Olivia McRitchie
Quiz by , created more than 1 year ago

NCLEX Nursing (Pediatrics) Quiz on Exemplar 2.8: Sickle Cell Disease, created by Olivia McRitchie on 06/11/2019.

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Olivia McRitchie
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Exemplar 2.8: Sickle Cell Disease

Question 1 of 38

1

What form of sickle cell disease does this describe?

-Most common in the US
-Heterozygous condition in which child inherits one sickle cell gene and one normal hemoglobin gene.
-Child is carrier and rarely has symptoms.

Select one of the following:

  • Sickle cell disease HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explanation

Question 2 of 38

1

What form of sickle cell disease does this describe?

-Homozygous condition
-Child is subject to sickle cell crisis

Select one of the following:

  • Sickle cell trait HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explanation

Question 3 of 38

1

What form of sickle cell disease does this describe?

-The child inherits a sickle cell gene from one parent and an abnormal hemoglobin gene from the other.
-Most often produces a less severe form of SCD

Select one of the following:

  • Sickle cell trait HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explanation

Question 4 of 38

1

What form of sickle cell disease does this describe?

-Child inherits a sickle cell gene from one parent and a gene for beta thalassemia from the other.
-Manifestations of SCD can range from mild to severe.

Select one of the following:

  • Sickle cell trait HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explanation

Question 5 of 38

1

You are delivering teaching to the parents of a child newly diagnosed with sickle cell anemia.

Which of the following statements shows that MORE teaching is needed?

Select one of the following:

  • "This disorder happens when some of the red blood cells get less oxygen and take on a sickle shape, meaning that they have a crescent shape."

  • "Red blood cells that become sickles become unable to flex. They can block small blood vessels and cause oxygen to not reach certain tissues."

  • "My child may experience blocking of his spleen. This may lead to it being removed, which causes the immune system to become lower."

  • "My child's red blood cells shouldn't become sickled unless he's feverish or placed in physically stressful situations."

Explanation

Question 6 of 38

1

Bacterial infections are the leading cause of death in young children with sickle cell disease.

Select one of the following:

  • True
  • False

Explanation

Question 7 of 38

1

Select the statements that show that the parents of a child with sickle cell anemia understands how to prevent a sickle cell (vaso-occlusive) crisis.

Select one or more of the following:

  • "Fevers can cause our child to be hospitalized because of a sickle cell crisis."

  • "We should teach our child relaxation techniques very early on in life. Physical and emotional stress can cause a crisis."

  • "Visiting our family members in Colorado shouldn't be a huge problem."

  • "Our child's asthma is so mild that we don't even keep an albuterol inhaler around anymore."

  • "We're so close to the school that our child walks to school. We just need to bundle him up more when it's cold outside."

Explanation

Question 8 of 38

1

Dehydration can predispose an individual to a sickle cell crisis.

Select one of the following:

  • True
  • False

Explanation

Question 9 of 38

1

Once cells are sickled, they cannot resume a normal shape.

Select one of the following:

  • True
  • False

Explanation

Question 10 of 38

1

Which of the following is the PRIORITY intervention when caring for a patient coming to ER due to a sickle cell crisis?

Select one of the following:

  • Place the patient on a nasal cannula running at 2L

  • Start a normal saline IV

  • Take a CBC w/differential

  • Contact with the physician

Explanation

Question 11 of 38

1

Sickle cell anemia is an autonomic recessive genetic defect.

Select one of the following:

  • True
  • False

Explanation

Question 12 of 38

1

You have a teenage patient with sickle cell anemia in the hospital for a sickle-cell crisis. The patient is in an anatomy and physiology class, and asks you what happens to her red blood cells biologically when she doesn't have enough oxygen.

Select the MOST ACCURATE teaching to give to this patient.

Select one of the following:

  • "You have 2 copies of a gene called Hb S. This changes the structure of something in hemoglobin called the 'beta chain'. When you don't get enough oxygen, Hb S becomes rod-like and forms long chains that deform the red blood cell."

  • "You have 2 copies of a gene called Hb S. This slightly changes the structure of your red blood cells.

  • "You have 1 copy of a gene called Hb S. This changes your hemoglobin so that it forms rod-like chains and doesn't carry oxygen correctly."

  • "You have 1 copy of a gene called Hb S. This changes the structure of the beta chain in hemoglobin. When you don't get enough oxygen, Hb S crystallizes into rod-like structures that form long chains which deform the erythrocyte."

Explanation

Question 13 of 38

1

You are explaining what a sickle-cell crisis is to the parents of a child recently diagnosed with sickle cell anemia.

Which of the following statements shows that teaching has been received?

Select one of the following:

  • "Because our child's red blood cells become sickle-shaped when they lack oxygen, they clump together and stop blood flow in smaller blood vessels, leading to oxygen loss in these tissues."

  • "Because our child's red blood cells are always sickle-shaped, they clump together and stop blood flow in smaller blood vessels, leading to oxygen loss in these tissues."

