Cystic Fibrosis

Which condition should the nurse expect to be included in the health history of a client with cystic​ fibrosis? (Select all that​ apply.) Using effort to breathe Performing effective use of huffing Feeling rested Experiencing periods of dyspnea Noticing changes in sputum

Using effort to breathe Performing effective use of huffing Experiencing periods of dyspnea Noticing changes in sputum

The nurse is teaching the parents of a child with cystic fibrosis​ (CF) about physical activity. Which statement by the parents indicates the teaching has been​ effective? (Select all that​ apply.) ​"Our child should eat during​ exercise, even if he is not​ hungry." ​"Our child should limit physical activity because of his weak​ lungs." ​"Our child should eat salty snacks during​ exercise." ​"Our child should not participate in physical activity because of​ CF." ​"Our child should keep fluids high in salt handy during​ activity."

​"Our child should eat during​ exercise, even if he is not​ hungry." ​"Our child should eat salty snacks during​ exercise." ​"Our child should keep fluids high in salt handy during​ activity."

During an​ exacerbation, clients may need CPT as often as

4 times a​ day, up to 1 hour each time

A patient with cystic fibrosis is diagnosed with pancreatic insufficiency. As the nurse you know that the patient will be lacking: A. Amylase B. Pepsin C. Protease D. Maltase E. Lipase

A. Amylase C. Protease E. Lipase

Select the systems below that are affected by cystic fibrosis: A. Reproductive B. Lymphatic C. Respiratory D. Gastrointestinal E. Neuro F. Integumentary

A. Reproductive C. Respiratory D. Gastrointestinal F. Integumentary

A patient is scheduled to take Pancreatin. When will you administer this medication to the patient? A. Right before all meals and snacks B. Right before meals only C. Immediately after meals and snacks D. Immediately after meals only

A. Right before all meals and snacks

The hallmark of cystic fibrosis is: A. chronic bronchiolitis B. diarrhea C. hematuria D. hypoventilation

A. chronic bronchiolitis

A patient that develops CFRD is treated with only insulin. A. true B. false

A. false

With the sweat chloride test being the gold standard for diagnosis of CF, it isn't clear in all individuals. What additional testing would be done to develop a definitive diagnosis? A. genetic testing B. differential count C. LFT D. guaiac test

A. genetic testing

A nurse is teaching the parents of a 10-year-old child diagnosed with cystic fibrosis. Which instruction by the nurse is most appropriate? A. "For Pseudomonas infections, we can use penicillin antibiotics." B. "Preventing respiratory infections is crucial for quality of life." C. "Unfortunately, your child is sterile and unable to have children." D. "With pancreatic enzymes, vitamin replacement is not needed."

ANS: B Cystic fibrosis (CF) is characterized by frequent, severe respiratory infections, often caused by Pseudomonas, which is treated with tobramycin (TOBI) or azithromycin (Zithromax). Preventing respiratory infections is a crucial part of caring for the child with CF. Reproduction is often affected in people with CF, but without testing, it is impossible to say that the child is sterile. Vitamin replacement is needed along with pancreatic enzyme replacement.

A nurse documents and reports the presence steatorrhea in a patient with cystic fibrosis (CF). What does this finding indicate about the patient? a. Is being adequately maintained on the present dose of pancreatic enzyme b. Is not adequately digesting food, leaving loose, fatty, sticky and foul smelling stool c. Has diarrhea related to excess mucus in the bowel d. Has inadequate hydration

ANS: B Foul, bulky stools are the result of inadequately digested food if oral pancreatic enzymes are inadequate.

The nurse is assessing a patient diagnosed with cystic fibrosis. Which findings support the patient's diagnosis? (Select all that apply.) A. Concave chest B. Dry, scaly skin C. Protuberant abdomen D. Wasted buttocks E. Thick extremities

ANS: C, D Protuberant abdomen, barrel chest, wasted buttocks, and thin extremities are common features in children with cystic fibrosis.

