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Exemplar 2.8: Sickle Cell Disease

Deleted user 38 questions Updated 4 Dec 2019

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1What 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.
1. ASickle cell disease HbAS
2. BSickle cell anemia HbSS
3. CSickle cell syndrome HbSC
4. DHb S beta thalassemia
2What form of sickle cell disease does this describe?

-Homozygous condition
-Child is subject to sickle cell crisis
1. ASickle cell trait HbAS
2. BSickle cell anemia HbSS
3. CSickle cell syndrome HbSC
4. DHb S beta thalassemia
3What 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
1. ASickle cell trait HbAS
2. BSickle cell anemia HbSS
3. CSickle cell syndrome HbSC
4. DHb S beta thalassemia
4What 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.
1. ASickle cell trait HbAS
2. BSickle cell anemia HbSS
3. CSickle cell syndrome HbSC
4. DHb S beta thalassemia
5You 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?
1. A"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."
2. B"Red blood cells that become sickles become unable to flex. They can block small blood vessels and cause oxygen to not reach certain tissues."
3. C"My child may experience blocking of his spleen. This may lead to it being removed, which causes the immune system to become lower."
4. D"My child's red blood cells shouldn't become sickled unless he's feverish or placed in physically stressful situations."
6Bacterial infections are the leading cause of death in young children with sickle cell disease.
1. ATrue
2. BFalse
7Select the statements that show that the parents of a child with sickle cell anemia understands how to prevent a sickle cell (vaso-occlusive) crisis.
1. A"Fevers can cause our child to be hospitalized because of a sickle cell crisis."
2. B"We should teach our child relaxation techniques very early on in life. Physical and emotional stress can cause a crisis."
3. C"Visiting our family members in Colorado shouldn't be a huge problem."
4. D"Our child's asthma is so mild that we don't even keep an albuterol inhaler around anymore."
5. E"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."
8Dehydration can predispose an individual to a sickle cell crisis.
1. ATrue
2. BFalse
9Once cells are sickled, they cannot resume a normal shape.
1. ATrue
2. BFalse
10Which of the following is the PRIORITY intervention when caring for a patient coming to ER due to a sickle cell crisis?
1. APlace the patient on a nasal cannula running at 2L
2. BStart a normal saline IV
3. CTake a CBC w/differential
4. DContact with the physician

