Sickle Cell Anemia

The school nurse reviews Mary's immunization records from her previous school. The nurse notes that Mary has had four scheduled doses of DTaP, three scheduled doses of Hib, and one dose of MMR and recieved her Hep B series as an infant. Which recommendation about immunization should the school nurse make to Mary's grandmother? A. Mary needs her second scheduled dose of MMR #2. B. Mary is currrent with her immunizations. C. Mary needs her Hepatitis A immunizations. D. Mary needs her influenza vaccine.

A. According to current immunization schedules, Mary should have had two scheduled doses of MMR, with the second due at 4 to 6 years of age.

Which statement by Mary indicates that she is meeting Erikson's stage of development for her age? A. "Look, I finished putting the puzzle together." B. "I don't want any of my friends to visit me here." C. "I need my stuffed dog so that I can go to sleep." D. "When I grow up I want to be a nurse just like you."

A. School-aged children are in Erikson's stage of developing a sense of industry, meaning they like to do and accomplish things.

The nurse calculates Mary's intake and output (I&O) for the shift. Mary has had 24 ounces of water, 8 ounces of apple juice, and three 4-ounce cartons of milk. She received 50 mL of IV fluids per hour for the last 12 hours and had a urinary output of 1200 mL, plus one episode of wetting the bed. What is Mary's total intake for this shift?

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The charge nurse is transcribing prescriptions at the nurse's station. Other responsibilities of the charge nurse include answering the phone, assisting with visitors' questions, and answering the clients' call lights. Which nursing task would be best for the charge nurse to delegate to the UAP? A. Take the hourly vital signs for a client receiving a unit of blood. B. Teach Mary's grandmother how to apply warm soaks to her joints. C. Clean the insertion sites of a client with skeletal traction. D. Change the morphine vial on the patient controlled analgesia pump

A. The UAP can take the vital signs but the RN must evaluate the results.

The emergency department nurse continually assesses Mary for signs and symptoms of hypoxia. Which areas should the nurse assess to determine cyanosis in persons with dark skin? A. Oral mucosa B. Palms of the hands C. Nail beds D. Sclera of the eyes

A. The oral mucosa should be assessed for cyanosis in persons with dark skin because blueness cannot be assessed in the lips or fingertips.

After attending school for 2 weeks without any problems, Mary reports to the school nurse that she doesn't feel well. The nurse determines Mary has a temperature of 102F. The school nurse calls Earlene at the restaurant and advises her to take Mary directly to the emergency department (ED). Mary complains of pain in her knees, in her elbows, and throughout her body. In the emergency department, the nurse confirms Mary's vital signs as T 102.4F, P 104, R 24, B/P 90/68, and pulse oximeter reading 91%. Which action should the emergency department nurse implement first? A. Request arterial blood gasses stat. B. Administer oxygen via nasal cannula. C. Send the grandmother to the admissions office. D. Prepare to administer analgesics as prescribed.

B. A pulse oximeter reading of less than 93% indicates hypoxia, which warrants oxygen administration. Oxygen will not help reverse the sickling crisis because oxygen cannot reach the enmeshed sickled erythrocytes in clogged vessels. However, it will help oxygenate the cells that are not sickled.

Mary's healthcare provider has advised Earlene to get pneumococcal and meningococcal vaccines for Mary at her follow-up office visit. Earlene asks the nurse, "Why does Mary need to have those other vaccines? I hate for her to get more shots. She cries, and I know it hurts." What is the best response by the nurse? A. "I will get the healthcare provider to explain why Mary needs the vaccines." B. "She is susceptible to infections. These vaccinations may help prevent a crisis." C. "These vaccines are required for all children younger than 10 years of age." D. "I know you don't like to see her hurt, but she must have these vaccines."

B. An individual with SCA has a functional reduction in splenic activity, which progresses to the point at which the spleen is no longer able to function. This increases Mary's susceptibility to infection.

Earlene is very upset and tells the nurse that she noticed that when Mary went to the bathroom to urinate there was blood in the commode. Which action should the nurse implement? A. Notify the healthcare provider immediately. B. Explain that blood in the urine is expected. C. Assess Mary's perineal area for bleeding. D. Request a stat sterile urine specimen.

B. Hematuria is an expected clinical manifestation during a vasooclusive sickle cell crisis.

Mary is transferred from the emergency department to the pediatric intensive care department. In developing the plan of care, the nurse identifies the nursing diagnosis, "Acute pain related to tissue ischemia" as a priority. Which intervention should be included in the care plan? A. Assess pain by using a numerical pain scale. B. Explain how to use a patient controlled analgesic pump. C. Apply cold compresses periodically to painful joints. D. Administer acetaminophen (Tylenol) PRN as needed for pain.

