CF Case Study

Which assessment supports the diagnosis of CF? A. A fever of 102 ºF (38.9 ºC), inflamed larynx with exudate. B. Eyes with redness and yellow exudate. C. Weight loss and delayed growth despite a hearty appetite. D. A brassy cough with inspiratory stridor.

C. Weight loss and delayed growth despite a hearty appetite.

The healthcare provider (HCP) prescribes a sweat test to confirm to diagnosis of CF. 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 2 hours to obtain the sweat. C. This procedure will require the child to be NPO (nothing by mouth). D. It is a simple and reliable test that measures the chloride in sweat.

D. It is a simple and reliable test that measures the chloride in sweat.

Parents bring their 24-month-old child to the metropolitan area's family care clinic. The reason for the visit is to establish a primary healthcare provider (HCP) for the family's healthcare maintenance. The child also appears to be suffering from a cold, experiencing rhinorrhea noted to be thick and tenacious and a dry cough which the parent states began a couple of days ago.

The nurse suspects the client may have cystic fibrosis.

The CNS explains that chronic respiratory infection is a major cause of lung damage in children with CF and that mobilizing secretions is an important aspect of nursing care. What actions should the nurse educator take to address this? Select all that apply. A. Instruct the parents to perform chest physiotherapy (CPT) either 2 hours before meals or 2 hours after meals. B. Aerosolized medications should be administered before CPT. C. Discuss the use of a daily oral glucocorticoid steroid therapy. D. Discuss the possibility of long-term oxygen administration to help with mobilizing secretions. E. Encourage "blowing" activities, such as bubbles or pinwheels.

A. Instruct the parents to perform chest physiotherapy (CPT) either 2 hours before meals or 2 hours after meals. B. Aerosolized medications should be administered before CPT. E. Encourage "blowing" activities, such as bubbles or pinwheels.

The mother shares with the nurse that she is thinking about separating from her husband for a little while. She states that because her child has been diagnosed with CF, she thinks she may want out of the marriage. Shortly after this conversation, the nurse walks down the hall and the father 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." Based on her previous conversation with the mother, what is the best statement 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 your child 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

B. I will sit with your child so you and your wife can talk. We have a quiet room you can use that is private and close by.

Client's pneumonia resolves. The CNS visits with the client & her parents. The parents share that they are looking forward to a relaxing vacation at the beach. They ask the CNS if any special precautions will be necessary for her. How should the CNS respond? A. Wish them a happy vacation and tell them nothing special needs to be done. B. Suggest that they add extra salt to Darla's diet and watch her for dehydration. C. Explain that the beach is not a good vacation place for her. D. Encourage them to limit client's play time at the beach to 30 minutes a day.

B. Suggest that they add extra salt to Darla's diet and watch her for dehydration.

One year after being discharged from the Children's Medical Center, the client is brought to the emergency department (ED). Her father reports that she started coughing and choking and was unable to catch her breath. She had passed out briefly and he put her in the car and drove straight to the hospital. The client is awake and oriented x 3. She appears lethargic and she is sitting in a tripod position. The child weighs 12 kg (26.5 lb) and her vital signs include temperature 99° F (37.2° C), heart rate 110 beats/min, respiration 22 breaths/min, pulse oximeter 91%. She is displaying retractions, nasal flaring, and grunting. Two days ago, her grandmother said that she noticed the child didn't want to eat and wasn't as active as usual. Which intervention should the nurse implement first? A. Notify the CF nurse educator to help support the parents. B. Administer IV antibiotic therapy. C. Insert a 24-gauge intravenous line. D. Administer oxygen 4L/min via high-flow nasal cannula.

D. Administer oxygen 4L/min via high-flow nasal cannula.

The primary nurse finds the client and the play therapist in the therapy room working with finger paints, and the nurse is unsure whether she should interrupt to assess her vital signs. She seeks advice from the RN team leader. 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.

D. Explain that play therapy is an intervention and should not be interrupted for vital signs.

The healthcare provider (HCP) orders gentamicin 2.5 mg/kg IV every 8 hours to be infused over 30 minutes with a peak and trough level at the second dose for treatment of pneumonia. The client weighs 12 kg (26.5 lb). The pharmacy sends 30.0 mg of the medication in a 50-mL bag of sodium chloride to be administered. At what rate should the nurse set the infusion pump? (Enter numerical value only. If rounding, round to the whole number.)

100 (50 mL/30 min) x (60 min/1 hr.) = 100 mL/hr.

