Sickle Cell
c
A child with suspected sickle cell disease (SCD) is in the clinic for laboratory studies. The parents ask the nurse what results will tell the physician that their child has SCD. The nurse responds that which is increased in this disease? a Hemoglobin b Platelet count c Reticulocyte count d Hematocrit
a
A client and her husband both carry the genetic trait for sickle cell disease (SCD). The client asks the nurse practitioner, "What is the risk that we will have a child with sickle cell disease?" Which response by the nurse practitioner is most accurate? a "There is a 25 percent chance that any of your children will have SCD." b "All of your children will have the SCD trait, but none will have SCD." c "All of your children will have some form of SCD, though severity will vary." d "There is a 50 percent chance that any of your children will have SCD."
a
A nurse admitting a 7-year-old client experiencing sickle cell crisis is planning care based on which of the diagnoses? a Delayed Growth and Development b Risk for Bleeding c Ineffective Airway Clearance d Risk for Constipation
a
A pregnant woman tells the nurse that there is a history of sickle cell disease in her family and she is afraid that the baby will have the disease. Following the teaching, what does the nurse expect the expectant mother to know? a Both the mother and father must carry the gene for the baby to be affected. b Genetic testing will be needed to determine if the baby is affected. c The baby needs only one parent to be a carrier to be affected. d Sickle cell is a male disease and would be passed on by the baby's father.
4-6
Because sickling is inhibited by high levels of fetal hemoglobin, children with SCD usually are asymptomatic until ___-____ months of age
crisis
Clients experiencing sickle cell _________ may experience: An episode that lasts 4 - 6 days Painful swelling of the hands and feet Painful swelling of the large joints Pain in the back and abdomen Acute chest syndrome (fever, chest pain, increasing WBC count, and pulmonary infiltrates) Pneumonia Pulmonary infarction Pulmonary embolism Death.
hemolytic
Sickling of RBCs produces __________ anemia, the primary manifestations of which include: Pallor Fatigue Jaundice Irritability.
2
Starting at ___ years of age, children with SCD should undergo routine ultrasound scanning of the head to evaluate cerebral blood flow.
b,c,d
The client with sickle cell disease (SCD) asks the nurse to explain vaso-occlusive crisis. Which information is most appropriate for the nurse to include in the explanation? (Select all that apply.) a Prolonged vaso-occlusive crisis may progress to a more severe condition called sickle cell crisis. b Infection increases the risk for developing vaso-occlusive crisis. c Conditions that impair oxygen transport to tissues may lead to vaso-occlusive crisis. d Vaso-occlusive crisis consists of pain resulting from ischemia caused by vascular occlusion. e Overhydration increases the risk for developing vaso-occlusive crisis.
c
The client with sickle cell disease (SCD) is ordered to receive hydroxyurea. To correctly explain the rationale for administering hydroxyurea to the client, which statement is most appropriate for the nurse to include in the teaching? a "Hydroxyurea will help decrease your nausea and vomiting." b "Hydroxyurea may cause you to have more pain." c "Hydroxyurea decreases the production of abnormal blood cells." d "Hydroxyurea decreases the body's production of fetal hemoglobin."
a,c,d,e
The client with sickle cell disorder (SCD) is preparing to be discharged to home. Which topics are most appropriate for the nurse to include when providing home care teaching for the client with SCD? (Select all that apply.) a Balancing rest with activity b Restricting oral fluid intake c Keeping current with vaccinations d Recognizing manifestations of splenic sequestration e Maintaining adequate nutritional intake
b
The nurse educator asks the nursing student to explain the primary cause of anemia related to sickle cell disease (SCD). Which response by the nursing student is most accurate? a Deoxygenation of affected RBCs b Early destruction of RBCs c Deformity of RBCs d Sickling of RBCs
c
The nurse educator is giving an in-service about the assessment and care of children who are diagnosed with sickle cell disease (SCD). Which information should the educator include in the in-service? a Beginning at 5 years of age, children with sickle cell disease should undergo routine ultrasound head scanning. b Newborn screening for sickle cell disease is recommended and optional for children who are born in the United States. c Newborn blood testing for sickle cell disease usually involves obtaining a blood sample by way of a heel stick. d Initial diagnosis of sickle cell disease is most often made by amniocentesis testing in the prenatal period.
