Cystic Fibrosis

Meet the Client: Debbie Baker

Paula and David Baker bring their 3-year-old adopted Caucasian daughter, Debbie, to the
pediatrician reporting that she has recently been having large, greasy-looking stools. The
pediatrician notes that Debbie has fallen below the 10th percentile in height and weight.
Debbie appears to be unhealthy and is very listless.
Clinical Manifestations
The pediatrician suspects that Debbie may have cystic fibrosis (CF).
1. Which statement by the mother supports the diagnosis of CF?
A) "My daughter went to bed feeling fine last night and woke up with a high fever and a
sore throat."
B) "Lately Debbie's eyes appear to have a yellow discoloration."
C) "When I kiss my daughter, her skin tastes like salt."
D) "I can hear her heart beating when I lay my head on her chest."
Correct answer(s): C
The pediatrician reviews Debbie's medical chart.
2. Which documentation further supports the diagnosis of CF?
A) A history of frequent respiratory infections.
B) An elevated white blood cell count (WBC).
C) Reports of episodic abdominal pain and crying.
D) A sweat chloride level of 35 mEq/L.
Correct answer(s): A
Diagnostic Test
The pediatrician prescribes a sweat test to confirm Debbie's diagnosis of CF.
3. What information will the nurse include when teaching about the sweat test?
A) Informed consent will be needed for this invasive, diagnostic test.
B) It will take two hours to obtain the sweat.
C) This procedure must be done twice a week for 3 weeks before confirming diagnosis.
D) It is a simple, painless, reliable test that measures the chloride in sweat.
Correct answer(s): D
Therapeutic Communication
David and Paula have been at Debbie's bedside since she was admitted to the hospital.
David asks to speak to the nurse outside in the hall. He tells the nurse that Paula is telling
everyone that Debbie is going to be fine, and that there has just been a mistake and
everything will be all right as long as they pray.
4. What action should the nurse take first?
A) Schedule an appointment for Paula with the hospital's chaplain.
B) Suggest that David and his wife sit down and talk about the situation.
C) Refer Paula to a professional counselor.
D) Make arrangements to meet with Paula privately at the first opportunity.
Correct answer(s): D
Ethical Issues

The nurse meets with Paula to discuss her denial of Debbie's diagnosis. During the
conversation, Paula finally breaks down and tells the nurse, "I just can't believe what I
have been thinking. If Debbie is going to die, maybe I should just give her back to the
adoption agency. I am such a terrible mother for having these thoughts, but I just can't
bear to watch my precious baby suffer."
5. What response reflects that the nurse values the principle of beneficence?
A) "No one ever knows when they will die. Debbie could live for a long time."
B) "This seems like an ethical dilemma to me. I'll refer it to the ethics committee."
C) "I think you probably should contact an attorney to discuss this if you're really serious."
D) "Paula, I know this has to be a very scary and difficult time for you. What can I do to
help you?"
Correct answer(s): D
Paula shares with the nurse that she is thinking about separating from David for a little
while. She states that she is the one who could not have children, and David really didn't
want to adopt a child at first. Because Debbie has been diagnosed with CF, Paula thinks
David may want out of the marriage.
Shortly after this conversation, the nurse is walking down the hall, and David asks to
speak to her in private. He tells the nurse, "I saw you speaking with my wife earlier. I think
she is going to leave me, and I am so scared."
6. What is the best response by the nurse?
A) "You are afraid your wife is going to leave you. Tell me more about what makes you
think that."
B) "I will sit with Debbie so you and your wife can talk. We have a quiet room you can use
that is private and close by."
C) "Ethically, I cannot discuss what your wife told me. Likewise, I cannot tell her what you
say, either."
D) "Most parents are anxious during situations like this. Would you like me to set you up
with a support group?"
Correct answer(s): B
Debbie's parents thank the nurse for offering, but choose to stay with Debbie. The nurse
tells them that she will be available if they change their minds later.
Nursing Diagnoses
The nurse has identified multiple nursing diagnoses due to the chronic and complex
disease process of CF.
7. Which nursing diagnosis has the highest priority?
A) Anticipatory grieving related to a potentially fatal diagnosis.
B) Ineffective coping (family) related to chronic illness.
C) Risk for altered growth and development related to stress of illness and hospitalization.
D) Altered nutrition: less than body requirements related to poor intestinal absorption.
Correct answer(s): D
The nurse also identifies, "Ineffective airway clearance related to excessive pulmonary
secretions" as a nursing diagnosis in Debbie's plan of care.
8. Which statement should the nurse record as the expected outcome for this nursing
diagnosis?
A) The child will maintain an oxygen saturation level greater than 95%.
B) The parents will list 3 symptoms that require notification of the healthcare provider.
C) The child will be able to remove mucus from the airway by coughing.

