CF

The HCP rx's gentamicin 2.5mg/kg IV q8h to be infused over 30min w/a peak and trough level at the 2nd dose for tx of pneumonia. The pharamcy sends 17mL of the med in a syringe to be admin. At what rate should the RN set the infusion pump?

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The RN shares w/the SN that the society of ped RN's standards of care includes primary, secondary, and tertiary nursing care prevention. The SN asks, "What type of care is given in secondary nursing care prevention?" Which statement by the RN is correct? a. "It's care such as the sweat test and DNA testing that the kid received" b. "It includes mainly teaching info about her CF" c. "This is the nursing care we do when we're taking care of her here in the hospital" d. "It's care that includes giving Darla immunizations to prevent specific dx's"

a. "It's care such as the sweat test and DNA testing that the kid received"

2d later, the pt's condition has improved, but she remains in the hospital for continued IV antibiotic therapy. The primary RN reports to the charge RN that the IV antibiotic rx was admin to another kid and the pt received the other kids rx antibiotic. When the primary RN realized the mistake, the IV antibiotic was d/c STAT Which action should the primary RN implement first? a. Assess the pt and the kid who received the wrong med b. Administer the antibiotic to the correct pts c. Complete a med incident report d. Notify the HCP STAT

a. Assess the pt and the kid who received the wrong med

The CNS refers the parents to a local chapter of the CF foundation. Which rationale best supports this referral? a. Community resources often provide support groups for parents of kids w/CF b. This foundation will provide health insurance for kids w/CF c. The CFF offers info on alternative therapy and tx modalities d. Referrals for resources offer parents hope that a cure may be found

a. Community resources often provide support groups for parents of kids w/CF

The CNS discusses w/the parents that chronic illnesses such as CF require ongoing full time responsibilities. The CNS offers the possibility of the parents searching for a daycare facility or home daycare setting that would allow for respite care for the parents. On the day of the pt's d/c, the CNS discusses routine immunizations w/the parents. What is the CNS's responsibility when discussing immunizations? a. Explain that the kid should receive all routine childhood immunizations b. Instruct the parents to ask the pediatrician which immunizations the kid should get c. Discuss why immunizations aren't given to kids w/chronic respiratory illnesses d. Teach the parents that the kids immunizations will be delayed d/t CF

a. Explain that the kid should receive all routine childhood immunizations

Her pneumonia resolves. The CNS visits w/the pt and her parents. The parents share that they're looking forward to a relaxing vaca at the beach this summer. They ask the RN educator if any special precautions will be necessary for her. How should the CNS respond? a. Wish them a happy vaca and tell them nothing special needs to be done b. Suggest they add extra salt to Darla's diet and watch her for dehydration c. Explain that the beach isn't a good vaca place for her d. Encourage them to limit her play time at the beach to 30min/day

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

The primary RN, who is assigned to care for 4 pts, is working w/a UAP during the 7-7a shift. What nursing task can be safely delegated to the UAP? a. D/c the kids peripheral IV b. Taking VS c. Change the rate on an IV pump controller d. Complete and admission assessment

b. Taking VS

A 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 kid w/CF, the meds and care the pt will require after d/c, and what S/S warrant a call to her HCP. The RN educator further explains that even though CF is incurable, the life expectancy of a kid w/CF has increased dramatically in recent years, and pt's have been known to live to age 40 or longer. The key to improving a kid's life expectancy is good management of the pt's nutrition and pulmonary care. Which statement by the parents indicates a correct understanding of proper nutritional protocol of a pt w/CF? (SATA) a. Her diet should include more cals than an unaffected kid b. Darla should use a salt substitute in place of regular table sat w/her meals c. Darla should consume pancreatic enzymes one hour after consuming her meals d. Darla should take Vitamin A, D, E, and K suppl along w/her diet e. Her diet should consist of high fat, high protein, and unrestricted fats

a. Her diet should include more cals than an unaffected kid d. Darla should take Vitamin A, D, E, and K suppl along w/her diet e. Her diet should consist of high fat, high protein, and unrestricted fats

The HCP reviews the pt's medical chart. Which documentation further supports the dx of CF? (SATA) a. Hx of frequent RI b. An elevated WBC count c. Reports of episodic abd pain and crying d. Sweat chloride level of 35 mEq/L e. Bulky loose stools

a. Hx of frequent RI e. Bulky loose stools

The CNS explains that chronic respiratory infection is a major cause of lung damage in kids w/CF and that mobilizing secretions is an important aspect of nursing care. What actions should the RN educator take to address this? (SATA) a. Instruct the parents to perform CPT either 2h before meals or 2h after meals b. Aerosolized meds should be administered before CPT c. Discuss the use of a daily oral glucocorticoid steriod therapy d. Discuss the possibility of long-term O2 admin to help w/mobilizing secretions e. Encourage "blowing" activities, such as bubbles or pinwheels.

a. Instruct the parents to perform CPT either 2h before meals or 2h after meals b. Aerosolized meds should be administered before CPT e. Encourage "blowing" activities, such as bubbles or pinwheels.