a
The nurse is analyzing and synthesizing assessment data, related to the care of the client with sickle cell disease (SCD), to formulate client-specific nursing diagnoses. Which client problem represents the highest priority when planning nursing care of the client with SCD? a Impaired oxygenation b Altered comfort c Activity intolerance d Imbalanced nutrition
d
The nurse is assessing Mr. Klarke Stamos, a 56-year-old male client who is of Greek heritage. Mr. Stamos was admitted to the hospital for treatment of acute exacerbation of sickle cell disease (SCD). When prioritizing nursing care and implementing interventions, which complaint by Mr. Stamos is most appropriate for the nurse to address first? a "I don't have the energy to exercise." b "I'm having a lot of leg pain." c "I don't eat a healthy diet." d "I'm feeling really short of breath."
a
The nurse is assessing the client with sickle cell disease (SCD) for manifestations of shock. Which assessment finding is most reflective of a classic manifestation of shock? a Pallor b Increased urine output c Persistent abdominal pain d Hypertension
a
The nurse is assessing the client with sickle cell disease (SCD) for signs and symptoms of acute chest syndrome. Which assessment finding is most reflective of a manifestation of acute chest syndrome? a Increased white blood cell count b Absence of adventitious breath sounds c Oxygen saturation of > 90% d Urine output > 0.5 mL/kg/hr
c
The nurse is caring for Alishia Baldwin, a 24-year-old African American client who is pregnant with her first child. Mrs. Baldwin and her husband are talking about their family history of sickle cell disease (SCD). Mr. Baldwin asks the nurse, "What factors affect our baby's chances of having sickle cell disease?" Which response by the nurse is most appropriate? a "If both you and your wife have the genetic trait for sickle cell disease, then there is a 50% chance that your baby will have the disease." b "There are several forms of sickle cell disease, but only one form of the disorder is influenced by factors related to inheritance of genes from the parents." c "Because sickle cell disease is caused by an autosomal recessive genetic defect, you both must have the trait in order for your child to have the disorder." d "Sickle cell disease occurs only in individuals who are of African American descent and approximately 50% of African Americans are born with some form of the disorder."
b
The nurse is caring for a child who is in the hospital experiencing sickle cell crisis. The parents are asking the nurse which treatment will help cure the child. Which statement by the nurse is most appropriate? a "Treatment with morphine will cure sickle cell disease." b "Treatment is aimed at pain control, oxygen therapy, and hydration, but does not provide a cure." c "Treatment with an exchange transfusion of blood will cure the child." d "There is no treatment for sickle cell crisis."
c
The nurse is caring for a 58-year-old Marc McRyan, who is diagnosed with sickle cell disease (SCD). Mr. McRyan's healthcare provider plans to use pharmacologic therapy to stimulate his production of fetal hemoglobin. Which therapy should the nurse anticipate will be ordered by Mr. McRyan's healthcare provider? a Oxygen b Penicillin c Hydroxyurea d Vitamin C
a
The nurse is evaluating a 16-year-old African American teenager in sickle cell crisis who was at risk for a cerebrovascular accident. The nurse monitors the child by evaluating which of the following? a Neurovascular system b Renal system c Cardiovascular system d Gastrointestinal system
c
The nurse is instructing the mother of a child with sickle cell disease about the risk factors for precipitation of sickle cell crisis. Which of the following, if identified by the mother as a precipitating factor, indicates the need for further teaching? a Trauma b Infection c Fluid overload d Stress
c
The nurse is planning care for a child who is newly diagnosed with sickle cell disease. Which of the treatment plan with the family will best promote the child's growth and development? a Nutritional support during hospitalizations b Emergency care in the school setting c Individualized school health plan d Renal health plan
d
The nurse is teaching a group of parents whose children have sickle cell anemia. When a parent asks the cause of the symptoms, what is the appropriate response by the nurse? a "The sickled cells mix with normal cells, which cause the immune system to be depressed." b "Sickled cells increase the blood flow through the body, which causes pain." c "Sickled cells cause bone marrow depression." d "Sickled cells clump in the smaller blood vessels and obstruct blood flow."