The nurse shares with the student nurse that the Society of Pediatric Nurses' Standards of Care includes primary, secondary, and tertiary nursing care prevention. The student nurse asks, "What type of care is given in secondary nursing care prevention?" Which statement by the nurse is correct? A. "It is care such as the sweat test and DNA testing that the child received." B. "It includes mainly teaching information about her cystic fibrosis." C. "This is the nursing care we do when we are taking care of her here in the hospital." D. "It is care that includes giving client immunizations to prevent specific diseases."

A. "It is care such as the sweat test and DNA testing that the child received."

A clinical nurse specialist (CNS) specializing in CF is assigned to provide intensive training for the parents. The CNS explains that she will be helping them by discussing what it means to have a child with CF, the medications and care that the client will require after discharge, and what signs and symptoms warrant a call to the HCP. 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, and clients have been known to live to age 40 or longer. The key to improving a child's life expectancy is good management of the client's nutrition and pulmonary care. Which statement by the parents indicates a correct understanding of a proper nutritional protocol of a client with CF? Select all that apply. A. Her diet should include more calories than an unaffected child. B. Darla should use a salt substitute in place of regular table salt with her meals. C. Darla should consume pancreatic enzymes one hour after consuming her meals. D. Darla should take vitamin A, D, E, and K supplementation along with her diet. E. Her diet should consist of high fat, high protein, and unrestricted fats.

A. Her diet should include more calories than an unaffected child. D. Darla should take vitamin A, D, E, and K supplementation along with her diet. E. Her diet should consist of high fat, high protein, and unrestricted fats.

What nursing task can be safely delegated to the UAP? A. Discontinue a child's peripheral IV. B. Taking vital signs. C. Change the rate on an IV pump controller. D. Complete an admission assessment.

B. Taking vital signs.

The CNS asks which immunizations the client has already received. Her mother has a copy of the immunization record and notices that the child has not had a second measles, mumps, rubella (MMR) immunization. The mother asks, "When should the second MMR be given to her?" What information provided by the nurse is correct? A. The immunization should be given as soon as possible. B. The second immunization is given between ages 4 and 6 years of age. C. The first immunization provides immunity until adulthood. D. The immunization is contraindicated because of her diagnosis of cystic fibrosis.

B. The second immunization is given between ages 4 and 6 years of age.

The nurse also identifies "ineffective airway clearance related to excessive pulmonary secretions" as a nursing diagnosis in the child's plan of care. Which statement should the nurse record as the expect outcome for this nursing diagnosis? A. The child will maintain an oxygen saturation level greater than 95%. B. The parents will list three symptoms that will require notification of the healthcare provider (HCP). 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.

C. The child will be able to remove mucus from the airway by coughing.

While discussing pancreatic enzymes, the CNS explains that the dosage of the pancreatic enzyme is adjusted according to stool formation, which indicates how well client is digesting her food. Which adjustment would the nurse anticipate will be required, if client has constipation? A. The pancreatic enzymes would not be administered for 24 hours. B. The amount of pancreatic enzymes would be decreased at each meal. C. The pancreatic enzymes would be increased with each meal and snack. D. No adjustment in the dosage would be made at this time.

C. The pancreatic enzymes would be increased with each meal and snack.

The healthcare provider (HCP) reviews the client's medical chart. Which documentation further supports the diagnosis of CF? (Select all that apply). 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 of 35 mEq/L (35 mmol/L) E. Bulky loose stools

A. A history of frequent respiratory infections E. Bulky loose stools

Two days later, the client's condition has improved, but she remains in the hospital for continued IV antibiotic therapy. The primary nurse reports to the RN charge nurse that the IV antibiotic ordered was administered to another child and the client received the other child's ordered antibiotic. When the primary nurse realized the mistake, the IV antibiotic was discontinued immediately. Which action should the primary nurse implement first? A. Assess the client and the child who received the wrong medication. B. Administer the antibiotic to the correct clients. C. Complete a medication incident report. D. Notify the healthcare provider (HCP) immediately.

A. Assess the client and the child who received the wrong medication.

The CNS refers the parents to the local chapter of the Cystic Fibrosis Foundation (CFF). 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.

A. Community resources often provide support groups for parents of children with CF.

What is the CNS's responsibility when discussing immunizations? A. Explain that the client should receive all routine childhood immunizations. B. Instruct the parents to ask the pediatrician which immunizations the child should get. C. Discuss why immunizations are not given to children with chronic respiratory illnesses. D. Teach the parents that the child's immunizations will be delayed due to the CF.

