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.
Sickle cell disease HbAS
Sickle cell anemia HbSS
Sickle cell syndrome HbSC
Hb S beta thalassemia
-Homozygous condition -Child is subject to sickle cell crisis
Sickle cell trait HbAS
-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
-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.
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?
"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."
Bacterial infections are the leading cause of death in young children with sickle cell disease.
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.
"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."
Dehydration can predispose an individual to a sickle cell crisis.
Once cells are sickled, they cannot resume a normal shape.
Which of the following is the PRIORITY intervention when caring for a patient coming to ER due to a sickle cell crisis?
Place the patient on a nasal cannula running at 2L
Start a normal saline IV
Take a CBC w/differential
Contact with the physician
Sickle cell anemia is an autonomic recessive genetic defect.
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.
"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."
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?
"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."
Which of the following patients is MOST LIKELY to have sickle cell disease?
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.
Which of the following is NOT a potential clinical manifestation of a vaso-occlusive sickle cell crisis?
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
What type of sickle cell crisis is the most common?
Vaso-occlusion (thrombotic)
Splenic sequestration
Aplastic crises
Acute chest syndrome
What conditions can precipitate a thrombotic (vaso-occlusion) sickle cell crisis?
Dehydration
Exposure to cold
Acidosis
Localized hypoxemia
Emotional or physical stress
What are the causes of a thrombotic (vaso-occlusion) sickle cell crisis?
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.
What are the clinical manifestations of a splenetic sequestration sickle cell crisis?
Hypovolemia
Shock
Pallor
Fatigue
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?
Stroke
Aplastic crisis
Cardiovascular collapse and death
Pulmonary failure and death
Why can splenic sequestration result in cardiovascular collapse?
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.
What are the clinical manifestations of an aplastic sickle cell crisis?
Aplastic sickle cell crises are often triggered by the influenza virus.
Which of the following is NOT a clinical manifestation of acute chest syndrome?
Cough
Chest and back pain
Dyspnea
Hypoxemia
Painful swelling of the hands and feet.
What is a common cause of hospitalization for sickle cell disease?
What can occur if acute chest syndrome is not treated?
Pulmonary infection
Pulmonary infarct
Fat embolism
Cardiovascular collapse
You can usually tell that a newborn has sickle cell disease immediately after birth.
Which of the following is UNTRUE about children with sickle cell disease?
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.
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?
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
Which of the following is NOT a proper intervention for a nurse treating a patient with dyspnea?
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.
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?
Dyspnea due to inadequate delivery of oxygen to cells, tissues, and organs,
Impaired circulation due to impaired flow of sickled RBCs.
Risk for infection due to inadequate circulation.
Pain due to vascular occlusion by RBCs.
Select the BEST interventions for a patient repeatedly presenting to the hospital with anemia.
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.
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?
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.
You are caring for a child with sickle cell disease that has tested positive for pneumonia.
Which of the following is your priority intervention?
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.
Which of the following is NOT an appropriate way to help a child with sickle cell disease with pain?
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.
In the U.S., newborn screening for sickle cell disease is mandatory.
Which of the following is TRUE about pharmacological therapy in children with sickle cell disease?
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.
What are the complications associated with repeated blood transfusions?
Hemosiderosis
Hypervolemia
Alloimmunization to red cell and platelet antigens
Frequent headaches
Infection
