PN Cystic Fibrosis HESI Case Study

Legal Issues

Maria's PN is a preceptor for a student PN from the local community college. The student PN says to the preceptor, "I keep hearing about standards of care. What are standards of care?"

Which action by the PN is warranted? a, Notify the social worker that Maria 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 1 week.

B. Document the observations, but take no action at this time. Because these behaviors indicate that Maria is developmentally on target, no action needs to be taken.

Page 7 Client Education

A nurse educator specializing in CF is assigned to Maria to provide intensive training for Abraham and Wendy. 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 Maria will require after discharge, and what signs/symptoms warrant a call to Maria's HCP. The nurse educator further explains that even though CF has increased dramatically in recent years.

Maria's respiratory infection resolves. The nurse educator visits with Maria and her parents to give them discharge instructions.

As the PN prepares for Maria and her parents to go home. Wendy and Abraham share that they are looking forward to a relaxing vacation at the beach this summer

The PN shares with the student PN that the Standards of Care of the Society of Pediatric Nurses includes primary, secondary, and tertiary nursing care. The student PN asks, "What type of care is given in secondary nursing care?" Which statement by the PN is correct? a. It is care such as vision, hearing, and scoliosis screening that Maria will get when she starts school. b. It includes mainly teaching information about Maria's cystic fibrosis c. This is the nursing care we do when we are taking care of Maria here in the hospital d. It is care that includes giving Maria immunizations to prevent specific diseases

A. "It is care such as vision, hearing, and scoliosis screening that Maria will get when she starts school." Secondary nursing care includes all types of screenings to help with early intervention. B. is an example of primary nursing care, D is an example of tertiary nursing care. D. Nursing care such as giving immunization is consider primary care.

The nurse educator further explains that because chronic respiratory infection is a major cause of lung damage in children with CF, mobilizing secretions is an important aspect of the nursing care. What action should the PN take to address this? a. When Maria is old enough, teach her to take a deep breath and then exhale while whispering the word "huff." b. Remind the parents to perform chest physiotherapy (CPT) immediately before Maria eats. c. Explain the importance of decreasing fluid intake during acute exacerbations. d. Discuss the use of a short-term, oral glucocorticoid therapy.

A. 1) When Maria is old enough, teach her 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.

Which explanation should the PN provide? a. Standards of care are published statements that describe the level of care that the client can expect. 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

A. Standards of care are published statements that describe the level of care that the client can expect. Standards of care are set by professional association and describe the level of care that can be expected.

What is the best response by the PN? 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 Maria 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 Maria 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 Maria will not be alone.

The PN asks Wendy which immunizations Maria has already received. Wendy has a copy of the immunization record and notices that Maria has not has a second MMR immunization. Wendy asks, "when should the second MMR be given to Maria?" How should the PN respond? a. It should be administered as soon as possible b. the second MMR is given when Maria is between 4 and 6 years of age c. I think the health care provider will probably let you know. d. It will be given until Maria is 11 to 12 years of age

B. "The second MMR is given when Maria is between 4 and 6 years of age." Based on the childhood immunization schedule, the second MMR should be given when a child is between 4 and 6 years of age. After careful review of all the discharge instructions, Maria is discharged home with scheduled follow-up appointments.

The HCP prescribes ceftriaxone sodium (Rocephin) 12.5 mg IVPB. the medication comes in 12.5 mg/25 mL and is to be infused over 1 hour. the medication will be administered via an intravenous infusion pump set at 25 mL/hr. The RN initiates the IVPB with the Rocephin at the prescribed rate. Then minutes later the PN notices the infusion pump is registering a flow rate of 50 mL/hr. What action should the PN take? a. Reset the IV infusion rate to the correct flow. b. Inform the RN that the infusion rate is too high. c. Record the infusion rate in the medical record. d. Complete an incident report about the mistake.

B. Inform the RN that the infusion rate is too high. The PN should inform the RN immediately of the problem with the infusion rate. Two days later, Maria's condition is improved, but she remains in the hospital for continued IV antibiotic therapy.

