HESI RN Case Study - Cystic Fibrosis Peds

Which statement by the mother supports the diagnosis of CF?

"When I kiss my daughter, her skin tastes like salt." Salty-tasting skin is a symptom of CF. It is caused by a high chloride and sodium concentration produced by the sweat glands.

Pamela shares with the nurse that she is thinking about separating from Donald for a little while. She states that because Darla has been diagnosed with CF, Pamela thinks she may want out of the marriage. Shortly after this conversation, the nurse walks down the hall and Donald 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 Pamela, what is the best response by the nurse?

"I will sit with Darla so you and your wife can talk. We have a quiet room you can use that is private and close by." The parents are more likely to leave the room to deal with their crisis if they are assured that Darla will not be alone.

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?

"It is care such as the sweat test and DNA testing that Darla received." Secondary nursing includes all types of screenings to help with early intervention.

The nurse meets with Pamela to discuss her denial of Darla's diagnosis. During the conversation, Pamela finally breaks down crying and tells the nurse, "I just can't believe what I have been thinking. Darla is going to die, and it is all my husband's fault! I should have never 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 had passed it onto 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?

"Pamela, I know this has to be a very scary and difficult time for you. What can I do to help you?" This response promotes good (beneficence) by offering a non-judgmental, supportive reply.

The nurse educator asks Pamela which immunizations Darla has already received. Pamela has a copy of the immunization record and notices that Darla has not had a second measles, mumps, rubella (MMR) immunization. Pamela asks, "When should the second MMR be given to Darla?" What response is most appropriate by the primary nurse?

"The second MMR is given between 4 and 6 years of age." Based on the Childhood Immunizations Schedule by the CDC, the second MMR should be given between 4 and 6 years of age.

The HCP prescribes gentamicin (Garamycin) 2.5 mg/kg every 8 hours IV to be infused over 30 minutes with a peak and trough at the second dose for treatment of pneumonia. The pharmacy sends a 20 mL syringe containing 17 mL of medication labeled gentamicin (Garamycin) 2 mg/mL. At what rate should the nurse set the infusion pump? (Enter numerical value only. If rounding is necessary, round to the whole number.)

30mL/hour Dose ordered is 2.5 mg/kg X 12 kg = 30 mg Dose on hand = 2 mg/mL :: 30 mg/(x) mL Dose needed = 15 mL of medication Medication to infuse within 30 minutes -> 15 mL/30 min :: (x) mL/60 min = 30 mL/60 min or 30 mL/hour is the rate for the syringe infusion pump to be set.

Which intervention should the nurse implement first?

Administer oxygen 6 L/min via simple face mask. According to Maslow's Hierarchy of Needs, oxygenation is the priority.

Which nursing diagnosis has the highest priority?

Altered nutrition: less than body requirements related to poor intestinal absorption. Oxygen, food, and water are the priority physiological needs according to Maslow's Hierarchy of Needs. These biological needs must be satisfied before a person can move to a higher level need.

Two days later, Darla'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 Darla was administered to another child and Darla received the other child's prescribed antibiotic. When the primary nurse realized the mistake, the IV antibiotic was discontinued immediately. Which action should the primary nurse implement first?

Assess Darla and the child that received the wrong medication. Assessment must be completed first to ensure that both children have suffered no adverse effects from being given the wrong medication. Then the HCP should be notified so the nurse can receive follow-up instructions, and then, if appropriate, the correct medications can be administered to both of the clients. A final step is the completion of an incident report.

Darla is discharged home with scheduled follow-up appointments. Her parents have been in contact with the local Cystic Fibrosis Foundation chapter and have made an appointment to talk to a support counselor there. Darla's parents have demonstrated and verbalized the appropriate needs and care that she will need to continue to grow and thrive with this disease. Darla and her family are ready to go home. One year after being discharged from the Children's Medical Center, Darla is brought to the emergency department (ED). Her father reports that Darla 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. Darla is admitted into the emergency department.

Darla is awake and orient x3. She appears lethargic and she sitting in a tripod position. Darla weighs 12 kg and her vital signs include: RR 22 breaths/min, HR 110 beats/min, pulse oximeter 91%, T 99° F (37.2° C). Darla is displaying retractions, nasal flaring, and grunting. Two days ago, Darla's grandmother said that she noticed Darla didn't want to eat and wasn't as active as usual.

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

Darla should take vitamin A, D, E, and K supplementation along with her diet. It is also important that these clients take supplemental fat-soluble vitamins of A, D, E, and K. Her diet should consist of 3 meals/day of high fat, high protein, and 2 to 3 snacks/day. Due to the need to increase the client's caloric intake, it is best to space out the caloric intake with 3 meals/day and 2 to 3 snacks/day, in order not to overwhelm the client with too much food at one sitting and to prevent compromised of their pulmonary status if their stomach is too full putting extra pressure on their diaphragm. Darla's diet should consume more calories than an unaffected child. A client diagnosed with CF should consume a diet that has increased calories, protein, and fat.

The primary nurse, who is assigned to care for four clients, is working with an unlicensed assistive personnel (UAP) during the 7 pm to 7 am shift. What nursing task can be safely delegated to the UAP?

Empty the Foley bag. This is a task that can safely be delegated to the UAP.

