Peds Cystic Fibrosis Case Study

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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's on my husband's fault! I should never have married him. No one in my family has ever had this disease. My husband is adopted his parents do not know anything about his biological back out. His biological parents must have had this nasty disease and now he has passed it on to my baby. I'm 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?

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

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 correct?

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

Which statement by the parents indicates a correct understanding of a proper nutritional protocol of a client with CF?

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

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 action should the nurse educator take to address this?

-Instruct the parents to perform CPT either 2 hours before meals or 2 hours after meals. -Aerosolized medications should be administered before CPT. -Encouraging "blowing" activities, such as bubbles or pinwheels.

The CNS refers to parents to the local chapter of the Cystic Fibrosis Foundation (CFF). Which rational best supports his referral?

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

Which instruction should the nurse educator give the client's parents concerning the use of pancreatic enzymes as an oral prescription?

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

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 charge nurse. What action should the charge nurse take?

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

What is the CNS's responsibility when discussing immunizations?

Explain that the client should receive all routine childhood immunizations.

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 her marriage. Shortly after this conversation, the nurse walks down the hall and the father asked to speak to her in private. He tells the nurse, "I saw you speaking with my wife earlier. I think she's 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?

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.

What information will the nurse include when teaching about the sweat test?

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

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?

Make arrangements to meet privately at the first opportunity.

The nurse recognizes that energy needs are increased as a result of malabsorption of nutrients and the extra effort is needed for respirations and frequent pulmonary infections. The nurse teaches the clients parents about pancreatic replacement enzymes. Which of the following statements by the parents would indicate a correct understanding of the teaching?

Pancreatic enzymes are needed to digest fats and proteins.

Patient background:

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 that the client may have cystic fibrosis (CF).

Which action indicates to the nurse educator that the parents need more instruction about the respiratory care of the child with CF complications?

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

The nurse is a preceptor for student nurse from the local community college. The student nurse asked 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?

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

Her pneumonia results. The CNS visit to the client and her parents. The parents share that they are looking forward to a relaxing vacation at the beach this summer. They asked the nurse educator if any special precautions will be necessary for her. How should the CNS respond?

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

The primary nurse, who is assigned to take care for for clients, is working with an unlicensed assistive personnel (UAP) during the 7:00 p.m. to 7:00 a.m. shift. What nursing task can be safely delegated to the UAP?

Taking vital signs.

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 is the expected outcome for this nursing diagnosis?

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

What discussing pancreatic enzymes, the CNS explain to the dosage of the pancreatic enzyme is adjusted according to stool formation, which indicates how well she is digesting her food. Which adjustment with the nurse anticipate will be required, if she has constipation?

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

The CNS asks which immunizations the child 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?

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

Which assessment supports the diagnosis of CF?

Weight loss and delayed growth despite a hearty appetite.

What laboratory result warrants immediate intervention?

White blood cell count (WBC) is 20,000/mcL

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

100

Which documentation further supports the diagnosis of CF? (SATA)

-A history of frequent respiratory infections -Bulky loose stools

If the child attends a daycare center, what should be shared with the daycare workers?

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

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 church nurse at the IV antibiotic prescribed was administered to another child and the client received the other child's prescribe antibiotic. When the primary nurse realized the mistake, the IV antibiotic was discontinued immediately. Which action should the primary nurse implement first?

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

When you're after being discharged from the Children's Medical Center, the child is brought to the ED. Her father reports that she started coughing and choking on his unable to catch her breath. She had passed out briefly and he put her in the car drove straight to the hospital. The client is awake and oriented x3. She appears lethargic and she is sitting in a tripod position. The child weighs 12 kg of her vital signs include temperature 99 degrees F, 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?

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


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