The RN arranges to sit down and explain the genesis of CF. The RN addresses the parent's concerns about how the pt inherited this dx. The RN explains to the pt's parents that neither are to blame for the kid's dx. The RN further explains to the parents that almost 21% of kids dx w/CF aren't identified until the age of 2-25 y.o. The RN has identified multiple nursing dx d/t the chronic and complex dx process of CF. The RN recognizes that energy needs are increased as a result of malabsorption of nutrients and that extra effort is needed for respirations and frequently pulmonary infections. The RN teaches the pt'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. Admin of pancreatic enzymes will replace the need to take vitamin suppl d. Pancreatic enzymes should be taken at night before bed

a. Pancreatic enzymes are needed to digest fats and proteins

Because kids w/CF are cared for at home, parent education is a priority. The parents agree that they need to learn everything they can about CF. They appear to be loving parents and have a very caring attitude toward their only daughter. They listen attentively to the RN educator, ask pertinent questions, and receive all of the written doc about CF that's provided. Which action indicates to the RN educator that the parents need more instruction about the respiratory care of the kid w/CF complications? a. Placing the kid in a prone position when she's having a dyspneic episode b. The kid's chest percussions and postural drainage should be performed no sooner than 1h before meals or 1.5h after meals c. Encourage the parents to have her participate in physical activities such as running, skipping and swimming d. Perform the kid's chest percussion and postural drainage after the admin of bronchodilators or nebulized meds as rx

a. Placing the kid in a prone position when she's having a dyspneic episode

If the kid attends a daycare center, what should be shared w/the daycare workers? (SATA) a. Proper handwashing between care of other kids b. Isolate the kid from any kids w/upper RIs c. Administer enzymes in mashed potatoes 2x/day d. Correct techniques for performing CPT e. Give the kid vitamins w/breakfast

a. Proper handwashing between care of other kids b. Isolate the kid from any kids w/upper RIs d. Correct techniques for performing CPT

The RN is a preceptor for a SN from the local community college. The SN asks the preceptor, "I keep hearing about standards of care. What are standards of care?" Which is the best explanation for the RN to provide to the student? a. Standards of care are published statements that describe the level of care that the pt can expect from RNs b. Standards of care are laws mandated by the legislature of each state that ensure safe care for pts c. Standards of care are rules that help healthcare professionals deal w/the ethical issues involved in the care of a pt d. Standards of care for working w/the pts w/CF (or other dx) are outlined in the Nurse Practice Acts of each state

a. Standards of care are published statements that describe the level of care that the pt can expect from RNs

The mom shares w/the RN that she's thinking about separating from her husband for a little while. She states that bc her kid has been dx w/CF, she thinks she may want out of the marriage. Shortly after this convo, the RN walks down the hall and the dad asks to speak to her in private. He tells the RN, "I saw you speaking w/my wife earlier. I think she's going to leave me, I'm so scared". Based on her previous convo w/the mom, what is the best statement by the RN? a. You're afraid your wife is going to leave you. Tell me more about what makes you think that b. I'll sit w/your kid so you and your wife can talk. We have a quiet room you can use that is private and close by c. Ethically, I can't discuss what your wife told me. Likewise, I can't tell her what you say either d. Most parents are anxious during situations like this. Would you like me to set you up w/a support group?

b. I'll sit w/your kid so you and your wife can talk. We have a quiet room you can use that is private and close by

The CNS asks which immunizations the kid has already received. Her mom has a copy of the immunization record and notices that the kid hasn't had a second MMR immunization. The mom asks, "When should the second MMR be given?" What info provided by the RN is correct? a. The immunization should be given ASAP b. The second immunization is given between 4-6y of age c. The first immunization provides immunity until adulthood d. The immunization is contra'd bc of her dx of CF

b. The second immunization is given between 4-6y of age

What lab result warrants immediate intervention? a. Serum K level of 3.8 b. WBC count of 20k c. Platelet count is 200x103 mcL d. Serum chloride level of 101

b. WBC count of 20k

While discussing pancreatic enzymes, the CNS explains that the dosage of the pancreatic enzyme is adjusted according to stool formation, which indicates how well she's digesting her food. Which adjustment would the RN anticipate will be required, if she has constipation? a. The pancreatic enzymes wouldn't be admin for 24h b. Amount of pancreatic enzymes would be decreased at each meal c. Pancreatic enzymes would be increased w/each meal and snack d. No adjustment in the dosage would be made