A father brings his 1-year-old son to the clinic and states that when he kisses the child's cheek, it tastes salty. Which diagnostic test does the nurse educate the father on based on the father's statement? A. Large bowel barium series B. Pancreatic enzyme analysis C. Pulmonary function studies D. Quantitative sweat chloride test

ANS: D Salty-tasting sweat and tears are a characteristic finding in cystic fibrosis. The diagnostic test for this disorder is the quantitative sweat chloride test. Pancreatic enzyme studies are invasive and not usually performed on children. Pulmonary function studies are done in older children who can cooperate. Large bowel barium studies are not needed.

Four children are in the pediatric clinic waiting to be seen. Which patient should the nurse see first? A. Afebrile, parent reports harsh barky cough B. Pulling on ear, temperature 103°F (39.5°C) C. Salty-tasting sweat, poor weight gain D. Wheezing, retracting, no wet diapers today

ANS: D This child not only has a respiratory problem, he or she is also dehydrated and is the sickest of the four. The nurse should see this patient first. The child with the barky cough is afebrile and has no signs of respiratory distress. Pulling on the ear indicates an ear infection, and these children often run high fevers. The child with salty-tasting sweat and poor weight gain is demonstrating a chronic condition, most likely cystic fibrosis.

The nurse is talking to a group of new nurses about techniques to promote airway clearance in the client who has cystic fibrosis​ (CF). Which intervention should the nurse include in the​ discussion? (Select all that​ apply.) Administering chest physical therapy​ (CPT) fewer times during an exacerbation Avoiding assisting​ caregivers, allowing them to learn by doing Avoiding cough suppressants to avoid an airway obstruction Administering mucolytics before chest physical therapy​ (CPT) Teaching the client huffing and coughing

Avoiding cough suppressants to avoid an airway obstruction Administering mucolytics before chest physical therapy​ (CPT) Teaching the client huffing and coughing

After testing, parents of a newborn discover that they are both carriers of the CTFH gene mutation. What is the chance that their newborn will have the mutation? A. 50% B. 25% C. 100% D. 45%

B. 25%

You're discussing nutrition with your patient who has cystic fibrosis. You explain that it is very important the patient regularly takes fat-soluble vitamins. This includes: A. Vitamin B B. Vitamin D C. Vitamin C D. Vitamin K E. Vitamin E F. Vitamin A

B. Vitamin D E. Vitamin E D. Vitamin K F. Vitamin A

What is the first symptom seen in adults with CF? A. weight gain B. cough C. frothy sputum D. oily stools

B. cough

. You're educating the parents of an 8-month-old, who was recently diagnosed with cystic fibrosis, about the disease. You explain to the parents that the child has a gene mutation on the ____________. The gene that is specifically mutated is called? A. endocrine glands; Hbg S gene B. exocrine glands; CFTR gene C. endocrine glands; Chromosome 21 D. exocrine glands; HTT gene

B. exocrine glands; CFTR gene

Is there a cure for Cystic Fibrosis? A.True B.False

B.False

The mother of a child with cystic fibrosis tells the nurse that her child makes "snoring" sounds when breathing. The nurse is aware that many children with cystic fibrosis have: A.Choanal atresia B.Nasal polyps C.Septal deviations D.Enlarged adenoids

B.Nasal polyps

The nurse should recognize that in order for a child to inherit cystic fibrosis​ (CF), which parent must possess the transmembrane conductance regulator​ (CFTR) gene​ mutation? Female parent with CFTR gene mutation Male parent Neither parent Both parents

Both parents

About one hour after eating a meal, your patient, who has cystic fibrosis, starts to experience abdominal pain and bloating. Then two hours later the patient has a bowel movement. The patient's stool appears to be greasy and have a foul odor. Which medication below that is being taken by the patient is not providing a desirable outcome for this patient and needs to be re-addressed by the physician? A. Guaifenesin B. Triamcinolone C. Pancrelipase D. Polyethylene Glycol

C. Pancrelipase

Pulmonary hypertension and cor pulmonale are seen in CF as a result of: A. disease regression B. right sided HF C. antibiotic resistance D. excessive exercise

C. antibiotic resistance

You're assisting a patient with performing chest physiotherapy. It is very important you have the patient ___________ during the therapy sessions. A. bear down B. use the incentive spirometer C. huff cough D. use a peak flow meter

C. huff cough

The graduate nurse knows that a patient with cystic fibrosis has thick mucous that lines the respiratory and intestines. This is caused by: A. low magnesium B. excess chloride C. low sodium D. loss of cilia