b,d,e
The nurse is teaching the client about the potential effects of sickle cell disease (SCD). When explaining the potential effects of sickling, which examples are most appropriate for the nurse to include in the teaching? (Select all that apply.) a Prolonged life span of RBCs b Occlusion of small blood vessels c Malformation of WBCs d Tissue damage e Organ damage
b
The nurse is teaching the client with sickle cell disease (SCD) about hemosiderosis, which is a complication associated with frequent blood transfusions. Which statement is most appropriate for the nurse to include in the client teaching? a "In most cases, hemosiderosis is caused by iron-chelating medications." b "Hemosiderosis is storage of iron in tissues and organs." c "Hemosiderosis occurs when your immune system reacts to antigens." d "Vitamin C can increase your risk for developing hemosiderosis."
b,d
The nursing student is creating a presentation about pharmacologic treatments used in the care of clients with sickle cell disease (SCD). Which information is most appropriate for the student to include in the presentation? (Select all that apply.) a Analgesics should be administered to clients with sickle cell disease only on a prn (as needed) basis. b Prophylactic antibiotic therapy may be ordered for clients with sickle cell disease. c Vaccinations are contraindicated for clients with sickle cell disease. d Patient-controlled analgesia (PCA) machines may be ordered for clients with sickle cell disease. e Analgesics are administered to clients with sickle cell disease to help reduce blood viscosity.
b
When explaining neonatal testing for sickle cell disease (SCD), which content is most appropriate for the nurse to include in the care plan? a Initial diagnosis of sickle cell disease (SCD) in newborns most commonly occurs by way of amniocentesis testing. b When testing for sickle cell disease (SCD) in newborns, a heel stick may be used to obtain blood. c Newborns who are diagnosed with sickle cell disease (SCD) should immediately begin diagnostic testing for prevention of stroke. d Newborn screening for sickle cell disease (SCD) is offered as an optional test in the United States.
a
When teaching the client about the cause and treatments associated with hemosiderosis, which statement by the nurse is most accurate? a It is a complication that is associated with frequent blood transfusions. b It may be prevented by avoiding giving iron-chelating pharmacologic agents to the client. c Treatment may include administration of vitamin C to promote iron absorption. d It is caused by an immune reaction to iron stored in donated tissues
a,c,d,e
Which conditions are known triggers for a sickle cell crisis? (Select all that apply.) a Physical stress b Hyperventilation c Fever d High altitudes e Emotional stress
d
Which example of a nursing observation during the evaluative phase best reflects the client's successful achievement of an identified outcome for sickle cell disease (SCD)? a The client experienced improved breathing. b The client produced urine output of > 0.2 ml/kg/hr. c The client's pain was tolerable. d The client maintained an oxygen saturation of > 90%.
a
Which factor influences the development and manifestations of sickle cell disease (SCD) in children? a A high level of fetal hemoglobin in children inhibits sickling of RBCs. b Children with sickle cell trait have two Hb S genes. c Sickle cell crisis is very common among children with sickle cell trait. d Children with sickle cell trait are commonly symptomatic at birth.
d
Which recommendation is most appropriate for the nurse to include in a teaching session for a client with sickle cell disease (SCD)? a Limit oral intake of fluids. b Exercise for at least 2 hours each day. c Understand that splenic sequestration is a nonemergent condition. d Keep current with recommended vaccinations
d
Which statement best describes the effects of sickle cell disease (SCD) on RBCs? a Sickled RBCs may cause ischemia along with intense vascular dilation. b Once sickled, an RBC cannot resume a normal shape. c The cellular membrane of a sickled RBC becomes tough and durable. d Sickling dramatically decreases the life span of affected RBCs.
d
Which symptom most accurately reflects a manifestation of a potential complication associated with sickle cell disease (SCD)? a Hypertension b Increased urination c Hyperalertness d Skin pallor
a,b,c,d
Which treatment options are appropriate for a client with sickle cell disease (SCD)? (Select all that apply.) a Bone marrow transplantation b Surgical removal of the client's spleen c Hematopoietic stem cell transplantation d Blood transfusions e Insulin administration