A. Explain that the client should receive all routine childhood immunizations.

The nurse arranges to sit down and explain the genesis of CF. The nurse addresses the parents' concerns about how the client inherited this disease. The nurse explains to the client's parents that neither are to blame for the child's disease. The nurse further explains to the parents that almost 21% of children diagnosed with CF are not identified until the age of 2 to 15 years old. The nurse has identified multiple nursing diagnoses due to the chronic and complex disease process of CF. The nurses recognize that energy needs are increased as a result of malabsorption of nutrients and that extra effort is needed for respirations and frequent pulmonary infections. The nurse teaches the client's parents about pancreatic replacement enzymes. Which of the following statements by the parents would indicate a correct understanding of the teaching? A. Pancreatic enzymes are needed to digest fats and proteins. B. Pancreatic enzymes are needed until the steatorrhea stops. C. Administration of pancreatic enzymes will replace the need to take vitamin supplements. D. Pancreatic enzymes should be taken at night before bedtime.

A. Pancreatic enzymes are needed to digest fats and proteins.

Which action indicates to the nurse educator that the parents need more instruction about the respiratory care of the child with CF complications? A. Placing the client in a prone position when she is having a dyspneic episode. B. The client's chest percussions and postural drainage should be performed no sooner than 1 hour before meals or 1.5 hours after meals. C. Encourage the parents to have client participate in physical activities such as running, skipping, and swimming. D. Perform the client's chest percussion and postural drainage after the administration of bronchodilators or nebulized medications as prescribed.

A. Placing the client in a prone position when she is having a dyspneic episode.

If the client attends a daycare center, what should be shared with the daycare workers? Select all that apply. A. Proper handwashing between care of the other children B. Isolate the child from any children with upper respiratory infections C. Administer the enzymes in mashed potatoes twice a day D. Correct techniques for performing chest physiotherapy. E. Give the client vitamins with breakfast.

A. Proper handwashing between care of the other children B. Isolate the child from any children with upper respiratory infections D. Correct techniques for performing chest physiotherapy.

The 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?" Which is the best explanation for the nurse to provide to the student? 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 health care professionals deal with the ethical issues involved in the care of a client. D. Standards of care for working with clients with cystic fibrosis (or other diseases) are outlined in the Nursing Practice Acts of each state.

A. Standards of care are published statements that describe the level of care that the client can expect from nurses.

What laboratory result warrants immediate intervention? A. Serum potassium level of 3.8 mEq/L (3.8 mmol/L). B. White blood cell count (WBC) is 20,000/mcL (20 x 109/L). C. Platelet count is 200 x 103/mcL (200 x 109/L) D. Serum chloride level of 101 mEq/L (101 mmol/L).

B. White blood cell count (WBC) is 20,000/mcL (20 x 109/L).

The nurse meets to discuss the denial of the child's diagnosis. During the conversation, the mother finally breaks down crying and tells the nurse, "I just can't believe what I have been thinking. My child is going to die, and it is all my husband's fault! I should never have married him. No one in my family has ever had this disease. My husband is adopted and his parents do not know anything about his biological background. His biological parents must have had this nasty disease and now he has passed it on to my baby. I am such a terrible wife and mother for having these thoughts, but I just can't bear to watch my precious baby suffer." What response reflects that the nurse values the principle of beneficence? A. "No one ever knows when they will die. She 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. "I know this has to be a very scary and difficult time for you. What can I do to help you?"

D. "I know this has to be a very scary and difficult time for you. What can I do to help you?"

The HCP meets with the parents and informs them that the sweat-chloride test results highly suggest the diagnosis of CF. The mother is very upset about the news and insists that there is another explanation for the child's small stature and that some girls are just more petite than others. The father asks the HCP if there is another test to definitely confirm the diagnosis. The HCP explains that there is another test and arranges for a DNA test to attempt to identify mutations on chromosome 7, which is responsible for the cystic fibrosis disease. The parents have been at the client's bedside since she was admitted to the hospital. The father asks to speak to the nurse outside in the hall. He tells the nurse that the mother is telling everyone that the client is going to be fine and that there has just been a mistake and everything will be all right. What action should the nurse take first? A. Schedule an appointment for Pamela with the hospital chaplain. B. Suggest they sit down and talk about the situation. C. Refer to a professional counselor. D. Make arrangements to meet privately at the first opportunity.

D. Make arrangements to meet privately at the first opportunity.

The parents are worried about their child 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. Which instruction should the nurse educator give the client'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 enzyme capsules and mix the beads in a nonprotein food. D. Ensure that the client swallows the pancreatic enzyme capsule whole. E. Ensure that enzymes are administered within 30 minutes of consuming meals and snacks

E. Ensure that enzymes are administered within 30 minutes of consuming meals and snacks