HESI: Cystic Fibrosis and Rationale

The healthcare provider (HCP) orders gentamicin 2.5 mg/kg IV every 8 hours to be infused over 30 minutes with a peak and trough level at the second dose for treatment of pneumonia. The pharmacy sends 17 ml of the medication in a syringe to be administered.

34 Medication to infuse within 30 minutes -> 17 mL/30 min :: (x) mL/60 min = 34 mL/60 min or 34 mL/hour is the rate for the syringe infusion pump to be set.

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? Schedule an appointment for Pamela with the hospital's chaplain. Suggest that they sit down and talk about the situation. Refer to a professional counselor. Make arrangements to meet privately at the first opportunity.

Make arrangements to meet privately at the first opportunity. One of the most supportive interventions for families of children with chronic health care conditions or special needs is to accept the family's emotional reactions to the diagnosis in a non-judgmental manner. The nurse should meet to assess the situation before taking further action.

The nurses recognize that energy needs are increased as a result of malabsorption of nutrients and that extra effort is needed for respirations and frequent pulmonary infections. The nurse teaches the client's 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. Pancreatic enzymes are needed until the steatorrhea stops. Administration of pancreatic enzymes will replace the need to take vitamin supplements. Pancreatic enzymes should be taken at night before bedtime.

Pancreatic enzymes are needed to digest fats and proteins. With cystic fibrosis, the body lacks the ability to excrete the pancreatic enzymes needed to digest fats and proteins, so replacement enzymes must be administered.

The 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 laws mandated by the legislature of each state that ensure safe care for clients. Standards of care are rules that help health care professionals deal with the ethical issues involved in the care of a client. Standards of care for working with clients with cystic fibrosis (or other diseases) are outlined in the Nursing Practice Acts of each state.

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.

Client's pneumonia resolves. The CNS visits with the client 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 her. How should the CNS respond? Wish them a happy vacation and tell them nothing special needs to be done. Suggest that they add extra salt to Darla's diet and watch her for dehydration. Explain that the beach is not a good vacation place for her. Encourage them to limit client's play time at the beach to 30 minutes a day.

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. Drinking plenty of fluids is always necessary for people with CF, but becomes especially important during the hot, humid months of summer. Individuals with cystic fibrosis lose more salt (sodium chloride) in their sweat than those without CF. In the summer months and during exercise, those with CF are at increased risk of becoming hyponatremic.

What nursing task can be safely delegated to the UAP? Discontinue a child's peripheral IV. Taking vital signs. Change the rate on an IV pump controller. Complete an admission assessment.

Taking vital signs. This is a task that can safely be delegated to the UAP.

Which statement should the nurse record as the expected outcome for this nursing problem? The child will maintain an oxygen saturation level greater than 95%. The parents will list three symptoms that require notification of the HCP. The client will be able to remove mucus from the airway by coughing. The child will rest comfortably and participate in age-appropriate activities.

The client will be able to remove mucus from the airway by coughing. Airway therapy is aimed at removing the mucus and moving it to large airways to be cleared. Client's ability to remove mucus or clear secretions from her airway would indicate that the outcome had been met.

While discussing pancreatic enzymes, the CNS explains that the dosage of the pancreatic enzyme is adjusted according to stool formation, which indicates how well client is digesting her food. Which adjustment would the nurse anticipate will be required, if client has constipation? The pancreatic enzymes would not be administered for 24 hours. The amount of pancreatic enzymes would be decreased at each meal. The pancreatic enzymes would be increased with each meal and snack. No adjustment in the dosage would be made at this time.

The pancreatic enzymes would be increased with each meal and snack. Pancreatic enzymes are adjusted to decrease the bulk of the stool.

What laboratory result warrants immediate intervention? Serum potassium level of 3.8 mEq/L (3.8 mmol/L). White blood cell count (WBC) is 20,000/mcL (20 x 109/L). Platelet count is 200 x 103/mcL (200 x 109/L) Serum chloride level of 101 mEq/L (101 mmol/L).

White blood cell count (WBC) is 20,000/mcL (20 x 109/L). This is an abnormal value. Normal values range 4500 to 13500/mcL (4.5 to 13.5 x 109/L). This is elevated, indicating that the client has an infection.

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 the child received." "It includes mainly teaching information about her cystic fibrosis." "This is the nursing care we do when we are taking care of her here in the hospital." "It is care that includes giving client immunizations to prevent specific diseases."

