PEDS Exam 2 CF HESI case study

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."

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?"

"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 nurse suspects that Darla may have cystic fibrosis (CF). 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.

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

A history of frequent respiratory infections. Reports of foul-smelling stools that have white streaks and are sticky.

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 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.

The nurse has identified multiple nursing diagnoses due to the chronic and complex disease process of CF. Which nursing diagnosis has the highest priority? 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.

Altered nutrition: less than body requirements related to poor intestinal absorption.

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

Darla's diet should consume more calories than an unaffected child. These clients also need to receive additional salt because they are excreting more of it through their sweat. The pancreatic digestive enzymes should be given with each meal and snacks or no earlier than 30 minutes prior, so the enzymes help the food to be digested in the small intestine. Darla should take vitamin A, D, E, and K supplementation along with her diet. Her diet should consist of 3 meals/day of high fat, high protein, and 2 to 3 snacks/day.

Health Promotion and Maintenance/Immunizations 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.

Safe and Effective Care/Diagnostic Test 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.

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

Pancreatic enzymes should be taken 30 minutes before eating or with the meal. Heat causes the enzymes to be inactivated. Open the capsules and mix the beads in a nonprotein food such as applesauce.

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 physical act of performing CPT and positioning of postural drainage can cause stress to the GI system and cause the child not wanting to eat or if has eaten to vomit their food.

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.

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? Select all that apply

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. Explain the importance of increasing fluid intake during acute exacerbations. Increasing fluids helps thin the secretions. Steroid therapy may be used in acute exacerbations, but it is not used to mobilize secretions. Oxygen administration is sometimes used in acute episodes. Long-term oxygen use can be harmful in children with cystic fibrosis because these children have chronic issues with retaining carbon dioxide.

Physiological Integrity/Psychosocial Integrity

The HCP meets with Darla's parents and informs them that the sweat-chloride test results highly suggest the diagnosis of CF. Darla's mother is very upset about the news and insists that there is another explanation for Darla's small stature and that some girls are just more petite than others. Darla's 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 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.

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.

What laboratory result warrants immediate intervention?

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

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.

A nurse educator specializing in CF is assigned to Darla to provide intensive training for Donald and Pamela. 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 Darla will require after discharge, and what signs and symptoms warrant a call to Darla's 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.