Olivia McRitchie
Quiz por , criado more than 1 year ago

NCLEX Nursing (Pediatrics) Quiz sobre Exemplar 2.8: Sickle Cell Disease, criado por Olivia McRitchie em 06-11-2019.

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Exemplar 2.8: Sickle Cell Disease

Questão 1 de 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.

Selecione uma das seguintes:

  • Sickle cell disease HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explicação

Questão 2 de 38

1

What form of sickle cell disease does this describe?

-Homozygous condition
-Child is subject to sickle cell crisis

Selecione uma das seguintes:

  • Sickle cell trait HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explicação

Questão 3 de 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

Selecione uma das seguintes:

  • Sickle cell trait HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explicação

Questão 4 de 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.

Selecione uma das seguintes:

  • Sickle cell trait HbAS

  • Sickle cell anemia HbSS

  • Sickle cell syndrome HbSC

  • Hb S beta thalassemia

Explicação

Questão 5 de 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?

Selecione uma das seguintes:

  • "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."

Explicação

Questão 6 de 38

1

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

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 7 de 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.

Selecione uma ou mais das seguintes:

  • "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."

Explicação

Questão 8 de 38

1

Dehydration can predispose an individual to a sickle cell crisis.

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 9 de 38

1

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

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 10 de 38

1

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

Selecione uma das seguintes:

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

  • Start a normal saline IV

  • Take a CBC w/differential

  • Contact with the physician

Explicação

Questão 11 de 38

1

Sickle cell anemia is an autonomic recessive genetic defect.

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 12 de 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.

Selecione uma das seguintes:

  • "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."

Explicação

Questão 13 de 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?

Selecione uma das seguintes:

  • "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."

Explicação

Questão 14 de 38

1

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

Selecione uma das seguintes:

  • 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.

Explicação

Questão 15 de 38

1

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

Selecione uma das seguintes:

  • 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

Explicação

Questão 16 de 38

1

What type of sickle cell crisis is the most common?

Selecione uma das seguintes:

  • Vaso-occlusion (thrombotic)

  • Splenic sequestration

  • Aplastic crises

  • Acute chest syndrome

Explicação

Questão 17 de 38

1

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

Selecione uma ou mais das seguintes:

  • Dehydration

  • Exposure to cold

  • Acidosis

  • Localized hypoxemia

  • Fever

  • Emotional or physical stress

Explicação

Questão 18 de 38

1

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

Selecione uma das seguintes:

  • 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.

Explicação

Questão 19 de 38

1

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

Selecione uma ou mais das seguintes:

  • Profound anemia

  • Hypovolemia

  • Shock

  • Pallor

  • Fatigue

Explicação

Questão 20 de 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?

Selecione uma das seguintes:

  • Stroke

  • Aplastic crisis

  • Cardiovascular collapse and death

  • Pulmonary failure and death

Explicação

Questão 21 de 38

1

Why can splenic sequestration result in cardiovascular collapse?

Selecione uma das seguintes:

  • 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.

Explicação

Questão 22 de 38

1

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

Selecione uma ou mais das seguintes:

  • Profound anemia

  • Pallor

  • Fatigue

  • Shock

  • Hypovolemia

Explicação

Questão 23 de 38

1

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

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 24 de 38

1

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

Selecione uma das seguintes:

  • Fever

  • Cough

  • Chest and back pain

  • Dyspnea

  • Hypoxemia

  • Painful swelling of the hands and feet.

Explicação

Questão 25 de 38

1

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

Selecione uma das seguintes:

  • Vaso-occlusion (thrombotic)

  • Splenic sequestration

  • Aplastic crises

  • Acute chest syndrome

Explicação

Questão 26 de 38

1

What can occur if acute chest syndrome is not treated?

Selecione uma ou mais das seguintes:

  • Pulmonary infection

  • Pulmonary infarct

  • Fat embolism

  • Cardiovascular collapse

  • Stroke

Explicação

Questão 27 de 38

1

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

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 28 de 38

1

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

Selecione uma das seguintes:

  • 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.

Explicação

Questão 29 de 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?

Selecione uma das seguintes:

  • 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

Explicação

Questão 30 de 38

1

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

Selecione uma das seguintes:

  • 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.

Explicação

Questão 31 de 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?

Selecione uma das seguintes:

  • 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.

Explicação

Questão 32 de 38

1

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

Selecione uma ou mais das seguintes:

  • 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.

Explicação

Questão 33 de 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?

Selecione uma das seguintes:

  • 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.

Explicação

Questão 34 de 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?

Selecione uma das seguintes:

  • 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.

Explicação

Questão 35 de 38

1

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

Selecione uma das seguintes:

  • 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.

Explicação

Questão 36 de 38

1

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

Selecione uma das opções:

  • VERDADEIRO
  • FALSO

Explicação

Questão 37 de 38

1

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

Selecione uma das seguintes:

  • 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.

Explicação

Questão 38 de 38

1

What are the complications associated with repeated blood transfusions?

Selecione uma ou mais das seguintes:

  • Hemosiderosis

  • Hypervolemia

  • Alloimmunization to red cell and platelet antigens

  • Frequent headaches

  • Infection

Explicação

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