Signs and Symptoms
Sickle Cell Crisis
Treatment
Maintenance
Education
100
What may be the first sign of sickle cell anemia in infants? A) Swollen hands and feet B) Frequent upper respiratory infections C) Pallor D) Jaundice
What is A) Swollen hands and feet?
100
The nurse is caring for a child with sickle cell disease experiencing severe chest pain, fever, a cough, and dyspnea. What is the nurse’s priority action? A) Administer 100% oxygen to relieve hypoxia. B) Administer pain medication to relieve symptoms. C) Notify the health care practitioner because chest syndrome is suspected. D) Notify the health care practitioner because child may be having a stroke.
What is C) Notify the health practioner because chest syndrome is suspected? Rationale: Severe chest pain, fever, a cough, and dyspnea are the signs and symptoms of chest syndrome. The nurse must notify the practitioner immediately
100
A child with SCA is being treated for a crisis. The doctor orders morphine sulfate 2mg IV. The concentration of the vial is 10mg/1mL of solution. How many mLs of solution should the nurse administer? Record your answer with one decimal place.
What is 0.2 mL? Rationale: 2 mg/10 mg X 1 ml= 0.2 mL
100
Which of the following activities would the nurse recommend for a child with sickle cell? A) A family vacation in the rocky mountains B) Attending the local boys club snow-skiing trip C) Traveling by airplane D) Visiting the museum of natural history by bus
What is D) Visiting the museum of natural history by bus? Rationale: Taking a trip to the museum is the only answer that doesn't pose a threat.
100
Parents of a 4 year old with SCA tell the nurse that they would like to have other children, but they're concerned about passing SCA onto them. Which health care team member would be the most appropriate person for the nurse to refer them to? A) Clergy B) Social worker C) Certified nurse mid wife D) Genetic counselor
What is D) Genetic Counselor?
200
The mother asks the nurse why the child's hemoglobin was normal at birth but now the child has hemoglobin S? Which of the following responses by the nurse is most appropriate? A) "The placenta bars passage of the hemoglobin S from the mother to the fetus.” B) “The red bone marrow does not begin to produce hemoglobin S until several months after birth.” C) “Antibodies transmitted from you to the fetus provide the newborn with temporary immunity.” D) “The newborn has a high concentration of fetal hemoglobin in the blood for some time after birth.”
What is D) "The newborn has a high concentration of fetal hemoglobin in the blood for some time after birth. Rationale: Sickle cell disease is an inherited disease that is present at birth. However, 60% to 80% of a newborns hemoglobin is fetal hemoglobin, which has a structure different from that of hemoglobin S or hemoglobin A. Sickle cell symptoms usually occur about 4 months after birth, when hemoglobin S begins to replace the fetal hemoglobin. The gene for sickle cell disease is transmitted at the time of conception, not passed through the placenta. Some hemoglobin S is produced by the fetus near term. The fetus produces all its own hemoglobin from the earliest production in the first trimester. Passive immunity conferred by maternal antibodies is not related to sickle cell disease, but this transmission of antibodies is important to protect the infant from various infections during early infancy.
200
A preschool age child with SCA is admitted to the health facility in vaso-occlusive crisis after developing a fever and joint pain. What is the nurses highest priority when caring for this child? A) Providing fluids B) Maintaining protective isolation C) Applying cool compresses to affected joints D) Administering antipyretics as ordered
What is A) Providing fluids?
200
To prevent thrombus formation in capillaries, as well as other problems from stasis and clotting blood in the sickling process, the nurse should: A) Administer oxygen B) See that the child maintains bed rest C) Increase fluids by mouth and use a humidifier D) Administer ordered heparin or other anticoagulants
What is C) Increase fluidsby mouth and use a humidifier? Rationale: Sickling is related to the concentration of hemoglobin within the cell. Because hypertonicity of the blood plasma increases the intracellular concentration of hemoglobin, dehydration promotes sickling.
200
The client has been diagnosed to have sickle cell anemia. Which of the following is appropriate nursing action when the client experiences joint pains? A) Apply cold compress to the area b) Apply warm compress to the area c) Immobilize the involved area d) Massage the involved area
What is B) Apply warm compresses to the area?
200
A child who was hospitalized for sickle cell crisis is being discharged. Which parent outcome demonstrates effective teaching regarding prevention of further crises. A) The parent verbalizes the need to stay away from persons with known infections B) The parent verbalizes appropriate dietary restrictions C) The parent verbalizes the need to restrict fluid intake. D) The parent participates in an aerobic exercise program
What is A) The parent verbalizes the need to stay away from persons with known infections? Rationale: preventing infections through proper hand washing and staying away from persons with known infections is an important measure in preventing sickle cell crises.
300
An adolescent admitted with sickle cell is most at risk for developing which complication? A) Swelling of the hands and feet B) Petechiae C) Leg ulcers D) Hemangiomas
What is C) Leg ulcers? RATIONALE: in SCA, sickling of RBCs leads to increased blood viscosity and impaired circulation. Diminished peripheral circulation makes the adolescent or adult with SCA susceptible to chronic leg ulcers.
300
A child is brough to the emergnecy room in sickle cell crisis. What position is best for this child to be placed in? A) Side-lying with knees flexed B) Knee-chest position C) High-fowlers with knes flexed D) Semi-Fowlers with legs extended on the bed
What is D) Semi-Fowlers with legs extended on the bed? Rationale: Semi-fowler's position with the legs extended provides the best oxygenation for this child.
300
The outpatient clinic nurse is caring for a 7-year-old child with sickle cell anemia. The child has a history of having a splenectomy at age four. At this time the nurse's priority of care would be: A) Assessing for jaundice B) Monitoring serial hematocrit readings C) Frequent assessments of the abdomen D) Keeping the child away from infectious contacts
What is D) Keeping the child away from infectious contacts? Rationale: The spleen plays a role in immunity; without a spleen, a child is more prone to infection, which can precipitate crisis.