  • "Because our child's red blood cells become sickle-shaped when they lack oxygen and have no possibility of going back to normal, they clump together and stop blood flow in smaller blood vessels, leading to oxygen loss in these tissues."

  • "Because our child's red blood cells become sickle-shaped when they don't have enough fluid, they clump together and stop blood flow in smaller blood vessels, leading to oxygen loss in these tissues."

Explanation

Question 14 of 38

1

Which of the following patients is MOST LIKELY to have sickle cell disease?

Select one of the following:

  • A child with 2 parents who are directly from South Africa

  • A child with one parent from Ethiopia and one parent from Jamaica.

  • A mixed Ugandan-Swedish child.

  • A mixed Ugandan-Saudi Arabian child.

Explanation

Question 15 of 38

1

Which of the following is NOT a potential clinical manifestation of a vaso-occlusive sickle cell crisis?

Select one of the following:

  • Stroke, caused by cerebral occlusion.

  • Extreme pain

  • Fever

  • Tissue engorgement

  • Painful swelling of joints in hands and feet

  • Severe abdominal pain

  • Priapism (in males)

  • Profound anemia

Explanation

Question 16 of 38

1

What type of sickle cell crisis is the most common?

Select one of the following:

  • Vaso-occlusion (thrombotic)

  • Splenic sequestration

  • Aplastic crises

  • Acute chest syndrome

Explanation

Question 17 of 38

1

What conditions can precipitate a thrombotic (vaso-occlusion) sickle cell crisis?

Select one or more of the following:

  • Dehydration

  • Exposure to cold

  • Acidosis

  • Localized hypoxemia

  • Fever

  • Emotional or physical stress

Explanation

Question 18 of 38

1

What are the causes of a thrombotic (vaso-occlusion) sickle cell crisis?

Select one of the following:

  • Stasis of blood with clumping of cells in the microcirculation, ischemia, and hypoxemia.

  • Pooling of blood in the spleen

  • Diminished production and increased destruction of red blood cells.

  • Medical professionals are unsure of the cause.

Explanation

Question 19 of 38

1

What are the clinical manifestations of a splenetic sequestration sickle cell crisis?

Select one or more of the following:

  • Profound anemia

  • Hypovolemia

  • Shock

  • Pallor

  • Fatigue

Explanation

Question 20 of 38

1

You have a young child with sickle cell disease that has profound anemia, hypovolemia, severe abdominal pain, and shock. You suspect that the patient may have splenic sequestration.

What do you know can result from this condition if not treated promptly?

Select one of the following:

  • Stroke

  • Aplastic crisis

  • Cardiovascular collapse and death

  • Pulmonary failure and death

Explanation

Question 21 of 38

1

Why can splenic sequestration result in cardiovascular collapse?

Select one of the following:

  • It's caused by blood pooling in the spleen, and the spleen can only hold so much of the body's blood supply that cardiovascular collapse can result.

  • It causes stasis of blood with clumping of cells in the microcirculation, leading to thrombosis and infarction of cardiac tissue and resulting in cardiac arrest.

  • It causes diminished production and increased destruction of red blood cells, leading to profound enough anemia to cause shock and cardiovascular collapse.

  • It causes pulmonary infiltrates of abnormal blood cells, leading to cardiovascular and pulmonary collapse.

Explanation

Question 22 of 38

1

What are the clinical manifestations of an aplastic sickle cell crisis?

Select one or more of the following:

  • Profound anemia

  • Pallor

  • Fatigue

  • Shock

  • Hypovolemia

Explanation

Question 23 of 38

1

Aplastic sickle cell crises are often triggered by the influenza virus.

Select one of the following:

  • True
  • False

Explanation

Question 24 of 38

1

Which of the following is NOT a clinical manifestation of acute chest syndrome?

Select one of the following:

  • Fever

  • Cough

  • Chest and back pain

  • Dyspnea

  • Hypoxemia

  • Painful swelling of the hands and feet.

Explanation

Question 25 of 38

1

What is a common cause of hospitalization for sickle cell disease?

Select one of the following:

  • Vaso-occlusion (thrombotic)

  • Splenic sequestration

  • Aplastic crises

  • Acute chest syndrome

Explanation

Question 26 of 38

1

What can occur if acute chest syndrome is not treated?

Select one or more of the following:

  • Pulmonary infection

  • Pulmonary infarct

  • Fat embolism

  • Cardiovascular collapse

  • Stroke

Explanation

Question 27 of 38

1

You can usually tell that a newborn has sickle cell disease immediately after birth.

Select one of the following:

  • True
  • False

Explanation

Question 28 of 38

1

Which of the following is UNTRUE about children with sickle cell disease?

Select one of the following:

  • They can experience chest tightness and shortness of breath, which are diagnostic for acute chest syndrome.

