?

A) Encourage the child to rest and hydrate
B) Administer pain medications and monitor for respiratory distress
C) Perform gentle palpation to assess for an enlarged spleen
D) Administer blood transfusions and monitor for signs of anemia

Answer 49

C) Perform gentle palpation to assess for an enlarged spleen

Rationale: Splenic sequestration can lead to rapid blood loss, so the nurse should gently palpate the abdomen to assess for splenic enlargement while monitoring for signs of shock.

Question 50

A 5-year-old child with sickle cell disease is scheduled for a blood transfusion. The nurse needs to prepare the child and family for the procedure.
Which of the following is the most appropriate nursing intervention to reduce the risk of transfusion reactions?

A) Use saline as the intravenous fluid before and after the transfusion

B) Allow the child to be active during the transfusion to reduce stress

C) Restrict fluid intake to prevent fluid overload during transfusion

D) Administer prophylactic antibiotics before starting the transfusion

Answer 50

A) Use saline as the intravenous fluid before and after the transfusion

Rationale: Saline should be used before and after a blood transfusion to prevent hemolysis, and it is important to monitor for any signs of transfusion reactions.

Question 51

A mother of a child with sickle cell disease asks the nurse about infection prevention strategies. The child has had frequent hospitalizations due to crises.
Which response by the nurse is most appropriate?

A) “Infection prevention is not a priority since the child is often hospitalized.”

B) “Your child should receive all recommended immunizations and start penicillin prophylaxis immediately.”

C) “There is no need for immunizations since the child is already receiving regular blood transfusions.”

D) “You can stop penicillin prophylaxis after the child turns 5 years old.”

Answer 51

B) “Your child should receive all recommended immunizations and start penicillin prophylaxis immediately.”

Rationale: Infection prevention is critical for children with sickle cell disease, including the need for all recommended immunizations and early initiation of penicillin prophylaxis to reduce the risk of pneumococcal infections.

Question 52

A 12-year-old child with sickle cell disease and a history of multiple hospitalizations is being referred for genetic counseling.
What should the nurse emphasize during this counseling session?

A) The importance of genetic counseling only for the child, not the family
B) The availability of stem cell transplantation as a definitive cure for the disease
C) The potential for genetic testing and the implications for future family planning
D) The need to discontinue treatment for sickle cell disease at puberty

Answer 52

C) The potential for genetic testing and the implications for future family planning

Rationale: Genetic counseling can help the family understand the risks of passing the disease to future children and guide them in decision-making regarding family planning and genetic testing.

Question 53

A 3-year-old child with sickle cell disease is being treated for a vaso-occlusive crisis. The nurse is preparing to administer analgesics.
Which of the following is the most important nursing consideration when administering analgesics to the child?

A) Provide analgesics on a scheduled, around-the-clock basis during a crisis
B) Administer analgesics only when the child is in severe pain
C) Limit pain medication to oral acetaminophen only
D) Avoid giving analgesics if the child shows no signs of distress

Answer 53

A) Provide analgesics on a scheduled, around-the-clock basis during a crisis

Rationale: Administering analgesics around the clock during a sickle cell crisis helps to manage pain effectively and prevent pain exacerbation.

Question 54

A child with sickle cell disease has been receiving monthly blood transfusions to prevent stroke. The nurse notices that the child’s ferritin levels are elevated.
What is the most appropriate intervention?

A) Start the child on iron chelation therapy
B) Discontinue blood transfusions immediately
C) Continue blood transfusions without any changes
D) Monitor the child’s ferritin levels over the next few months

Answer 54

A) Start the child on iron chelation therapy

Rationale: Chronic blood transfusions can lead to iron overload, and iron chelation therapy is needed to prevent tissue damage from excess iron.

Question 55

A 9-year-old child with sickle cell disease is experiencing pain in both legs and is unable to walk. The child is anxious and asks about possible long-term effects.
What is the nurse’s best response regarding the long-term management of the child’s condition?