300
A clinic nurse instructs the mother of a child with sickle cell anemia about the precipitating factors related to sickle cell crisis. Which of the following if identified by the mother as a precipitating factor, indicates the need for further instructions? A) Stress B) Trauma C) Infection D)Fluid overload
What is D) Fluid overload? Rationale: Sickle cell crises are accute exacerbations of the disease which vary considerably in severity and frequency. These include vaso-occlusive crisis, splenic sequestration, and aplastic crisis. Sickle cell crisis may be precipitated by infection, dehydration, hypoxia, trauma, or physical or emotional stress.
300
A nurse is giving instructions to parents of a school age child diagnosed with SCA. The instructions should include: A) Applying cold to affected areas to reduce the childs discomfort B) Restricting the child’s fluids during crisis situations. C) Avoiding areas of low oxygen concentration such as high altitudes D) Encouraging the child to exercise to reduce the likelihood of crisis.
What is C) Avoiding areas of low oxygen concentration such as high altitudes? Rationale: the child should avoid areas of low oxygen, such as high altitudes because they can precipitate sickle cell crisis.
400
A child suspected of having sickle cell disease is seen in a clinic, and laboratory studies are performed. A nurse checks the lab results, knowing that which of the following would be increased in this disease? A) Platelet count B) Hematocrit level C) Reticulocyte count D) Hemoglobin level
What is C) Reticulocyte? Rationale: A diagnosis is established based on a complete blood count, examination for sickled red blood cells in the peripheral smear, and hemoglobin electrophoresis. Laboratory studies will show decreased hemoglobin and hematocrit levels and a decreased platelet count, and increased reticulocyte count, and the presence of nucleated red blood cells. Increased reticulocyte counts occur in children with sickle cell disease because the life span of their sickled red blood cells is shortened.
400
Which nursing interventions should the nurse expect to implement for a child in an acute sickle cell crisis? Select all that apply. A) Maintain adequate hydration B) Providing adequate pain control C) Assessing family education needs D) Encouraging healthful eating habits E) Anticipating play needs
What is A), B), & E)?
400
An adolescent is sickle cell crisis (pain episode) is complaining of right-knee pain. The best nursing intervention would be: A) Decrease IV fluids B) Wrap the knee in a cold pack C) Apply a warm soak to the right knee D) Give morphine 0.5 mg as ordered
What is C) Apply a warm soak to the right knee? Rationale: Warmth causes vasodilation, which will help lessen the pain of vaso-oclusive crisis.
400
An important nursing consideration when caring for a child with sickle cell anemia is which of the following? A) Refer parents and child for genetic counseling. B) Teach parents and child how to recognize signs and symptoms of crises. C) Help the child and family adjust to a short-term disease. D) Observe for complications of multiple blood transfusions
What is B) Teach the parents how to recognize the signs and symptoms of a crises?
400
The couple with the lowest risk of having a child with sickle cell anemia disease is the one in which the: A) Father is HbS and the mother is HbS B) Father is HbS and mother is HbAS C) Father is HbA and the mother is HbS D) Father is HbAS and mother is HbAS
What is C) Father is HbA and the mother is HbS?
500
What makes Sickle Cell Anemia hemolytic? A) The abnormal shape of the sickled cells B) The life-span of sickled cells (12-15 days) C) A lack of oxygen carried by the sickled cells D) All of the above
What is B) The life-span of sickled cells?
500
A child is to receive a blood transfusion of packed RBC’s for sickle cell crisis. The nurse identifies the child using 2 patient identifiers, and then prepares to administer the transfusion. Place the following steps in the order the nurse should follow to administer this product. Use all options A) Flush the IV tubing and line with normal saline. B) Check the blood bag against the child’s information. C) Watch for a transfusion reaction. D) Record vital signs E) Put on gloves, a gown, and a face shield. F) Check the PRBC’s for date and abnormalities
What is D), F), B), E), A), C)?
500
A nurse is reviewing a physician's precriptions for a child with sickle cell anemia who was admitted to the hospital for the treatment of vaso-occlusive crisis. Which prescriptions documented in the child's record should the nurse question? Select all that apply. A) Restrict fluid intake B) Position for comfort C) Avoid strain on painful joints D) Apply nasal oxygen at 2 L/min E) Provide a high-calorie, high protein diet F) Give meperidine (Demerol), 25 mg IV, every 4 hours
What is A) Restrict fluid intake & F) Give Demerol 25 mg IV every 4 hours? Rationale: Sickle cell anemia is one of a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S. It is caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain. Hemoglobin S is sensitive to changes in the oxygen content of the red blood cell; insufficient oxygem causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow. Oral and IV fluids are an important part of treatment. Meperidine (Demerol) is not recommended for a child with sickle cell disease because of the risk for normeperidine-induced seizures. Positioning for comfort, avoiding strain on painful joints, oxygen, and a high-calorie and high-protein diet are also important parts of the treatment plan.
500
A nurse is preparing to discharge a patient with sickle cell anemia. What patient need should the nurse emphasize in her discharge assessment? A) The need to have pain medications readily available. B) The need for adequate support structure C) The need to maintain good hydration D) The need to follow up with physician visits
What is B) The need for adequate support structure?
500
A child with sickle cell anemia is being discharged after treatment for a crisis. Which instructions for avoiding future crises should the nurse provide to the client and his family? Select all the apply. A) Avoid foods high in folic acid B) Drink plenty of fluids C) Use cold packs to relieve join pain D) Report a sore throat to an adult immediately E) Restrict activity to quiet board games F) Wash hands before meals and after playing
What is B, D, & F?
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