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

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 is all my husband's fault! I should never have 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 has passed it on to 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? "No one ever knows when they will die. She could live for a long time." "This seems like an ethical dilemma to me. I'll refer it to the ethics committee." "I think you probably should contact an attorney to discuss this if you're really serious." "I know this has to be a very scary and difficult time for you. What can I do to help you?"

"I know this has to be a very scary and difficult time for you. What can I do to help you?" Beneficence is characterized by acts or personal qualities of mercy, kindness, generosity, and charity. It is suggestive of altruism, love, humanity, and promoting the good of others. This response promotes good (beneficence) by offering a non-judgmental, supportive reply.

Which documentation further supports the diagnosis of CF? Select all that apply A history of frequent respiratory infections. An elevated white blood cell count (WBC). Reports of episodic abdominal pain and crying. A sweat chloride level of 35 mEq/L (35 mmol/L). Bulky loose stools

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. Bulky loose stools 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.

Which intervention should the nurse implement first? Notify the CF nurse educator to help support the parents. Administer IV antibiotic therapy. Insert a 24-gauge intravenous line. Administer oxygen 4L/min via high-flow nasal cannula.

Administer oxygen 4L/min via high-flow nasal cannula. According to Maslow's Hierarchy of Needs, oxygenation is the priority. The clinical signs and symptoms of hypoxia include apprehension, restlessness, inability to concentrate, decreased level of consciousness, dizziness, and behavioral changes. The patient with hypoxia is unable to lie flat and appears both fatigued and agitated. Vital sign changes include an increased pulse rate and increased rate and depth of respiration. During early stages of hypoxia the blood pressure is elevated unless the condition is caused by shock. As the hypoxia worsens, the respiratory rate declines as a result of respiratory muscle fatigue. Oxygen therapy is widely available and used in a variety of settings to relieve or prevent tissue hypoxia. The goal of oxygen therapy (AARC, 2007) is to prevent or relieve hypoxia by delivering oxygen at concentrations greater than ambient air (21%).

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 RN charge nurse that the IV antibiotic ordered was administered to another child and the client received the other child's ordered 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. Administer the antibiotic to the correct clients. Complete a medication incident report. Notify the healthcare provider (HCP) immediately.

Assess the client and the child who 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 healthcare provider (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.

The CNS refers the parents 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. This foundation will provide health insurance for children with CF. The CFF offers information on alternative therapy and treatment modalities. Referrals for resources offer parents hope that a cure may one day be found.

Community resources often provide support groups for parents of children with CF. The Cystic Fibrosis Foundation has chapters throughout the United States to provide education and services to patients and professionals. Meeting other parents who have experienced similar situations can provide hope, help, encouragement, and support.

Which instruction should the nurse educator give the client's parents concerning the use of pancreatic enzymes as an oral prescription? (Select all that apply. One, some, or all options may be correct.) Administer pancreatic enzymes in the morning and at night. Mix the pancreatic enzymes with hot, starchy foods such as macaroni or pasta. Open the enzyme capsules and mix the beads in a nonprotein food. Ensure that the client swallows the pancreatic enzyme capsule whole. Ensure that enzymes are administered within 30 minutes of consuming meals and snacks

Ensure that enzymes are administered within 30 minutes of consuming meals and snacks Enzymes are administered with a meal or within 30 minutes of a meal.

The primary nurse finds the client and the play therapist in the therapy room working with fingerpaints, and the nurse is unsure whether she should interrupt to assess her vital signs. She seeks advice from the RN team leader. What action should the charge nurse take? Ask the play therapist to take the vital signs. Interrupt the play therapy session to take the vital signs. Reprimand the primary nurse for delaying the taking of the vital signs. Explain that play therapy is an intervention and should not be interrupted for vital signs.

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.

On the day of the client's discharge from the hospital, the CNS discusses routine immunizations with the parents. What is the CNS's responsibility when discussing immunizations? Explain that the client should receive all routine childhood immunizations. Instruct the parents to ask the pediatrician which immunizations the child should get. Discuss why immunizations are not given to children with chronic respiratory illnesses. Teach the parents that the child's immunizations will be delayed due to the CF.

Explain that the client 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.