A) “There is no long-term management needed; you can expect complete recovery soon.”

B) “The pain you are feeling is temporary, and pain management is the primary treatment.”

C) “With frequent hospitalizations, your child’s condition may lead to permanent disabilities.”

D) “With appropriate treatment and support, children with sickle cell disease can lead relatively normal lives.”

Answer 55

D) “With appropriate treatment and support, children with sickle cell disease can lead relatively normal lives.”

Rationale: With early diagnosis, proper management, and ongoing care, children with sickle cell disease can lead relatively normal lives despite potential complications.

Question 56

A 10-year-old child with sickle cell disease and a history of recurrent crises is seen for a routine check-up. The physician notes that the child’s growth has been slow.
What should the nurse do to assess the child’s nutritional status?

A) Perform a physical exam and provide general nutrition advice

B) Ask the parents for a 24-hour dietary recall and conduct a full nutritional assessment

C) Suggest a low-fat, low-sodium diet and monitor the child’s growth in follow-up visits

D) Refer the child to a dietitian for a complete nutritional plan without further assessment

Answer 56

B) Ask the parents for a 24-hour dietary recall and conduct a full nutritional assessment

Rationale: A 24-hour dietary recall and a full nutritional assessment are important to identify any deficiencies and ensure the child’s nutritional needs are being met.

Question 57

A 6-month-old infant with sickle cell disease is being admitted to the hospital. The parents are concerned about the potential risks of infection.
What should the nurse emphasize to the parents regarding infection prevention for this infant?

A) Infection is rarely a concern for infants with sickle cell disease.

B) The infant should start on penicillin prophylaxis immediately to prevent pneumococcal infections.

C) The infant does not need any vaccinations until age 2.

D) No special precautions are necessary unless the child is febrile.

Answer 57

B) The infant should start on penicillin prophylaxis immediately to prevent pneumococcal infections.

Rationale: Early administration of penicillin prophylaxis is essential to reduce the risk of life-threatening infections in infants with sickle cell disease.

Question 58

A 5-year-old child with sickle cell disease is receiving hydroxyurea treatment. The nurse is discussing potential side effects of the medication with the child’s parents.
Which of the following is the most important side effect to explain to the parents?

A) Increased risk of respiratory infections
B) Risk of excessive weight gain
C) Bone marrow suppression and the potential for lowered blood counts
D) Potential for gastrointestinal upset and diarrhea

Answer 58

C) Bone marrow suppression and the potential for lowered blood counts

Rationale: Hydroxyurea can suppress bone marrow, leading to low blood counts, and the parents should be educated on the need for regular blood tests to monitor this.

Question 59

A 2-year-old child with sickle cell disease is hospitalized due to an acute pain crisis. The nurse needs to assess the child’s pain level.
Which of the following is the most appropriate method for assessing pain in this child?

A) Use a numeric pain scale
B) Use the Wong-Baker FACES Pain Rating Scale
C) Ask the child’s parent about the severity of the pain
D) Rely on vital signs as a measure of pain severity

Answer 59

B) Use the Wong-Baker FACES Pain Rating Scale

Rationale: The Wong-Baker FACES Pain Rating Scale is appropriate for young children who may have difficulty verbalizing pain, helping them to express their pain level using faces.

Question 60

A child with sickle cell disease is receiving pain medication via patient-controlled analgesia (PCA). The nurse notices that the child is not using the PCA button for additional doses.
What should the nurse do first?

A) Ask the child if they are in pain and if the PCA is effective

B) Discontinue the PCA and administer the prescribed dose of pain medication via injection

C) Inform the physician that the PCA is not being used properly

D) Increase the PCA dose immediately to ensure the child’s comfort

Answer 60

A) Ask the child if they are in pain and if the PCA is effective

Rationale: The nurse should assess the child’s pain and comfort level before making adjustments to the PCA settings.