D) The child will rest comfortably and participate in age-appropriate activities.

Correct answer(s): C
Legal Issues
Debbie's nurse is a preceptor for a student nurse from the local community college. The
student nurse asks the preceptor, "I keep hearing about standards of care. What are
standards of care?"
9. The nurse provides which explanation?
A) Standards of care are published statements that describe the level of care that the
client can expect from nurses.
B) Standards of care are laws mandated by the legislature of each state that ensure safe
care for clients.
C) Standards of care are rules that help healthcare professionals deal with ethical issues.
D) Standards of care are outlined in the Nursing Practice Acts of each state.
Correct answer(s): A
The nurse shares with the student nurse that the Society of Pediatric Nurses' Standards of
Care includes primary, secondary, and tertiary nursing care. The student nurse asks,
"What type of care is given in secondary nursing care?"
10. Which statement by the nurse is correct?
A) "It is care like vision, hearing, and scoliosis screening that Debbie will get when she
starts school."
B) "It includes mainly teaching information about Debbie's cystic fibrosis."
C) "This is the nursing care we do when we are taking care of Debbie here in the hospital."
D) "It is care that includes giving Debbie immunizations to prevent specific diseases."
Correct answer(s): A
Parent Teaching
A Nurse Educator specializing in CF is assigned to Debbie to provide intensive training for
David and Paula. The Nurse Educator explains that she will be helping them by discussing
what it means to have a child with CF, the medications and care that Debbie will require
after discharge, and what signs/symptoms warrant a call to Debbie's healthcare provider.
The Nurse Educator further explains that even though CF is incurable, the life expectancy
of a child with CF has increased dramatically in recent years.
11. Which statement indicates a correct understanding of the etiology of CF?
A) Both of Debbie's biological parents must have the CF gene for Debbie to get it.
B) Debbie must have some Asian heritage because it occurs more often in that ethnic
group.
C) Only one of the biological parents carried the gene for CF, and that parent transmitted
it to Debbie.
D) There is no genetic predisposition for CF.
Correct answer(s): A
Because children with CF are cared for at home, parent education is a priority. David and
Paula agree that they need to learn everything they can about CF. They are loving parents
and have a very caring attitude towards their only daughter. They listen attentively to the
Nurse Educator, ask pertinent questions, and receive all the written documentation about
CF that is provided.
12. Which instruction should the Nurse Educator include when discussing respiratory
complications?

A) Place the child in a prone position when she is having a dyspneic episode.
B) Demonstrate the correct way to administer oxygen at 10 L/min.
C) Explain the importance of using a cool mist humidifier in Debbie's room.
D) Perform chest percussion and postural drainage four times a day.
Correct answer(s): D