What reinforcement should the PN give to Maria's parents? a. Wish them a happy vacation and tell them nothing special needs to be done b. Remind them to add extra slat to Maria's diet and watch her for dehydration c. Tell them that the beach is not a good vacation place for Maria d. Encourage them to limit Maria's play time at the beach to 30 minutes a day

B. Remind them to add extra salt to Maria's diet and watch her for dehydration. There is an increased risk for an electrolyte imbalance secondary to dehydration from excessive perspiring during hot weather.

The PN recognizes that which laboratory result warrants immediate intervention? a. Serum potassium level of 3.o mEq/L b. White blood cells count is 20,000/mm3 c. Platelet count is 200,000/mm3 d. Maria has a serum chloride level of 101 mEq/L

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

Maria is improving, and she is scheduled to attend play therapy in the afternoon, Wendy asks the PN, "What is play therapy?" What is the best response by the PN? a. the volunteers bring toys to Maria to play with in her room b. Play therapy is designed to improve gross motor skills, such as walking. c. Toys and games are used to help with Maria's physical and emotional needs d. The HCP prescribed this to see how well Maria can tolerate playing.

C. "Toys and games are used to help with Maria's physical and emotional needs." Play therapy uses toys instead of words to address growth and developmental needs, to practice social skills, and to express feelings. Play therapy takes place in a specially designed and decorated room that is furnished with carefully selected toys and special equipment

Which statement made by the mother should the practical nurse (PN) recognize that supports the suspected diagnosis of Cystic Fibrosis (CF)? a. My daughter went to bed feeling fine last night and woke up with a high fever and a sore throat. b. Maria's eyes appear to have a yellow discoloration lately 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

C. "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 nurse educator also discusses information about a level of recommended activity for Maria after she is discharged from the hospital. Which intervention should the PN review with Maria's parents concerning the child's level of activity? a. Discourage Maria from engaging in any strenuous activity. b. Encourage Wendy and Abraham to enroll Maria in a weekly gymnastics program c. Discuss the importance of playing games that require Maria to do physical activity such as running and jumping d. Give Maria her inhaler prior to her participating in any physical activity.

C. Discuss the importance of playing games that require Maria to do physical activity such as running and jumping. Research shows that children with CF who regularly participate in aerobic exercise such as jogging or swimming have fewer pulmonary complications.

Which response reflects that the PN values the principle of beneficence? a. No one ever knows when they will die. Maria could live for a long time. b. This seems like a 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. Wendy, I know this has to be a very scary and difficult time for you. What can I do to help you?

D. "Wendy, 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.

Which intervention should the PN implement first? a. notify the social worker to help support Maria's parents b. Administer IV antibiotic therapy c. Insert a 24 gauge intravenous line d. Administer oxygen 2 L/min via pediatric face mask

D. Administer oxygen 2 L/min via pediatric face mask. According to Maslow's Hierarchy of Needs, oxygenation is the priority. The PN should apply oxygen, determine the oxygen saturation level, and ask an RN to start the IV line and administer the antibiotic, and then the PN can contact the social worker.

Nursing Diagnosis The PN reviews Maria's plan of care, which contains multiple nursing diagnoses due to the chronic and complex disease process of CF. Which nursing diagnosis would the PN expect to have 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 off illness and hospitalization. d. Altered nutrition: less than body requirements related to poor intestinal absorption.

D. 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 of need.

Which action(s) should the PN reinforce when discussing respiratory complications? (Select all that apply.) a. Placement of the child in a prone position when she is having a dyspneic episode b. Correct way to administer oxygen at 10 L/min c. Importance of using a cool mist humidifier in Maria's room d. Chest percussion and postural drainage 4 times a day. e. Clearing of retained secretions from the child's mouth.

D. Chest percussion and postural drainage 4 times a day. This will loosen secretions and move them from the peripheral airways into the main airways where they can be expectorated E. Clearing of retained secretions from the child's mouth. This will help prevent aspiration and risk for respiratory infection.