B. Opioids are the mainstay for analgesic treatment for acute pain, and when they are administered via PCA pump the client has better control of the pain. Both the child and the parent (in this case, the grandmother) should receive information about the use of the PCA pump

Mary's grandmother goes downstairs to get something to eat from the hospital cafeteria. The unlicensed assistive personnel (UAP) who is staying with Mary while her grandmother is gone informs the primary nurse that Mary urinated in the bed, is crying, and wants her grandmother. Which intervention should the nurse implement first? A. Change Mary's bed linens. B. Help Mary change her clothes. C. Find Earlene in the cafeteria. D. Document the incident in Mary's chart.

B. The nurse should address Mary's physical needs first, then find Mary's grandmother, have the linens changed, and document the incident. Enuresis is expected due to increased fluid intake.

Mary finally falls asleep. The night nurse allows her to sleep throughout the night without interruptions. Mary wakes up the next morning and asks the night nurse, "Will I go to sleep one night like my Mommy and never wake up?" The nurse gives Mary a hug and offers what response? A. "Tell me why you think that your Mommy will never wake up from her sleep." B. "Mary, your Mommy did not go to sleep. Remember, she died in a car accident." C. "Your grandma will take good care of you and make sure you always wake up." D. "If you are very careful and do what you are told, you will not have to worry about that."

B. The nurse should explain that going to sleep is not like dying. Young school-aged children are concrete thinkers who often still believe in magical thinking. Any misconceptions about death and illness need to be corrected in a caring manner.

After talking with Earlene and assessing Mary's situation, the CPS nurse takes Mary to meet her grandmother for the first time. The CPS nurse escorts Mary to her grandmother's house. As the front door opens, Mary hides behind the nurse's back. Which action will the CPS nurse take first? A. Ask the grandmother to wait until the house until she talks to Mary. B. Get down on the knees to calmly talk to Mary face-to-face. C. Explain that the nurse won't leave until Mary says it is all right. D. Hold Mary's hand and calmly walk into the house while talking to Mary.

B. The nurse's first priority must be to Mary. Talking to her at the child's level provides comfort and security and is the best initial response to Mary's behavior.

Mary is discharged home with a home health referral. The home health nurse visits Mary the day after she is discharged from the hospital. Earlene asks the home health nurse, "I received some information from the Sickle Cell Foundation, but I have never heard of it. What kind of group is it?" How should the nurse respond? A. "It is a foundation that deals primarily with research to find the cure for sickle cell anemia." B. "It provides information on the disease and on support groups in this area." C. "They didn't discuss this organization with you when Mary was in the hospital?" D. "The foundation arranges for families with children who have sickle cell to meet each other."

B. This correctly describes the mission of the foundation. Knowing about resources helps decrease the primary caregiver's feelings of frustration and helplessness.

Earlene listens attentively to the nurse discuss Mary's condition and what she must do to care for her. After reviewing the needed care, the nurse asks the grandmother if she has any other questions. Earlene asks, "How did Mary get this awful disease?" How should the nurse respond? A. As a black woman, you must be aware that this is an inherited autosomal disease. B. Mary has the disease because she inherited the gene from both of her parents, who were carriers. C. It may be an awful disease but Mary can live a long, productive life. D. She was exposed to a virus while her mother was pregnant.

B. This explains the etiology in terms that a layperson can understand. For a child to acquire the disease, both parents must have the SCA trait. Each child of a couple with the SCA trait has a 1 in 4 chance of developing the disease.

Earlene asks many questions about sickle cell anemia. She is very concerned about her granddaughter and what will happen to her in the future. The nurse is aware that there are many serious complications that Mary could experience. Which potentially fatal complication(s) can occur? Select all that apply. A. Vasooclusive criss. B. Cerebral vascular accident. C. Priapism. D. Hypertensive crisis. E. Heart failure.

B.E. Due to the sickling of the blood and to tissue hypoxia, a CVA or stroke may occur and is potentially fatal. The patient with SCA can develop heart failure related to cardiomegaly.

Mary is in the Intensive Care Department for 3 days and is transferred to the pediatric floor. Earlene has been at the hospital every day and is very concerned about Mary and her condition. Earlene asks the nurse, "What can I do to make sure this never happens again?" What is the best initial response by the nurse? A. "When Mary gets a fever give her 1 baby aspirin." B. "Keep Mary away from anyone who has an infection." C. "There is no way you can make sure this never happens again." D. "Make sure she does not participate in any strenuous activitty."

C. A child with sickle cell anemia will experience crises. Earlene needs to understand that she cannot prevent a crisis from ever happening again. The nurse should instruct Earlene and Mary about measures to reduce the frequency of crises, as well as how to recognize crises when they do occur.