Question 61

A child with sickle cell disease is receiving a blood transfusion. The nurse notices the child is becoming restless and complaining of itching.
What is the most likely cause of these symptoms?

A) A normal response to blood transfusion
B) An allergic reaction to the transfusion
C) A sign of iron overload from frequent transfusions
D) A sign of acute chest syndrome

Answer 61

B) An allergic reaction to the transfusion

Rationale: Restlessness and itching can be symptoms of an allergic reaction to blood transfusions, which should be addressed immediately by stopping the transfusion and notifying the physician.

Question 62

A 9-year-old child with sickle cell disease is being seen for a follow-up appointment. The parents mention that the child is frequently absent from school due to hospitalizations.
What is the nurse’s most appropriate action?

A) Discuss the potential for academic tutoring and support during hospitalizations

B) Recommend reducing school attendance to avoid infection

C) Advise the parents to wait for the child to fully recover before resuming school

D) Suggest the parents avoid telling the school about the child’s condition

Answer 62

A) Discuss the potential for academic tutoring and support during hospitalizations

Rationale: The nurse should recommend academic support and tutoring to help the child keep up with their studies while managing their illness.

Question 63

Which of the following best describes the process that leads to sickle cell formation in patients with sickle cell disease?

a) Hemoglobin S becomes oxygenated, resulting in the formation of sickle-shaped cells.

b) Hemoglobin S loses its oxygen and crystallizes, forming rod-like structures that bend the erythrocyte into a crescent shape.

c) Red blood cells enlarge and lose their ability to carry oxygen efficiently.

d) Sickle-shaped erythrocytes adhere to endothelial cells, leading to vascular constriction and ischemia.

Answer 63

b) Hemoglobin S loses its oxygen and crystallizes, forming rod-like structures that bend the erythrocyte into a crescent shape.

Rationale: When hemoglobin S loses its oxygen, it becomes insoluble, forming rod-like structures that bend the erythrocyte into the characteristic sickle shape.

Question 64

What is the most common precipitating factor for a sickle cell crisis in children with sickle cell disease?

a) Dehydration and fever
b) Overhydration and high oxygen tension
c) Infection and hypoxia
d) Nutritional deficiency and high altitude

Answer 64

c) Infection and hypoxia

Rationale: Infections and hypoxia (low oxygen levels) are the primary triggers for sickle cell crises.

Question 65

A child with sickle cell disease develops a fever and is in severe pain. Which of the following would be the priority intervention for the nurse?

a) Administer oxygen and fluids to rehydrate the child.
b) Increase the child’s physical activity to promote circulation.
c) Provide analgesics as ordered and monitor vital signs.
d) Initiate blood transfusions immediately.

Answer 65

a) Administer oxygen and fluids to rehydrate the child.

Rationale: Administering oxygen and fluids is essential to alleviate hypoxia and dehydration, which are primary triggers for sickle cell crises.

Question 66

A nurse is educating a family with a child diagnosed with sickle cell disease. Which statement by the parent indicates a need for further teaching?

a) “I will make sure my child stays hydrated to prevent a crisis.”
b) “We should limit our child’s exposure to high altitudes to prevent sickling.”
c) “My child can play sports, but only if they are well-rested and hydrated.”
d) “Our child should avoid vaccines to reduce the risk of infections.”

Answer 66

b) “We should limit our child’s exposure to high altitudes to prevent sickling.”

Rationale: Vaccines are important for preventing infections, which can trigger a sickle cell crisis. The child should not avoid vaccinations.

Question 67

Which of the following conditions can increase blood viscosity, thereby contributing to a sickle cell crisis?

a) Increased fluid intake
b) High levels of physical activity
c) Fever and dehydration
d) Adequate oxygenation

Answer 67

c) Fever and dehydration

Rationale: Fever and dehydration increase blood viscosity, which can trigger a sickle cell crisis by reducing blood flow and oxygen delivery to tissues.