C. low sodium

How is CF diagnosed? A. Sweat test B. Blood test C. Lung volume test D. A and B

D. A and B

Cystic fibrosis is an autosomal recessive genetic disorder. Which option below best describes what most likely happens for a child to develop this condition? A. One parent, who is a carrier of the mutated gene, has to pass it to the child B. One of the parents has to have cystic fibrosis in order to pass it to their offspring C. Both of the parents must have cystic fibrosis in order for the child to develop it D. Both parents, who are carriers of the mutated gene, each pass one mutated gene to the child

D. Both parents, who are carriers of the mutated gene, each pass one mutated gene to the child

A nurse teaching soon to be parents about cystic fibrosis. The nurse know that teaching was effective when the parents state the cystic fibrosis is: A. autosomal dominant B. preterm labor complication C. recessive gene from the mother D. autosomal recessive

D. autosomal recessive

The nurse knows to teach parents of a very active teenager in sports with CF about altering their diet to consume additional: A. potassium B. pepsin C. bronchodilator D. salt

D. salt

Cystic fibrosis is diagnosed by: A.Echocardiogram B.Complete blood panel C.Chest X-ray D.Sweat test

D.Sweat test

Which cause should the nurse recognize related to delayed puberty in an adolescent with cystic fibrosis​ (CF)? ​High-calorie diet Medications prescribed Delayed growth and development ​Thick, sticky secretions

Delayed growth and development

The nurse caring for an adult with cystic fibrosis​ (CF) should recognize which concern as being increasingly ​important? Ongoing sweat testing Fertility Ability to take medication Nutritional supplementation

Fertility

The nurse is admitting a client with cystic fibrosis​ (CF) who is experiencing a pulmonary exacerbation. Which should the nurse include in the physical examination portion of the​ assessment? Nasal flaring Client positioning Level of fatigue Overall appearance

Nasal flaring

Which medication should the nurse expect to be ordered to decrease inflammation for a client with cystic​ fibrosis? Bronchodilator Mucolytic Pancreatic enzymes Nonsteroidal​ anti-inflammatory agents

Nonsteroidal​ anti-inflammatory agents

The nurse is tasked with teaching a client with cystic fibrosis and her mother about chest physical therapy​ (CPT). Which warning is most important for the nurse to​ include? Hold each position for at least 10 minutes. Percuss or vibrate only over the​ sternum, breastbone,​ stomach, or lower ribs and back and never the upper ribs. Perform percussion only when the stomach is full. Percuss or vibrate only over the upper ribs and never over the​ sternum, breastbone,​ stomach, or lower ribs and back.

Percuss or vibrate only over the upper ribs and never over the​ sternum, breastbone,​ stomach, or lower ribs and back.

The nurse should understand that the genetic defect of cystic fibrosis causes which change to the mucus​ secretions? They become thin and watery. They become bloody. They become thick and sticky. They become yellow with pus.

They become thick and sticky.

The physician orders chest physiotherapy on your patient with cystic fibrosis. This is best performed: A. immediately after a meal B. right before a meal C. 1-2 hours after a meal D. only at bedtim

C. 1-2 hours after a meal

The sweat glands of CF patients secrete normal volumes of sweat, but sodium chloride cannot be absorbed form sweat as it moves through the sweat duct. How much sodium and chloride is lost through sweat? A. 3 times the normal amount B. 2 times the normal amount C. 4 times the normal amount D. 5 times the normal amount

C. 4 times the normal amount

A patient completed a sweat test yesterday. The results are back and are 45 mmol/L. As the nurse you know this means: A. The patient tested positive for cystic fibrosis. B. The patient tested negative for cystic fibrosis. C. The patient needs further testing because results are not conclusive.