c. Pancreatic enzymes would be increased w/each meal and snack

The RN also identifies "ineffective airway clearance r/t excessive pulmonary secretions" as a nursing dx in the kid's POC. Which statement should the RN record as the expected outcome for this nursing dx? a. The kid will maintain an O2 sat >95% b. The parents will list 3 symptoms that require notification of the HCP c. The kid will be able to remove mucous from the airway by coughing d. The kid will rest comfortably and participate in age-appropriate activities

c. The kid will be able to remove mucous from the airway by coughing

Parents bring their 24mo old kid to the metropolitan area's family care clinic. The reason for the visit is to establish a 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 RN suspects that the pt may have CF. Which assessment supports the diagnosis of CF? a. Fever of 102 F, inflammed larynx w/exudate b. Eyes w/redness and yellow exudate c. Weight loss and delayed growth despite a hearty appetite d. A brassy cough w/inspiratory stridor

c. Weight loss and delayed growth despite a hearty appetite

The RN meets to discuss the denial of the kid's dx. During the conversation, the mom finally breaks down crying and tells the RN, "I just can't believe what I have been thinking. My kid is going to die, and it's all my husband's fault! I should've never married him. No one in my family has ever had this dx. My husband is adopted and his parents don't know anything about his bio background. His bio parents must have had this nasty dx and now he's passed it on to my baby. I am such a terrible wife and mom for having these thoughts, but I just can't bear to watch my precious baby suffer." What response reflects that the RN values the principles of beneficience? a. "No one every knows when they'll 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 kid is d/c home w/scheduled follow up appts. Her parents have been in contact w/the local CFF chapter and have made an appt to talk to a support counselor there. Her parents have demonstrated and verbalized knowledge of the appropriate care that she'll need to continue to grow and thrive w/this dx. The pt and her family are ready to go home. One year after being d/c from CCHMC, the pt is brought to the ED. Her dad 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 pt is A&O x3. She appears lethargic and she's sitting in a tripod position. The kid weighs 12kg and her VS include temp 99F, HR 110 BPM, RR 22 breaths/min, pulse ox 91%. She's displaying retractions, nasal flaring, and grunting. 2d ago, her grandma said that she noticed the kid didn't want to eat and wasn't as active as usual. Which intervention should the RN implement first? a. Notify the CF RN educator to help support the parents b. Administer IV antibiotic therapy c. Insert a 24g IV line d. Administer O2 4L/min via high-flow NC

d. Administer O2 4L/min via high-flow NC

The primary RN finds the pt and the play therapist in the therapy room working w/fingerpaints, and the RN is unsure whether she should interrupt to assess her VS. She seeks advice from the charge RN. What action should the charge RN take? a. Ask the play therapist to take the VS b. Interrupt the play therapy session to take the VS c. Reprimand the primary RN for delaying the taking of VS d. Explain that play therapy is an intervention and shouldn't be interrupted for VS

d. Explain that play therapy is an intervention and shouldn't be interrupted for VS

The HCP rx's a sweat test to confirm the dx of CF. What info will the RN include when teaching about the sweat test? a. Informed consent will be needed for this invasive, dx test b. It will take 2h to obtain the sweat c. This procedure will require the kid to be NPO d. It's a simple and reliable test that measures the chloride in sweat

d. It's a simple and reliable test that measures the chloride in sweat

The HCP meets w/the parents and informs them that the sweat-chloride test results highly suggest the dx of CF. The mom is very upset about the news and insists that there is another explanation for the kid's small stature and that some girls are just more petite than others. The father asks the HCP if there's another test to definitely confirm the dx. The HCP explains that there's another test and arranges for a DNA test to attempt to identify mutations on chromosome 7, which is responsible for the CF dx. The parents have been at the pt's bedside since she was admitted. The dad asks to speak to the RN outside in the hall. He tells the RN that the mom is telling everyone that the pt is going to be fine and that there's just been a mistake and everything will be all right. What action should the RN take first? a. Schedule an appt for Pamela w/the hospital's chaplain b. Suggest they sit 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 kid meeting her nutritional needs. The RN educator explains that the thick mucous blocks the pancreatic ducts, preventing enzymes such as trypsin, amylase, and lipase from being secreted into the small intestines. Which instruction should the RN educator give the pt's parents concerning the use of pancreatic enzymes as an oral rx? a. Administer pancreatic enzymes in the AM and PM b. Mix the pancreatic enzymes w/hot, starchy foods such as macaroni or pasta c. Open the enzyme capsules and mix the beads in a nonprotein food d. Ensure the kid swallows the pancreatic enzymes capsule whole e. Ensure that enzymes are admin within 30min of consuming meals/snacks

e. Ensure that enzymes are admin within 30min of consuming meals/snacks