Question 68

Which of the following statements is accurate regarding the genetic nature of sickle cell disease?

a) Sickle cell disease occurs when both parents carry the sickle cell trait.

b) Sickle cell disease is inherited in an autosomal dominant pattern.

c) Sickle cell disease only occurs in individuals of African descent.

d) Sickle cell disease results from a recessive gene from one parent.

Answer 68

a) Sickle cell disease occurs when both parents carry the sickle cell trait.

Rationale: Sickle cell disease is inherited in an autosomal recessive pattern, meaning both parents must carry the sickle cell trait for the child to inherit the disease.

Question 69

A child is diagnosed with sickle cell disease. The parents are unsure how their child contracted the disease. Which explanation by the nurse is the most appropriate?

1. “Both the mother and the father have the sickle cell trait.”
2. “The mother has the trait, but the father doesn’t.”
3. “The father has the trait, but the mother doesn’t.”
4. “The mother has sickle cell disease, but the father doesn’t have the disease or the trait.”

Answer 69

1. “Both the mother and the father have the sickle cell trait.”

Rationale: Sickle cell disease is an autosomal recessive disorder; both parents must have the trait in order for a child to have the disease.

Question 70

The charge nurse on a pediatric unit is making a room assignment for a school-age child diagnosed with sickle cell disease, who is in splenic sequestration crisis. Which room assignment is most appropriate for this client?

1. Semiprivate room
2. Reverse-isolation room
3. Contact-isolation room
4. Private room

Answer 70

4. Private room

Rationale: Splenic sequestration can be life-threatening, and there is profound anemia. The child does not need an isolation room but should not be placed in a room with any child who may have an infectious illness. The private room is appropriate for this child.

Question 71

The nurse is providing care for an adolescent client who is experiencing pain related to a sickle cell crisis. Which medication does the nurse prepare to administer to this client?

1. Morphine sulfate
2. Meperidine
3. Acetaminophen
4. Ibuprofen

Answer 71

1. Morphine sulfate

Rationale: The pain during a sickling crisis is severe, and morphine is needed for pain control around the clock or by patient-controlled analgesia (PCA). Meperidine is not used for pain control for clients with sickle cell pain crisis because it could cause seizures. Acetaminophen or ibuprofen is used for mild pain and would not be effective for the severe pain experienced by a child in sickle cell pain crisis.

Question 72

The nurse is teaching parents how to prevent a sickle cell crisis in the child with sickle cell disease. Which precipitating factors to a sickle cell crisis will the nurse include in the explanation?
Select all that apply.

1. Fever
2. Dehydration
3. Regular exercise
4. Altitude
5. Increased fluid intake

Answer 72

1. Fever
2. Dehydration
3. Altitude

Question 73

The nurse is administering packed RBCs to a child with sickle cell disease (SCD). The nurse is monitoring for a transfusion reaction and knows it is most likely to occur during which time frame?

1. Six hours after the transfusion is given
2. Within the first 20 minutes of administration of the transfusion
3. At the end of the administration of the transfusion
4. Never; children with SCD do not have reactions.

Answer 73

2. Within the first 20 minutes of administration of the transfusion

Rationale: Blood reactions can occur as soon as the blood transfusion begins or within the first 20 minutes. The nurse should remain with the child for the first 20 minutes of the transfusion.

Question 74

The nurse is caring for a child who is in a sickle cell crisis and has severe pain. Which nursing intervention is the most appropriate for this child?

1. Giving comfort measures, such as back rubs
2. Suggesting diversional activities, such as coloring
3. Administering pain medication
4. Preparing the child for painful procedures

Answer 74

3. Administering pain medication

Rationale: Severe pain requires administration of pain medication for pain relief. Comfort measures and diversional activities are not effective against severe pain in children. Comfort measures should be given to every child and can be used after pain medication is given. A child in severe pain is not capable of participating in or enjoying diversional activities. Preparing the child for painful procedures is not appropriate when the child is already in pain.