The nurse is aware that the African American culture has expected family roles in the family organization. This information is critical when discussing Mary's entrance and acceptance into this already established family unit. What role does the grandmother usually play in African American culture? A. In most African American families, the grandmother must be cared for by family. B. Many times the grandmother is the bread winner, especially if there is no father. C. The grandmother usually plays a critical role in child care and economic support. D. The grandmother is usually the disciplinarian in the African American family.

C. An older person, especially a grandmother, is respected for her insight and plays one of the most central roles in the family.

Mary is starting to feel better and is requiring less pain medication, and she is sleeping as the nurse makes evening rounds. Earlene shares with the nurse it has been a long time since she had a 7-year-old in her home. Earlene says, "I have no idea what Mary should be allowed to do so she can have some fun." Which statement will be the best response by the nurse? A. "You should like you are worried about raising Mary by yourself." B. "I would recommend enrolling her in a sport with running, such as soccer." C. "Seven-year-olds really like being in groups like Girl Scouts or Girls' Clubs." D. "Mary should not be around a lot of children, so her activities will be limited."

C. Formal organizations foster self-esteem and competence as children earn ranks and merit badges. Interaction with peers, acquisition of new skills, and a sense of belonging to a group outside the family can help increase self-esteem.

Mary is scheduled for discharge the next day. The nurse is completing discharge teaching with Earlene when Earlene tells the nurse that she is planning to take Mary to Colorado to visit her oldest daughter and their family for the Christmas holidays. Mary is very excited and can't wait to meet her cousins. What is the best response by the nurse? A. "I know that Mary will enjoy meeting her family." B. "I think you should talk to Mary's healthcare provider before you go." C. "Your planned trip may put Mary at risk for a crisis. D. "Could your daughter come here for the Christmas holidays?"

C. High altitudes have decreased oxygen, which could lead to a sickle cell crisis. In addition, cold will cause constriction of blood vessels, further decreasing the oxygen supply.

The emergency department physician completes the assessment and diagnoses Mary with a vasoocclusive sickle cell crisis, probably secondary to pneumonia. Which prescription should the nurse anticipate? A. Administer meperidine (Demerol) intravenously. B. Admit Mary to a private room and keep her in reverse isolation. C. Infuse 5% Dextrose in 0.33% NS at 75 mL/hr via pump. D. Insert a 22 French indwelling urinary catheter with an urimeter.

C. Increased intravenous fluid reduces the viscosity of blood, thereby preventing further sickling due to dehydration. This rate is higher than usual for a 7-year-old, but it is indicated during a sickle cell crisis.

The day shift is just coming on duty to the pediatric department where Mary is a client. The staff available includes two experienced RNs, one new graduate who has just finished the 3-month pediatric internship, and two unlicensed assistive personnel (UAP). Which client should the charge nurse assign to the new graduate nurse? A. A 4-year-old child newly diagnosed with Cystic Fibrosis. B. Mary, who is scheduled as a probable discharge for tomorrow. C. A 9-year-old child who had an appendectomy 2 days ago. D. A 6-year-old child being evaluated for possible physical abuse.

C. This child had a routing surgery. There is no data indicating that the child is unstable; therefore, a new graduate should be able to provide safe care for this child.

The nurse notifies the emergency department physician of Mary's vital signs, which are: T 102.4F, P 104, R 24, B/P 90/68. The nurse anticipates the prescription of which diagnostic test by the emergency department physician? A. Spinal tap B. Hemoglobin electrophoresis C. Sickle-turbidity test (Sickledex) D. Blood cultures

D. An elevated temperature is the first sign of bacteremia, which leads to a sickle cell crisis. The bacteria must be identified so the appropriate antibiotic can be prescribed to treat the infection.

To evaluate the discharge teaching completed at the hospital, the home health nurse discusses acute exacerbations of SCA with Mary's grandmother. Which behavior indicates to the nurse that Earlene understands about acute exacerbations or sickle cell anemia? A. She is able to take Mary's radial pulse within 4 beats of the nurse. B. She does not allow Mary to go outside unless she is with her. C. She measures Mary's fluid intake to remain under 1 liter a day. D. She demonstrates how to accurately read an oral thermometer.

D. An elevated temperature is the first sign of infection, which can, and many times does, lead to an acute exacerbation of SCA. Therefore, being able to take Mary's temperature correctly is vital.

While visiting with Earlene, the CPS nurse states, "Your son never told you that he had a daughter? He was aware that he had a child. Mary's mother attempted to get child support before he left town. Do you know where he is and why he hasn't been a part of Mary's life?" Earlene looks at the nurse and hesitates before responding. Which statement by Mary's grandmother supports a common cultural communication expectation in the African-American heritage? A. My son apparently wasn't ready to be a father. B. Can''t you have the police find him? C. Do you think Mary knows where her father is? D. I really don't want to talk about my son's situation.

