Question 1

A couple who recently married and want to have children ask the nurse what the chances are that their children will inherit thalassemia from them,as they both are carriers.What information from the nurse is most accurate?

Accepted Answer

Thalassemia is an autosomal recessive disorder.Each of their children has a 25% chance of having only normal genes,a 25% chance of inheriting both defective genes from the parents and expressing the disease,and a 50% chance of being a carrier.

Question 2

Which health promotion measure does the nurse teach as being most important for the child with sickle cell disease?

Accepted Answer

All options are appropriate for the child with sickle cell disease; however,vaccinations are vital to prevent sepsis and death from preventable diseases.

Question 3

A child presents to the emergency department with sickle cell crisis.Which intervention does the nurse perform first?

Accepted Answer

All interventions are appropriate for this child.However,airway and breathing come first,so the nurse administers oxygen then starts an IV.

Question 4

An acutely ill,anemic child's peripheral blood smear shows small,dense,spherical RBCs.What action by the nurse takes priority?

Accepted Answer

The peripheral blood smear indicates spherocytosis,which,when acute,is treated with transfusions.The nurse ensures informed consent is obtained and present on the chart.Assessing and treating pain is important but does not take priority.Chelation therapy is not indicated in the question.A bone marrow transplant may or may not be considered,but is not the primary need of this child.

Question 5

A 2-year-old child's hemoglobin is 8.2 g/dL.What action by the nurse is best?

Accepted Answer

The normal hemoglobin for a child this age is 10.55-12.7 g/dL,so this child is somewhat anemic.The nurse should assess for other manifestations of anemia,including normal activity level.The findings should be documented,but this is not the only action that the nurse should take.The provider needs to be notified,but it does not have to be done immediately,as this is not an emergency.After a full evaluation,the provider may or may not want to repeat the laboratory work in 6 months.

Question 6

A nurse is assessing an infant for the most common type of anemia worldwide.What action by the nurse is most helpful?

Accepted Answer

The most common type of anemia worldwide is iron-deficiency anemia,which can be caused by ingesting non-iron-fortified formula if the child is not breastfed.This type of anemia is not genetic.Pallor,either of the skin or mucous membranes,would be seen in any type of anemia.Range of motion of the hips or shoulders is an important assessment in sickle cell disease,in which avascular necrosis can occur.

Question 7

A child with sickle cell disease is receiving hypertransfusion therapy,and the current serum ferritin level is 1,035 µg/L.What medication does the nurse prepare to administer?

Accepted Answer

A complication of hypertransfusion is iron overload,diagnosed with a serum ferritin level of greater than 1,000 µg/L.The treatment is chelation therapy with an agent such as deferoxamine.Iron would be contraindicated.Lasix is given for fluid overload.Morphine is given for pain.

Question 8

A nurse working in pediatrics learns that the normal hemoglobin value for an infant is high at birth,then decreases by 2 months of age before increasing again as the child grows.The nurse knows the reason for this shift is which of the following?

Accepted Answer

The fetus needs a higher hemoglobin level to compensate for the relatively low-oxygen environment of the uterus.The other answers are incorrect.

Question 9

A nursing student asks the instructor why he was marked off on his care plan when explaining a low hemoglobin level as being caused by "anemia." What response by the instructor is best?

Accepted Answer

Anemia is a symptom that can be caused by many disease states.It is not a disease that explains low hemoglobin.The other answers are incorrect.

Question 10

An adolescent has been taught to administer replacement factors for bleeding episodes related to hemophilia.What action by the teen indicates that further instruction is needed?

Accepted Answer

Replacement factors are given intravenously.The other actions are appropriate.

Question 11

A child is taking desmopressin acetate (DDAVP)for von Willebrand's disease.What teaching about this medication does the nurse provide?

Accepted Answer

DDAVP can cause hypervolemia and hyponatremia.The child may show a rapid weight gain,which should be reported.Avoiding aspirin and using ice packs for nosebleeds are care measures for the disease,not the medication.DDAVP is given intranasally for this condition.