The Nurse Educator is discussing information about a level of recommended activity for
Debbie after being discharged from the hospital.
13. Which intervention should the Nurse Educator discuss with Debbie's parents?
A) Discourage Debbie from engaging in any strenuous activity.
B) Encourage Paula and David to enroll Debbie in a weekly gymnastics program.
C) Discuss the importance of playing games that require Debbie to do physical activity
such as running, jumping, or skipping.
D) Give Debbie her inhaler prior to participating in any physical activity.
Correct answer(s): C
The Nurse Educator explains that chronic respiratory infection is a major cause of lung
damage in children with CF, and mobilizing secretions is an important aspect of the
nursing care.
14. What action should the Nurse Educator take to address this?
A) Teach Debbie to take a deep breath and then exhale while whispering the word "huff."
B) Instruct the parents to perform chest physiotherapy (CPT) immediately prior to eating.
C) Explain the importance of decreasing fluid intake during acute exacerbations.
D) Discuss the use of a short-term oral glucocorticoid steroid therapy.
Correct answer(s): A
Paula and David are worried about Debbie meeting her nutritional needs. The Nurse
Educator explains that the thick mucus blocks the pancreatic ducts, preventing enzymes
such as trypsin, amylase, and lipase from being secreted into the small intestines.
15. Which instruction should the Nurse Educator give Debbie's parents concerning the use
of pancreatic enzymes as an oral prescription?
A) Administer pancreatic enzymes in the morning and at night.
B) Mix the pancreatic enzymes with hot, starchy foods such as macaroni or pasta.
C) Open the capsules and mix the beads in a food like applesauce.
D) Ensure that Debbie swallows the pancreatic enzyme capsule whole.
Correct answer(s): C
The nurse reiterates the importance of keeping Debbie away from people with respiratory
infections, and discusses how to provide a diet high in calories. She also reviews with the
parents guidelines concerning when to call the healthcare provider.
While discussing pancreatic enzymes, the Nurse Educator explains that the dosage of the
pancreatic enzyme is adjusted according to stool formation.
16. Which adjustment will be required if Debbie has loose, fatty stools?
A) Do not administer the pancreatic enzymes for 24 hours.
B) Decrease the amount of pancreatic enzymes at each meal.
C) Increase the pancreatic enzymes with each meal and snack.
D) No adjustment in the dosage will be made at this time.
Correct answer(s): C
Community Resources
The Nurse Educator refers Paula and David to the local chapter of the Cystic Fibrosis
Foundation (CFF).
17. Which rationale best supports this referral?
A) Community resources often provide support groups for parents of children with CF.
B) This foundation will provide health insurance for children with CF.
C) The CFF offers information on alternative therapy and treatment modalities.
D) Referrals for resources offer parents hope that a cure may one day be found.
Correct answer(s): A

Growth and Development

The nurse caring for Debbie observes her building a tower of eight blocks. Debbie is able
to say her first name, last name, and state her age while holding up 3 fingers.
18. What action by the nurse is warranted?
A) Notify the social worker that Debbie is developmentally delayed.
B) Document the observations, but take no action at this time.
C) Complete a formal growth and developmental evaluation chart.
D) Repeat the developmental testing in one week.
Correct answer(s): B
Debbie is improving and is scheduled to attend play therapy in the afternoon. Paula asks
the nurse, "What is play therapy?"
19. What is the best response by the nurse?
A) "The volunteers bring toys for Debbie to play with in her room."
B) "Play therapy is designed to improve gross motor skills, such as walking."
C) "Play therapy uses toys and games to help with Debbie's physical and emotional
needs."
D) "The healthcare provider prescribed this to see how well Debbie can tolerate playing."
Correct answer(s): C
Immunizations
On the day of Debbie's discharge from the hospital, the Nurse Educator discusses routine
immunizations with Paula and David.
20. What is the Nurse Educator's responsibility when discussing immunizations?
A) Explain that Debbie should receive all routine childhood immunizations.
B) Instruct the parents to ask the pediatrician which immunizations Debbie should get.
C) Discuss why immunizations are not given to children with chronic respiratory illnesses.
D) Teach Paula and David that Debbie's immunizations will be delayed due to the CF.
Correct answer(s): A
The Nurse Educator asks Paula which immunizations Debbie has already received. Paula
has a copy of the immunization record and notices that Debbie has not had a second
measles, mumps, rubella (MMR) immunization. Paula asks, "When should the second MMR
be given to Debbie?"
21. What response is most appropriate by the primary nurse?
A) "It should be administered as soon as possible."
B) "The second MMR is given between 4 and 6 years of age."
C) "I think the healthcare provider will probably let you know."
D) "It will not be given until 11 to 12 years of age."
Correct answer(s): B