The HCP explains to Maria's mother that a diagnosis of CF is suspected and prescribes a sweat test to confirm the diagnosis. What information should the PN include when reviewing information about the sweat test to Maria's mother? a. Informed consent will be needed for this invasive diagnostic test. b. It will take 2 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.

D. 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 gold standard for diagnosing CF for the past 40 years.

What action should the PN take first? a. Schedule an appointment for Wendy with the hospital's chaplain. b. Suggest that Abraham and his wife sit down and talk about the situation. c. Refer Wendy to a professional counselor. d. Make arrangements to meet with Wendy privately at the first opportunity.

D. Make arrangements to meet with Wendy privately at the first opportunity. The PN should meet with Wendy to determine the situation before taking further action.

Page 4.5

Maria's parents thank the PN for offering to sit with the infant, but they choose to stay with her instead. The PN tells them that she will be available if they change their minds later.

Wendy shares with the PN that she is thinking about separating from Abraham for a little while. She states that she is the one who could not have children, and Abraham really didn't want to adopt a child at first, Because Maria has been diagnosed with CF. Wendy thinks Abraham may want out of the marriage.

Shortly after this conversation, the PN is walking down the hall, and Abraham asks to speak to her in private. He tells the PN. "I say you speaking with my wife earlier. I think she is going to leave me, and I am so scared."

Growth and Development

The PN caring for Maria observes her building a lower of eight blocks. Maria is able to say her first name and last name and to state her age while holding up three fingers.

Page 4 Ethical Issues

The PN meets with Wendy to discuss and evaluate her denial of Maria's diagnosis. During the conversation, Wendy breaks down and tells the PN, "I just can't believe what I have been thinking. If Maria 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."

Management

The PN reports to the charge nurse that a PO antibiotic prescribed for another child was administered to Maria. When the PN realized the mistake, it was reported immediately.

COMMUNITY RESOURCES

The nurse educator refers Wendy and Abraham to the local chapter of the Cystic Fibrosis Foundation (CFF). The PN coordinates with Wendy and Abraham to set up a meeting with the CFF.

Page 3 Therapeutic Communication

The sweat test shows an elevated sodium level and the HCP admits Maria to the Children's Medical center with a diagnosis of Cystic Fibrosis Maria's parents, Abraham and Wendy have been at Maria's bedside since she was admitted to the hospital. Abraham asks to speak with the PN out in the hallway. He communicates to the PN that Wendy is telling everyone that Maria is going to be fine, that there has just been a mistake, and that everything will be all right as long as they pray.

COMPLICATIONS

Three months after being discharged from children's Medical Center, Maria is brought into the emergency Department by her father. He reports that she started coughing and choking, was unable to catch her breath, and eventually passed out> He immediately put her in the car and drove straight to the hospital, where Maria is admitted through the ED.

Meet the Client Maria Maria is an adopted 3-year-old toddler whose family has recently moved to a new city. Maria's mother wants to establish a new health care provider for her daughter and brings Maria in for her 6-months check-up. After a thorough history and physical, the HCP suspects that Maria may have cystic fibrosis (CF).

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The PN reviews Maria's medical records from her previous HCP. Which documentation in Maria's medical record further supports the suspected diagnosis of CF? a. A history of frequent respiratory infections. b. An elevated blood cell (WBC) count. c. Reports of episodic abdominal pain and crying. d. A serum chloride level of 35 mEq/L

A. A history of frequent respiratory infections. The respiratory system is affected by abnormally thick, sticky secretion that cause airway obstruction to the lungs. Other clinical manifestations of CF include: poor growth and/or weight loss, a dry, non-productive cough, and increased bleeding tendencies caused by a deficiency of the fat-soluble vitamin K.