C. The patient needs further testing because results are not conclusive.

A 2 year-old patient with cystic fibrosis is scheduled to take Pancrelipase. How will you administer this medication? A. orally with yogurt B. orally with pudding C. orally with applesauce D. orally with ice cream

C. orally with applesauce

A pt with CF presents with DIOS. The nurse calls the attending physician to recommend: A. docusate sodium B. Prilosec C. polyethylene glycol D. loperamide

C. polyethylene glycol

The pediatric nurse describes the effects of cystic fibrosis on the body systems to the parents of a child recently diagnosed with the disease. Which statements does the nurse include to the parents? (Select all that apply.) A. Altered protein and vitamin metabolism causes a type of dementia in older children. B. Increased mucus obstructs the airways, and stasis of fluid causes infections. C. Pancreatic ducts are often blocked by mucus, leading to poor nutrition. D. Reproduction is affected, as ovarian ducts and the vas deferens are occluded. E. Thick mucus affects several body systems, preventing some organs from working.

ANS: B, C, D, E Cystic fibrosis is an inherited autosomal recessive disorder that causes the production of thick mucus that blocks exocrine glands and affects several body systems, including the respiratory, gastrointestinal, and reproductive systems. It does not lead to a type of dementia.

The nurse is preparing a presentation on cystic fibrosis​ (CF). Which statement should the nurse include to describe the cause of the genetic mutation of​ CF? Absence or dysfunction of protein consumed by the mother Absence or dysfunction of a protein Absence or dysfunction of the cystic fibrosis transmembrane conductance regulator​ (CFTR) protein Absence or dysfunction of the tumor necrois factor​ (TNF) protein

Absence or dysfunction of the cystic fibrosis transmembrane conductance regulator​ (CFTR) protein

The nurse is providing discharge teaching to a client with cystic fibrosis​ (CF). Which intervention should the nurse include that will promote effective​ breathing? (Select all that​ apply.) Ensure the floating marker is as high as possible during incentive spirometry. Administer bronchodilators before any airway clearance techniques. Use incentive spirometry to practice taking​ slow, deep breaths. Contact the healthcare provider if a decrease in appetite or weight occurs. Administer cough suppressants if client is unable to sleep due to coughing.

Administer bronchodilators before any airway clearance techniques. Use incentive spirometry to practice taking​ slow, deep breaths. Contact the healthcare provider if a decrease in appetite or weight occurs.

The nurse is assessing clients at the community clinic. Which group does the nurse consider as appropriate for genetic testing for cystic fibrosis​ (CF) via blood or​ saliva? All couples expecting a baby Only the parent with a family​ history, when expecting a baby ​Couples, with a family history of​ CF, expecting a baby Those with no history of CF

All couples expecting a baby

The nurse educator is teaching a group of new nurses about pregnancy in cystic fibrosis​ (CF). Which statement should the nurse include in the​ teaching? (Select all that​ apply.) ​"A cesarean section is necessary for most​ cases." ​"CF mothers have a lower than average risk of gestational​ diabetes." ​"Some CF medications may need to be discontinued until after​ pregnancy." ​"Nutritional supplementation is usually necessary for maternal and fetal​ well-being." ​"The outcome of the pregnancy depends on the​ mother's health."

​"Some CF medications may need to be discontinued until after​ pregnancy." ​"Nutritional supplementation is usually necessary for maternal and fetal​ well-being." ​"The outcome of the pregnancy depends on the​ mother's health."

You're providing care to an 18-year-old male who has cystic fibrosis. Select all the possible complications this patient can experience due to cystic fibrosis: A. Blood glucose 255 mg/dL B. Hearing disturbances C. Hemoptysis D. Greasy, foul smelling stools E. Weight gain F. Meconium ileus G. Excessive mucus production H. Dyspnea I. Coughing J. Hyperoxemia K. Infertility

A. Blood glucose 255 mg/dL C. Hemoptysis D. Greasy, foul smelling stools G. Excessive mucus production H. Dyspnea I. Coughing K. Infertility

What conditions could a person with CF develop? (SATA) A. GERD B. renal calculi C. constipation D. type 2 diabetes mellitus E. bradypnea F. gallstones E. DIOS

A. GERD C. constipation F. gallstones E. DIOS

A non compliant patient with CF would present with: (SATA) A. barrel chest B. hypoglycemia C. steatorrhea D. wheezing E. frequent pneumonia

A. barrel chest C. steatorrhea D. wheezing E. frequent pneumonia

A school nurse notices a child with CF from common manifestations of: A. over developed neck and shoulder muscles B. splinter hemorrhages C. failure to thrive D. fingertips are abnormally larger E. yellow sclera F. foul/ fatty stools E. jugular vein distention