Question 12

The parents of an 8-year-old child with sickle cell anemia call the clinic to report that the child developed chest pain after playing soccer.What advice from the nurse is most appropriate?

Accepted Answer

In sickle cell disease,the abnormally shaped RBCs are sticky and adhere to the blood vessel walls,creating obstructions to circulation.This creates the potential for tissue ischemia and death.The child could be having a heart attack and needs immediate evaluation.

Question 13

A child with chronic immune thrombocytopenia presents to the emergency department,where the parents report a 3-day history of severe headache and recent change in mental status.What diagnostic test does the nurse prepare to facilitate as the priority?

Accepted Answer

A child with ITP is at risk for intracerebral hemorrhage,manifested by changes in level of consciousness,headaches,visual changes,ataxia,and/or slurred speech.The diagnostic test of choice is a CT scan of the head.A lumbar puncture is often used to diagnose meningitis; because this child does not have a fever,meningitis is a low probability.Platelet count and complete blood count (including WBCs)will be done,but the priority is to obtain a head CT.

Question 14

A child has been hospitalized with a sickle cell crisis and given morphine sulfate (Duramorph)for severe pain.On assessment 45 minutes later,the child appears to be sleeping quietly with a respiratory rate of 6 breaths/minute.What action by the nurse is most appropriate?

Accepted Answer

This child's respiratory rate is dangerously low,brought on by the narcotic analgesic.The nurse should prepare to administer Narcan per protocol.Letting the child sleep could lead to respiratory arrest,although the findings and subsequent actions should be documented.The provider should be notified afterward to adjust the next dose of pain medication.The child may or may not be able to cooperate with deep breathing instructions.

Question 15

A teenager is hospitalized with sickle cell disease and vaso-occlusive crisis.What pain medication regimen does the nurse assist the patient with?

Accepted Answer

A teenager is able to manage his or her own pain control,so a PCA pump is ideal.Morphine is often considered the drug of choice in sickle cell crises.Tylenol would be ineffective for pain this severe.Demerol is avoided due to its side effects.Toradol is a good choice; however,it is given parenterally for severe,acute pain.

Question 16

The nurse working in the pediatric intensive care unit understands that the priority for treating disseminated intravascular coagulation (DIC)is to do which of the following?

Accepted Answer

There are several treatment modalities to support the patient in DIC,but because this disorder is always secondary to another problem,treating the primary medical condition is the priority.Antibiotics alone are not used in DIC.Organ donation requests are premature when looking at treatment options.Transfusions may be required.

Question 17

A child has mild anemia and the parent asks why this makes the child have difficulty concentrating.What response by the nurse is best?

Accepted Answer

Anemia leads to decreased oxygenation of body tissues,including the brain.A lowered cerebral oxygen concentration can lead to dizziness and difficulty concentrating.Stating that all sick children have this problem is inaccurate and vague.The child may be tired,but this answer is also vague and does not really address the question.Describing the possibility of another medical problem is not warranted at this time.

Question 18

A toddler had a minor fall and now has a swollen,bruised,painful knee.What diagnostic test is most important for the nurse to educate the parents about?

Accepted Answer

The child has manifestations consistent with hemophilia.The most important diagnostic testing for this disease is a direct assay of plasma factor activity level for hemophilia A and B.A CBC will also most certainly be done,as will a platelet count.Plasma ferritin measures iron and is not warranted.

Question 19

A nurse is assessing a child who presents to the pediatric clinic,where the parent reports new bruising and petechiae.What question asked by the nurse would elicit the most helpful information?

Accepted Answer

These manifestations may be those of acute immune thrombocytopenia (ITP).This often follows a viral infection,so asking about recent infections is most appropriate.The other questions are not related to this disease.

Question 20

The nurse has educated parents on administration of iron to their child.What statement by the parents indicates a need for further instruction?

Accepted Answer

Black,tarry stools are a common side effect of iron and the parents need not call the provider.The other statements show good understanding of iron and its administration.

Quiz 32: Caring for the Child With a Hematological Condition