A Complication Occurs
Debbie is discharged home with scheduled follow-up appointments.
Three months after being discharged from the Children's Medical Center, Debbie is
brought by her father to the emergency room. He reports that she started coughing,
choking, and was unable to catch her breath. She passed out. He put her in the car and
drove straight to the hospital. Debbie is admitted into the emergency department.
22. What laboratory result warrants immediate intervention?
A) Serum potassium level of 3.8 mEq/L.
B) White blood cell count (WBC) is 20,000 mm3.
C) Platelet count is 200,000 mm3.
D) Debbie has a serum chloride level of 101 mEq/L.
Correct answer(s): B
23. Which intervention should the nurse implement first?
A) Notify the CF Nurse Educator to help support Debbie's parents.
B) Administer IV antibiotic therapy.
C) Insert a 24 gauge intravenous line.
D) Administer oxygen 2 L/min via pediatric face mask.
Correct answer(s): D
The healthcare provider prescribes ceftriaxone sodium (Rocephin) 12.5 mg IVPB. The
medication comes in 12.5 mg/25 ml and is to be infused over one hour. The medication
will be administered via an intravenous infusion pump.
24. At what rate should the nurse set the infusion pump?
A) 12.5 ml/hr.
B) 25 ml/hr.
C) 37.5 ml/hr.
D) 50 ml/hr.
Correct answer(s): B
Management
Two days later, Debbie's condition has improved, but she remains in the hospital for
continued IV antibiotic therapy.
The primary nurse reports to the charge nurse that the IV antibiotic prescribed for Debbie
was administered to another child and Debbie received that child's prescribed antibiotic.
When the primary nurse realized the mistake, the IV antibiotic was discontinued
immediately.
25. Which action should the primary nurse implement first?
A) Assess Debbie and the child that received the wrong medication.
B) Administer the antibiotic to the correct clients.
C) Complete a medication incident report.
D) Notify the healthcare providers immediately.
Correct answer(s): A

The primary nurse, who is assigned to care for four clients, is working with an unlicensed
assistive personnel (UAP) during the 7pm to 7am shift.
26. What nursing task can be safely delegated to the UAP?
A) Discontinue a child's peripheral IV.
B) Empty the Foley bag.
C) Change the rate on an IV pump controller.
D) Complete an admission assessment.
Correct answer(s): B
The primary nurse finds Debbie and the play therapist in the therapy room working with
fingerpaints, and she is unsure whether she should interrupt for vital signs. She seeks
advice from the charge nurse.
27. What action should the charge nurse take?
A) Ask the play therapist to take the vital signs.
B) Interrupt the play therapy session to take the vital signs.
C) Reprimand the primary nurse for delaying the taking of the vital signs.
D) Explain that play therapy is an intervention and should not be interrupted for vital
signs.
Correct answer(s): D
Going Home
Debbie's pneumonia resolves. The Nurse Educator visits with Debbie and her parents. The
parents share that they are looking forward to a relaxing vacation at the beach this
summer. They ask the Nurse Educator if any special precautions will be necessary for
Debbie.
28. How should the Nurse Educator respond?
A) Wish them a happy vacation and tell them nothing special needs to be done.
B) Suggest that they add extra salt to Debbie's diet and watch her for dehydration.
C) Explain that the beach is not a good vacation place for Debbie.
D) Encourage them to limit Debbie's play time at the beach to thirty minutes a day.
Correct answer(s): B
Case Outcome
Debbie is being discharged from the hospital. The Nurse Educator has obtained a contact
number for a CF clinic near the beach in case an exacerbation occurs. Paula and David
now feel confident as they plan their first vacation with Debbie.