A. over developed neck and shoulder muscles C. failure to thrive D. fingertips are abnormally larger F. foul/ fatty stools

A patient with a history of CF comes into the clinic complaining of bone pain. The nurse doing an assessment knows that it is related to: A. vitamin D intake B. excess parathyroid hormone C. excess broccoli in their diet D. electrolyte imbalance of sodium and chloride

A. vitamin D intake

At what age is this disease diagnosed? A.Early years [0-5] B.Childhood years [5-12] C.Teenage years [12-17] D.Adult years [18-50] E.50+

A.Early years [0-5]

The recommended diet for someone with cystic fibrosis is: A.High calorie and high fat diet B.High fat diet C.Low fat diet D.High calorie diet

A.High calorie and high fat diet

When administering pancrelipase (Pancreases capsules) to child with cystic fibrosis, nurse Faith knows they should be given: A.With meals and snacks B.Every three hours while awake C.On awakening, following meals, and at bedtime D.After each bowel movement and after postural drainage

A.With meals and snacks

A patient with cystic fibrosis (CF) furiously refuses any more manual chest physiotherapeutic treatment. Which alternative is appropriate for the nurse to suggest? a. Flutter mucus device b. Increase ambulation to 1 to 2 hours a day c. Steam inhalator several times a day d. Drink 3 quarts of fluid per day

ANS: A A flutter mucus clearance device is a handheld vibrating tool that helps loosen and evacuate secretions in the lung.

The parents of a child diagnosed with cystic fibrosis (CF) consult the nurse, stating they want to have more children but are worried about subsequent children also having the disease. Which information does the nurse provide the parents? A. "Each child has a 25% chance of inheriting the disease." B. "This disease is rare, so other children should not be affected." C. "Unfortunately, there is no way to predict if they will have it." D. "You should have genetic testing to see who the carrier is."

ANS: A Cystic fibrosis is an inherited autosomal recessive disorder. When both parents carry the defective gene, each child has a 25% chance of inheriting the defective gene from both parents and manifesting the disease. It is a common disorder, especially in Caucasians, affecting 1 in 3,000 live births.

The pediatric nurse is providing care to a neonate diagnosed with cystic fibrosis. When discussing the clinical manifestations of this disease process, which topics will the nurse include in the teaching session? (Select all that apply.) A. Anemia B. Malnutrition C. Scant, hard stools D. Meconium ileus E. Rectal prolapse

ANS: A, B, D, E The initial presentation of cystic fibrosis in the neonate appears in the gastrointestinal system. The newborn may have a meconium ileus, with meconium so thick that it causes obstruction and requires surgical removal. The infant may initially have bulky stools that are frothy and foul-smelling. Prolapse of the rectum may also occur in infancy and childhood. Malnutrition, anemia, and growth failure persists despite normal caloric intake.

The nurse is assessing a client with suspected cystic fibrosis​ (CF). Which clinical manifestation should the nurse recognize as a hallmark sign of the disease that would support this​ suspicion? An increased amount of chloride in the sweat A decreased amount of chloride in the nasal secretions A decreased amount of chloride in the sweat An increased amount of chloride in the nasal secretions

An increased amount of chloride in the sweat

The recommended diet for someone with cystic fibrosis is: A) High fat diet B) High calorie and high protein diet C) Low fat diet D) High calorie diet

B) High calorie and high protein diet

Cystic fibrosis (CF) is a genetic disorder that causes the endocrine glands to work incorrectly. A. True B. False

B. False

The nurse reviewing infectious organisms with a CF patient knows education was not effective when the patient states: (SATA) A. infections are caused from pseudomonas aeruginosa B. infections are caused by streptococcus agalactiae C. infections are caused by steroid therapy D. infections are caused by staphylococcus aureus E. infections are caused by Burkholderia cepacia F. infections are caused by haemophilus infuenzae

B. infections are caused by streptococcus agalactiae C. infections are caused by steroid therapy

A 26 y/o male with CF is being educated by a nurse about the disease process. What resources should the nurse include in the teaching? (SATA) A. occupational therapy B. social worker C. reproduction counseling D. cardiologist E. neurologist F. urologist