  • Sickling is inhibited by high levels of fetal hemoglobin, so affected children are usually asymptomatic until 4-6 months of age.

  • 10% of children with sickle cell disease will have a stroke.

  • Children with the sickle cell trait will usually develop minor symptoms when confronted with abnormally low oxygen conditions.

Explanation

Question 29 of 38

1

A child presents to the ED with hypoxia, lethargy, fatigue, shortness of breath, tachypnea, and decreased O2 stat.

What clinical manifestation of sickle cell disease is this child experiencing?

Select one of the following:

  • Dyspnea due to inadequate delivery of oxygen to cells, tissues, and organs.

  • Anemia due to increased RBC destruction, impaired RBC production, or both.

  • Impaired circulation due to impaired flow of sickled RBCs

  • Risk for infection due to inadequate circulation

  • Pain due to vascular occlusion by RBCs

Explanation

Question 30 of 38

1

Which of the following is NOT a proper intervention for a nurse treating a patient with dyspnea?

Select one of the following:

  • Place patient on a nasal cannula running at 2L.

  • Elevate head of bed until patient can breathe comfortably.

  • Obtain consent to administer a blood transfusion.

  • Monitor VS, including O2 stat, and auscultate lung sounds every 4 hrs, or less as indicated.

  • Discuss with the physician the possibility of bone marrow or stem cell transplantation.

Explanation

Question 31 of 38

1

You have a child presenting with hypoxia, lethargy, fatigue, pallor, shortness of breath, dyspnea, and tachycardia. Labs were taken, and the RBC and Hgb was low.

What clinical manifestation is this child presenting with?

Select one of the following:

  • Dyspnea due to inadequate delivery of oxygen to cells, tissues, and organs,

  • Anemia due to increased RBC destruction, impaired RBC production, or both.

  • Impaired circulation due to impaired flow of sickled RBCs.

  • Risk for infection due to inadequate circulation.

  • Pain due to vascular occlusion by RBCs.

Explanation

Question 32 of 38

1

Select the BEST interventions for a patient repeatedly presenting to the hospital with anemia.

Select one or more of the following:

  • Obtain consent to administer a blood transfusion.

  • Discuss with the physician the possibility of a bone marrow or stem cell transplant.

  • Elevate the head of the bed.

  • Begin an IV line and obtain an order for maintenance fluids.

  • Encourage the client to adhere to current vaccination recommendations.

Explanation

Question 33 of 38

1

You have a 3-year-old child presenting to the ED with impaired oxygenation, tachypnea, tachycardia, and edema. A CT scan shows that the child has splenic sequestration. In addition, the child's hands and feet are swollen, and the child is in pain and has a fever.

Which of the following is NOT an appropriate intervention for this patient?

Select one of the following:

  • Obtain consent for a blood transfusion.

  • Open an IV line and obtain an order for maintenance fluids.

  • Politely ask that the parents not argue in front of their child, as it seems to upset him a great deal.

  • Prepare the child for an emergency splenectomy.

  • Alleviate pain with ice packs.

Explanation

Question 34 of 38

1

You are caring for a child with sickle cell disease that has tested positive for pneumonia.

Which of the following is your priority intervention?

Select one of the following:

  • Administer O2 via nasal cannula set at 2 L/min.

  • Administer antibiotics, as ordered.

  • Encourage the parents to continue the vaccine regimen.

  • Initiate droplet precautions.

Explanation

Question 35 of 38

1

Which of the following is NOT an appropriate way to help a child with sickle cell disease with pain?

Select one of the following:

  • Encourage the parents to keep giving their child oral fluids.

  • Start an IV line and obtain orders for maintenance fluids.

  • Obtain orders for PRN IV push or IM morphine.

  • Obtain an order for supplemental oxygen.

  • Administer hydroxyurea, as ordered.

  • Encourage the parents to massage the places on the child's body where it hurts.

  • Raise the head of the bed to a semi-Fowler's position.

Explanation

Question 36 of 38

1

In the U.S., newborn screening for sickle cell disease is mandatory.

Select one of the following:

  • True
  • False

Explanation

Question 37 of 38

1

Which of the following is TRUE about pharmacological therapy in children with sickle cell disease?

Select one of the following:

  • Oxygen is only implicated if the child is experiencing dyspnea.

  • Oral and IV fluids can prevent crises, but they do not work as a form of treatment when a child is having a crisis.

  • Hydroxyurea is a cytotoxic drug that decreases production of abnormal blood cells, but it has a long enough list of side effects that it's rarely given.

  • Treatment in children between 2 months and 5 years of age usually includes daily administration of prophylactic penicillin.

  • Pain management in these children is about the same as it is with any other patient.

Explanation

Question 38 of 38

1

What are the complications associated with repeated blood transfusions?

Select one or more of the following:

  • Hemosiderosis

  • Hypervolemia

  • Alloimmunization to red cell and platelet antigens

  • Frequent headaches

  • Infection

Explanation