On the day of Darla's discharge from the hospital, the nurse educator discusses routine immunizations with Pamela and Donald. What is the nurse educator's responsibility when discussing immunizations?

Explain that Darla should receive all routine childhood immunizations. Children with CF should receive all routine childhood immunizations at the ages recommended by the American Academy of Pediatrics.

Darla's pneumonia resolves. The nurse educator visits with Darla 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 Darla. How should the CF nurse educator respond?

Suggest that they add extra salt to Darla's diet and watch her for dehydration. There is an increased risk for an electrolyte imbalance secondary to dehydration during hot weather.

What laboratory result warrants immediate intervention?

White blood cell count (WBC) is 20,000 mm3. This is elevated, indicating that Darla has an infection.

The nurse educator refers Pamela and Donald to the local chapter of the Cystic Fibrosis Foundation (CFF). Which rationale best supports this referral?

Community resources often provide support groups for parents of children with CF. Meeting other parents who have experienced similar situations can provide hope, help, encouragement, and support.

The primary nurse finds Darla and the play therapist in the therapy room working with fingerpaints, and the nurse is unsure whether she should interrupt to assess Darla's 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. Play therapy is an important part of the child's hospitalization and should not be interrupted except for emergency situations. Taking vital signs can wait.

The HCP prescribes a sweat test to confirm Darla's diagnosis of CF. What information will the nurse include when teaching about the sweat test?

It is a simple, painless, reliable test that measures the chloride in sweat. The sweat test is a simple, painless, and reliable diagnostic test that is performed to determine the amount of chloride in the client's sweat. It has been the gold standard for diagnosing CF for the past 40 years.

Donald and Pamela have been at Darla's bedside since she was admitted to the hospital. Donald asks to speak to the nurse outside in the hall. He tells the nurse that Pamela is telling everyone that Darla is going to be fine and that there has just been a mistake and everything will be all right as long as they pray. What action should the RN take first?

Make arrangements to meet with Pamela privately at the first opportunity. The nurse should meet with Pamela to assess the situation before taking further action.

Pamela and Donald are worried about Darla 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 Darla's parents concerning the use of pancreatic enzymes as an oral prescription?

Open the capsules and mix the beads in a nonprotein food such as applesauce. Darla is 2 years old and probably unable to swallow a whole capsule. The capsules may be opened and the beads mixed with a small amount of nonprotein food such as applesauce, rice, or cereal.

Meet the Client: Darla WilliamsPamela and Donald Williams bring their 24-month-old daughter, Darla, 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. Darla also appears to be suffering from a cold. She is experiencing rhinorrhea noted to be thick and tenacious and a dry cough which Darla's mother states began a couple of days ago.

Physiological Integrity/Clinical Manifestations The nurse suspects that Darla may have cystic fibrosis (CF).

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

Placing Darla in a prone position when she is having a dyspneic episode. Darla should be placed in an upright position, not flat on the stomach, when experiencing a dyspneic episode.

The healthcare provider (HCP) reviews Darla's medical chart. Which documentation further supports the diagnosis of CF?

Reports of foul-smelling stools that have white streaks and are sticky. The term for undigested fat in the stools of clients with CF is steatorrhea. The foul smell is a result of the presence of protein. Large, loose and sticky are also terms that characterize the stools of a client with CF. A history of frequent respiratory infections. The respiratory system is affected by abnormally thick, sticky secretions that cause airway obstruction to the lungs. Other clinical manifestations of CF include poor growth and/or weight loss, a dry and non-productive cough, and increased bleeding tendencies caused by a deficiency of the fat-soluble vitamin K.

Darla'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?" 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. Standards of care are set by professional associations and describe the level of care that can be expected from nurses.

The nurse educator 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 the nursing care. What actions should the nurse educator take to address this?

Teach Darla to take a deep breath and then exhale while whispering the word "huff." This is called "huffing," which is a controlled coughing technique that has proved to help mobilize secretions. Instruct the parents to perform chest physiotherapy (CPT) either 1 hour before meals or 2 hours after meals, if possible. CPT should be done at least 1 hour prior to meals or minimum of 1-2 hours after meals to reduce gastrointestinal upset, and the possibility of vomiting. Explain the importance of increasing fluid intake during acute exacerbations. Increasing fluids helps thin the secretions.

The nurse also identifies, "Ineffective airway clearance related to excessive pulmonary secretions" as a nursing diagnosis in Darla's plan of care. Which statement should the nurse record as the expected outcome for this nursing diagnosis?

The child will be able to remove mucus from the airway by coughing. Darla's ability to remove mucus or clear secretions from her airway would indicate that the outcome had been met.

The nurse arranges to sit down and explain the genesis of CF. The nurse addresses the parents' concerns about how Darla inherited this disease. The nurse explains to Darla's parents that neither are to blame for Darla's disease. The nurse further explains to Darla's 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.

While discussing pancreatic enzymes, the nurse educator explains that the dosage of the pancreatic enzyme is adjusted according to stool formation, which indicates how well Darla is digesting her food. Which adjustment would the nurse anticipate will be required, if Darla has loose, fatty stools?

The pancreatic enzymes would be increased with each meal and snack. The dosage must be increased to obtain a well-formed, normal stool; the presence of the loose, fatty stools is an indicator that she requires more pancreatic enzymes.