B. social worker C. reproduction counseling

Correct 5) A male adult client with cystic fibrosis is admitted to an acute care facility with an acute respiratory infection. Prescribed respiratory treatment includes chest physiotherapy. When should the nurse perform this procedure? A.Immediately before a meal B.At least 2 hours after a meal C.When bronchospasms occur D.When secretions have mobilized

B.At least 2 hours after a meal

An 8-year-old client with cystic fibrosis is admitted to the hospital and will undergo a chest physiotherapy treatment. The therapy should be properly coordinated by the nurse with the respiratory therapy department so that treatments occur during: A.After meals B.Between meals C.After medication D.Around the child's play schedule

B.Between meals

A client with cystic fibrosis is taking pancreatic enzymes. The nurse should administer this medication: A.Once per day in the morning B.Three times per day with meals C.Once per day at bedtime D.Four times per day

B.Three times per day with meals

The foul-smelling, frothy characteristic of the stool in cystic fibrosis results from the presence of large amounts of which of the following: A.sodium and chloride B.undigested fat C.semi-digested carbohydrates D.lipase, trypsin and amylase

B.undigested fat

Since pseudomonas infections can become resistant to antibiotics, what medication is used for patients that are chronically infected and treated every other month, every day, twice a day to decrease frequency and increase lung function? A. ciprofloxacin B. vancomycin C. tobramycin D. albuterol

C. tobramycin (Tobi)

A child who has recently been diagnosed with cystic fibrosis is in a pediatric clinic where a nurse is performing an assessment. Which later finding of this disease would the nurse not expect to see at this time?A.Positive sweat test B.Bulky greasy stools C.Moist, productive cough D.Meconium ileus

C.Moist, productive cough

One of the most important pulmonary treatments in cystic fibrosis is: A.inhaled beta agonists. B.inhaled corticosteroids. C.chest physiotherapy. D.oral enzymes.

C.chest physiotherapy.

A patient with CF presents with an increased HR, CO, and hemoptosys, but a decrease in temp and BP would be indicative of: A. anemia B. infection C. respiratory alkalosis D. hypovolemia

D. hypovolemia

Blebs and cyst that develop in the lungs as a severe manifestation of lung destruction from CF can lead to: A. pleural effusion B. emphysema C. stridor D. pneumothorax

D. pneumothorax

Which of the following is an airway clearance technique used for a patient with CF? A. orthopenic position B. high Fowler's C. Trendelenbug D. postural drainage

D. postural drainage

The nurse is providing a health teaching to the mother of an 8-year-old child with cystic fibrosis. Which of the following statement if made by the mother would indicate to the nurse the need for further teaching about the medication regimen of the child? A."My child might need an extra capsule if the meal is high in fat" B."I'll give the enzyme capsule before every snack" C."I'll give the enzyme capsule before every meal" D."My child hates to take pills, so I'll mix the capsule into a cup of hot chocolate

D."My child hates to take pills, so I'll mix the capsule into a cup of hot chocolate

Body systems affected by cystic fibrosis include: A.Pancreas B.Sweat glands C.Lungs D.All of these

D.All of these

Cystic fibrosis is treated by: A.Airway clearance techniques that aim to move the thick mucus from the lungs B.Taking special vitamins C.Taking pancreatic enzymes to help digest food D.All of these

D.All of these

A new graduate nurse is caring for a stable client with cystic fibrosis​ (CF). Which intervention by the graduate nurse requires the nurse preceptor to​ intervene? (Select all that​ apply.) Giving an​ over-the-counter cough suppressant Recommending a​ high-calorie diet Providing constant oxygen Administering a prescribed bronchodilator after chest percussion

Giving an​ over-the-counter cough suppressant Providing constant oxygen Administering a prescribed bronchodilator after chest percussion

Cystic​ fibrosis- (CF-) related digestive problems mean that many children with the disease have a hard time consuming adequate nutrients. Which condition should the nurse expect as a resultant​ effect? (Select all that​ apply.) Vomiting Poor weight gain Diarrhea Delayed growth and development Failure to thrive

Poor weight gain Delayed growth and development Failure to thrive