Which statement by the parents indicates a correct understanding of a proper nutritional protocol of a client with CF? Select all that apply Her diet should include more calories than an unaffected child. Client should use a salt substitute in place of regular table salt with her meals. Client should consume pancreatic enzymes one hour after consuming her meals. Client 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.

Her diet should include more calories than an unaffected child. A client diagnosed with CF should consume a diet that has increased calories, protein, and fat. Client 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 high fat, high protein, and unrestricted fats. 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 compromising pulmonary status (if the stomach is too full, putting extra pressure on the diaphragm).

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 the marriage. Shortly after this conversation, the nurse walks down the hall and the father 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 the mother, what is the best statement by the nurse? You are afraid your wife is going to leave you. Tell me more about what makes you think that. 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. Ethically, I cannot discuss what your wife told me. Likewise, I cannot tell her what you say, either. Most parents are anxious during situations like this. Would you like me to set you up with a support group?

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. With a diagnosis that has long term impacts, the nurse should allow the parents time to express emotions and work through feelings naturally. The parents are more likely to leave the room to deal with their crisis if they are assured that the client will not be alone.

What actions should the nurse educator take to address this? Select all that apply Instruct the parents to perform chest physiotherapy (CPT) either 2 hours before meals or 2 hours after meals. Aerosolized medications should be administered before CPT. Discuss the use of a daily oral glucocorticoid steroid therapy. Discuss the possibility of long-term oxygen administration to help with mobilizing secretions. Encourage "blowing" activities, such as bubbles or pinwheels.

Instruct the parents to perform chest physiotherapy (CPT) either 2 hours before meals or 2 hours after meals. CPT should be performed before meals or 1-1.5 hours after a meal to reduce gastrointestinal upset, and the possibility of vomiting. Aerosolized medications should be administered before CPT. These medications should be administered after CPT. Encourage "blowing" activities, such as bubbles or pinwheels. Methods to stimulate deep breathing include blowing through a straw, blowing cotton balls or a ping pong ball on a table, blowing balloons, or blowing pinwheels.

What information will the nurse include when teaching about the sweat test? Informed consent will be needed for this invasive, diagnostic test. It will take 2 hours to obtain the sweat. This procedure will require the child to be NPO (nothing by mouth). It is a simple and reliable test that measures the chloride in sweat.

It is a simple and 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. The sweat chloride test involves stimulating the production of sweat with a special device and collecting the sweat on filter paper, and measuring the sweat electrolytes. It has been the gold standard for diagnosing CF for the past 40 years.

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 client in a prone position when she is having a dyspneic episode. The client's chest percussions and postural drainage should be performed no sooner than 1 hour before meals or 1.5 hours after meals. Encourage the parents to have client participate in physical activities such as running, skipping, and swimming. Perform the client's chest percussion and postural drainage after the administration of bronchodilators or nebulized medications as prescribed.

Placing the client in a prone position when she is having a dyspneic episode. Patients with dyspnea should be placed in a position to promote maximum ventilatory efficiency and airway patency. The child should be placed in an upright position, not flat on the stomach, when experiencing a dyspneic episode.

If the client attends a daycare center, what should be shared with the daycare workers? Select all that apply Proper handwashing between care of the other children Isolate the child from any children with upper respiratory infections Administer the enzymes in mashed potatoes twice a day Correct techniques for performing chest physiotherapy. Give the client vitamins with breakfast.

Proper handwashing between care of the other children Children with CF are susceptible to infections and proper handwashing is important in this setting. Isolate the child from any children with upper respiratory infections Children with CF are susceptible when around others with URIs. Correct techniques for performing chest physiotherapy. Children with CF should have chest physiotherapy performed routinely to prevent respiratory infections.

The CNS asks which immunizations the client 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 immunization should be given as soon as possible. The second immunization is given between ages 4 and 6 years of age. The first immunization provides immunity until adulthood. The immunization is contraindicated because of her diagnosis of cystic fibrosis.

The second immunization is given between ages 4 and 6 years of age. The MMR is given to children 12-15 months or at 4-6 years of age, concurrent with other immunizations.

Which assessment supports the diagnosis of CF? A fever of 102 ºF (38.9 ºC), inflamed larynx with exudate. Eyes with redness and yellow exudate. Weight loss and delayed growth despite a hearty appetite. A brassy cough with inspiratory stridor.

Weight loss and delayed growth despite a hearty appetite. Pancreatic insufficiency and malabsorption are characteristic of CF and result in weight loss and delayed growth.