D. In the African American culture, what transpires within the family is viewed as private and not appropriate for discussion with strangers.

After a few minutes, Earlene opens up her arms, Mary goes into them, and they hug. The CPS nurse stays with them for a few hours until feeling assured that the child and grandmother are beginning to feel comfortable together. Both Earlene and Mary are talking and laughing as the nurse leaves the home. Earlene takes Mary to the local elementary school, where Mary is enrolled in the second grade. She meets with the school nurse and discusses Mary's disease. The school nurse has cared for several children with SCA and is very knowledgeable about the disease and Mary's needs. The school nurse discusses Mary's condition with the classroom teacher. Which intervention should the nurse implement? A. Explain that the other children should be really nice to Mary. B. Instruct the teacher to have Mary sit at the front of the classroom. C. Encourage Mary to participate in all playground activities. D. Request that Mary be allowed to go the bathroom whenever she asks.

D. Mary needs increased hydration to prevent a sickling crisis. This increased fluid intake will lead to increased urination, so it is important that Mary be allowed to go to the bathroom as needed.

When Mary's paternal grandmother, Earlene, is informed that she has a 7-year-old granddaughter, she immediately agrees to care for Mary. Earlene is a waitress and lives alone in a middle-class neighborhood in a small town about 85 miles from where Mary currently lives. Before placing Mary in a new home, CPS evaluates Earlene's physical, financial, and psychological ability to care for Mary. Earlene is not familiar with SCA. A nurse from CPS meets with her to discuss Mary's health condition. Earlene says to the nurse, "I have heard of sickle cell anemia, and I know it can be very bad, but I don't know exactly what it is." What is the best response by the nurse to Earlene's statement? A. I have some written material that will explain all about the disease. B. It is a disease of the blood that requires taking medication every day. C. Mary will probably have episodes of severe joint pain and will need to be hospitalized. D. Red blood cells become 'C' shaped, stiff, and sticky, which blocks the blood vessels.

D. Sickle cell anemia is a disorder of the red blood cells characterized by abnormally shaped red cells that block and damage blood vessels leading to oxygen deprivation, pain, anemia, serious infections, and damage to vital organs.

The Intensive Care Department night nurse is making rounds and hears Mary crying. The nurse says, "Mary, are you hurting?" Mary cried harder and replies, "No, I just miss my Mommy." Mary's grandmother has gone home to rest for a few hours. How should the nurse intervene initially? A. Say, "I know how hard it is to lose your Mommy. Mine is gone too." B. Tell Mary she needs to try and quit crying and get some sleep. C. Call Mary's grandmother and ask her to come to the hospital. D. Pull up a chair, hold Mary's hand, and allow her to cry.

D. Taking the time to make contact by touching Mary and allowing her to cry is the most therapeutic action at this time. There is nothing the nurse can say to make Mary's mother come back.

After the nurse talks to Mary, the nurse and Mary walk into the grandmother's living room. Earlene and Mary just stare at each other without saying anything. Mary's grandmother gets tears in her eyes. Mary will not let go of the nurse's hand. What should the nurse do? A. Take no action and allow Mary to make the first move. B. Sit down in the chair farthest from Earlene. C. Encourage Mary to hold her grandmother's hand. D. Calmly introduce her paternal grandmother to Mary.

D. The nurse should calmly and quietly introduce Mary to her grandmother and observe her response to determine the next action needed.

The night nurse assessed Mary and notes that her vital signs are now T 98.3F, P 108, R 22, B/P 96/60. Which action should the nurse implement? A. Notify the healthcare provider immediately B. Retake and assess the vital signs in 1 hour C. Encourage Mary to turn, cough, and deep breathe D. Document the findings on the graphic sheet.

D. These are normal vital signs for a 7-year-old child; normal pulse is 70 to 110, normal respirations 18 to 22, B/P systolic: 83 to 121 and diastolic 45 to 79. The healthcare provider will use a detailed breakdown of height by age norms to determine normal vs. hypertensive.

Case outcome

Mary goes back to school the next day, and Earlene returns to work at the restaurant. Earlene's daughter and family, along with her son and his family, are planning a wonderful Christmas at the grandmother's home. Mary has not asked any questions about her father, but she often talks about her mother and is slowly adapting to living with her special "Gram."

Introduction

Mary, a 7-year-old African American girl, was diagnosed with sickle cell anemia (SCA) when she was 5 months old. She is now in the custody of Child Protective Services (CPS) because her 24-year-old mother died in a car accident 2 weeks ago. The biological father moved out of state when Mary was 10 months old. Mary's mother was not married to the father, and Mary has never met him.