11Sickle cell anemia is an autonomic recessive genetic defect.
1. ATrue
2. BFalse
12You 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.
1. A"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."
2. B"You have 2 copies of a gene called Hb S. This slightly changes the structure of your red blood cells.
3. C"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."
4. D"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."
13You 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?
1. A"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."
2. B"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."
3. C"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."
4. D"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."
14Which of the following patients is MOST LIKELY to have sickle cell disease?
1. AA child with 2 parents who are directly from South Africa
2. BA child with one parent from Ethiopia and one parent from Jamaica.
3. CA mixed Ugandan-Swedish child.
4. DA mixed Ugandan-Saudi Arabian child.
15Which of the following is NOT a potential clinical manifestation of a vaso-occlusive sickle cell crisis?
1. AStroke, caused by cerebral occlusion.
2. BExtreme pain
3. CFever
4. DTissue engorgement
5. EPainful swelling of joints in hands and feet
6. FSevere abdominal pain
7. GPriapism (in males)
8. HProfound anemia
16What type of sickle cell crisis is the most common?
1. AVaso-occlusion (thrombotic)
2. BSplenic sequestration
3. CAplastic crises
4. DAcute chest syndrome
17What conditions can precipitate a thrombotic (vaso-occlusion) sickle cell crisis?
1. ADehydration
2. BExposure to cold
3. CAcidosis
4. DLocalized hypoxemia
5. EFever
6. FEmotional or physical stress
18What are the causes of a thrombotic (vaso-occlusion) sickle cell crisis?
1. AStasis of blood with clumping of cells in the microcirculation, ischemia, and hypoxemia.
2. BPooling of blood in the spleen
3. CDiminished production and increased destruction of red blood cells.
4. DMedical professionals are unsure of the cause.
19What are the clinical manifestations of a splenetic sequestration sickle cell crisis?
1. AProfound anemia
2. BHypovolemia
3. CShock
4. DPallor
5. EFatigue
20You 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?
1. AStroke
2. BAplastic crisis
3. CCardiovascular collapse and death
4. DPulmonary failure and death
21Why can splenic sequestration result in cardiovascular collapse?
1. AIt'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.
2. BIt causes stasis of blood with clumping of cells in the microcirculation, leading to thrombosis and infarction of cardiac tissue and resulting in cardiac arrest.
3. CIt causes diminished production and increased destruction of red blood cells, leading to profound enough anemia to cause shock and cardiovascular collapse.
4. DIt causes pulmonary infiltrates of abnormal blood cells, leading to cardiovascular and pulmonary collapse.
22What are the clinical manifestations of an aplastic sickle cell crisis?
1. AProfound anemia
2. BPallor
3. CFatigue
4. DShock
5. EHypovolemia
23Aplastic sickle cell crises are often triggered by the influenza virus.
1. ATrue
2. BFalse
24Which of the following is NOT a clinical manifestation of acute chest syndrome?
1. AFever
2. BCough
3. CChest and back pain
4. DDyspnea
5. EHypoxemia
6. FPainful swelling of the hands and feet.
25What is a common cause of hospitalization for sickle cell disease?
1. AVaso-occlusion (thrombotic)
2. BSplenic sequestration
3. CAplastic crises
4. DAcute chest syndrome
26What can occur if acute chest syndrome is not treated?
1. APulmonary infection
2. BPulmonary infarct
3. CFat embolism
4. DCardiovascular collapse
5. EStroke
27You can usually tell that a newborn has sickle cell disease immediately after birth.
1. ATrue
2. BFalse
28Which of the following is UNTRUE about children with sickle cell disease?
1. AThey can experience chest tightness and shortness of breath, which are diagnostic for acute chest syndrome.
2. BSickling is inhibited by high levels of fetal hemoglobin, so affected children are usually asymptomatic until 4-6 months of age.
3. C10% of children with sickle cell disease will have a stroke.
4. DChildren with the sickle cell trait will usually develop minor symptoms when confronted with abnormally low oxygen conditions.
29A 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?
1. ADyspnea due to inadequate delivery of oxygen to cells, tissues, and organs.
2. BAnemia due to increased RBC destruction, impaired RBC production, or both.
3. CImpaired circulation due to impaired flow of sickled RBCs
4. DRisk for infection due to inadequate circulation
5. EPain due to vascular occlusion by RBCs
30Which of the following is NOT a proper intervention for a nurse treating a patient with dyspnea?
1. APlace patient on a nasal cannula running at 2L.
2. BElevate head of bed until patient can breathe comfortably.
3. CObtain consent to administer a blood transfusion.
4. DMonitor VS, including O2 stat, and auscultate lung sounds every 4 hrs, or less as indicated.
5. EDiscuss with the physician the possibility of bone marrow or stem cell transplantation.
31You 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?
1. ADyspnea due to inadequate delivery of oxygen to cells, tissues, and organs,
2. BAnemia due to increased RBC destruction, impaired RBC production, or both.
3. CImpaired circulation due to impaired flow of sickled RBCs.
4. DRisk for infection due to inadequate circulation.
5. EPain due to vascular occlusion by RBCs.
32Select the BEST interventions for a patient repeatedly presenting to the hospital with anemia.
1. AObtain consent to administer a blood transfusion.
2. BDiscuss with the physician the possibility of a bone marrow or stem cell transplant.
3. CElevate the head of the bed.
4. DBegin an IV line and obtain an order for maintenance fluids.
5. EEncourage the client to adhere to current vaccination recommendations.
33You 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?
1. AObtain consent for a blood transfusion.
2. BOpen an IV line and obtain an order for maintenance fluids.
3. CPolitely ask that the parents not argue in front of their child, as it seems to upset him a great deal.
4. DPrepare the child for an emergency splenectomy.
5. EAlleviate pain with ice packs.
34You are caring for a child with sickle cell disease that has tested positive for pneumonia.

Which of the following is your priority intervention?
1. AAdminister O2 via nasal cannula set at 2 L/min.
2. BAdminister antibiotics, as ordered.
3. CEncourage the parents to continue the vaccine regimen.
4. DInitiate droplet precautions.
35Which of the following is NOT an appropriate way to help a child with sickle cell disease with pain?
1. AEncourage the parents to keep giving their child oral fluids.
2. BStart an IV line and obtain orders for maintenance fluids.
3. CObtain orders for PRN IV push or IM morphine.
4. DObtain an order for supplemental oxygen.
5. EAdminister hydroxyurea, as ordered.
6. FEncourage the parents to massage the places on the child's body where it hurts.
7. GRaise the head of the bed to a semi-Fowler's position.
36In the U.S., newborn screening for sickle cell disease is mandatory.
1. ATrue
2. BFalse
37Which of the following is TRUE about pharmacological therapy in children with sickle cell disease?
1. AOxygen is only implicated if the child is experiencing dyspnea.
2. BOral and IV fluids can prevent crises, but they do not work as a form of treatment when a child is having a crisis.
3. CHydroxyurea 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.
4. DTreatment in children between 2 months and 5 years of age usually includes daily administration of prophylactic penicillin.
5. EPain management in these children is about the same as it is with any other patient.
38What are the complications associated with repeated blood transfusions?
1. AHemosiderosis
2. BHypervolemia
3. CAlloimmunization to red cell and platelet antigens
4. DFrequent headaches
5. EInfection

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Description: User has deleted their subject information Quiz on Exemplar 2.8: Sickle Cell Disease, created by Deleted user on 08/11/2019.
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Language: English
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History: Created on 8 Nov 2019 · updated on 4 Dec 2019
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Exemplar 2.8: Sickle Cell Disease

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