While Maria's mother is discussing with the PN what she learned from the nurse educator, the PN recognizes which statement indicates a correct understanding of the etiology of CF? a. Both of Maria's biological parents must have the CF gene for Maria to get it b. Maria 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 Maria d. There is no genetic predisposition for CF

A. Both Maria's biological parents must have the CF gene for Maria to get it. CF is transmitted as an autosomal recessive trait, which means that both parents must carry the gene for the child to be affected. Each offspring of these parents has a 1 in 4 chance of having the disease.

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 with children with CF. Meeting other parents who have experienced similar situations can provide hope, help, encouragement, and support.

Which action should the PN implement first? a. Determine if Maria is having any reactions or adverse effects. b. Administer the antibiotic to the correct client. c. Complete a medication incident report. d. Notify the HCP immediately.

A. Determine if Maria is having any reactions or adverse effects. First ensure that Maria has suffered no adverse effects from being given the wrong medication. Then, the mediation should be administered to the other client, Maria's HCP should be notified, and an incident report should be completed.

Immunizations on the day of Maria's discharge from the hospital, The PN reviews routine immunizations with Wendy and Abraham. What is the PN's responsibility when reviewing immunizations? a. Explain that Maria should receive all routine childhood immunizations b. Instruct the parents to ask the HCP which immunizations Maria should get. c. Discuss why immunizations are not given to children with chronic respiratory illnesses d. Remind Wendy and Abraham that Maria's immunizations will be delayed due to the CF.

A. Explain that Maria 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.

The PN, who is assigned to care for four clients, is working with an unlicensed assistive personnel (UAP) during the 7 p.m. to 7 a.m. shift. What nursing task can be safely assigned to the UAP? a. Discontinuing a child's peripheral IV b. Emptying a Foley bag c. Changing the rate on an IV pump controller. d. Completing the paper work for an admission.

B. Emptying a Foley bag. This is a task that can be assigned to the UAP.

The PN reiterates the importance of keeping Maria away from people with respiratory infections and discusses how to provide a diet high in calories. She also recalls with the parents the guidelines given by the nurse educator concerning when to call the HCP. While discussing pancreatic enzymes, the PN explains that the dosage of the pancreatic enzyme is adjusted according to stool formation. Which adjustment will be required if Maria 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

C. Increase the pancreatic enzymes with each meal and snack. The dosage must be increased to obtain a well-formed, normal stool.

Which information should the PN reinforce with Maria's parents concerning the use of pancreatic enzymes as an oral prescription? (select all that apply) a. Administer pancreatic enzymes in the morning and at night. b. give the enzymes 30 minutes after each meal has been consumed c. Open the capsules and mix the beads in a food like applesauce. d. Dissolve the capsules in formula so the medication can be given with a bottle. e. Store the enzymes at room temperature away from extreme heat or cold.

C. Open the capsules and mix the beads in a food like applesauce. Maria is 3 years old and unable to swallow a whole capsule. The capsules may be opened and the beads mixed with a small amount of non-protein food such as applesauce, rice, or cereal. e. Store the enzymes at room temperature away from extreme heat or cold. Pancreatic enzymes should be stored between 59 and 86 degrees Fahrenheit away from extreme heat, cold, moisture or light and should never be stored in the refrigerator.

The PN reviews the plan of care under the diagnosis of "ineffective airway clearance related to excessive pulmonary secretions" Which statement should the PN find 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 three symptoms that require notification of the 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. Maria's ability to remove mucus or clear secretions from her airway would indicate that the outcome has been met.

When making rounds to take hourly assessments, the PN finds Maria and the play therapist in the therapy room working with finger-paints. What action should the PN take? a. Ask the play therapist to take the vital signs. b. Interrupt the play therapy session to take the vital signs c. Wait until the next set of vital signs is scheduled d. Delay taking vital signs until play therapy is over

D. Delay taking vital signs until play therapy is over. Play therapy is an important part of the child's hospitalization and should not be interrupted except for emergency situations. Taking vitals signs can wait until the play therapy session is completed.

Wendy and Abraham are worried about Maria's nutritional needs being met. 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 intestine.