Week 2 Cystic Fibrosis Flashcards
The nurse has explained the pathophysiology of cystic fibrosis to a nursing student. Which statement indicates an understanding of the information?
“A client with cystic fibrosis is at risk for gallstone formation because peristalsis in the digestive system is decreased.”
“In the female reproductive system, abnormal mucus production can block the fallopian tubes causing infertility.”
“Impaired bone development can occur due to a combination of factors associated with cystic fibrosis.”
“The lack of digestive enzymes in the small intestine can cause malabsorption related to diarrhea.”
“Impaired bone development can occur due to a combination of factors associated with cystic fibrosis.”
In the skeletal system, a combination of factors, including frequent infections, poor absorption of calcium and fat-soluble vitamins like vitamin D and K, and reproductive abnormalities, can impair bone development, causing lower bone mineral density and osteoporosis.
The nurse has admitted a client to the unit with a history of cystic fibrosis who has developed pneumonia. What is the nurse’s priority action?
Initiate the administration of prescribed aerosolized gentamicin 80 mg
Prepare the client for chest x-ray
Obtain blood glucose
Perform a focused respiratory assessment
Perform a focused respiratory assessment
The nurse’s priority for a client with cystic fibrosis admitted for pneumonia is to begin by assessing the client’s vital signs, focusing on their respiratory status. The nurse should check for chest congestion, cough, and sputum production, then assess the oxygen saturation, rate and depth of breathing, breath sounds, and use of accessory muscles.
The nurse is reviewing the medication administration record (MAR) for a client with cystic fibrosis, experiencing a cough and wheezing. Which prescription should the nurse anticipate administering to help ease the client’s respiratory
symptoms?
Gentamicin 80 mg aerosolized every 8 hours
Esomeprazole 40 mg PO once daily
Pancrelipase 500 lipase units/kg per meal
Dornase alfa 2.5 mg via nebulizer
Dornase alfa 2.5 mg via nebulizer
Dornase alfa is a mucolytic that can be administered to help thin the mucus, making it easier to expel.
The nurse is caring for a client suspected of having cystic fibrosis. Which diagnostic testing should the nurse prepare for the client?
24-hour urine cortisol
Polymerase chain reaction (PCR)
Sweat chloride test
Rapid plasma reagin (RPR)
Sweat chloride test
The gold standard test to diagnose cystic fibrosis is a sweat chloride test, which helps detect high amounts of chloride ions in the sweat of a client with cystic fibrosis.
The nurse is preparing to assess a female client with cystic fibrosis. Which signs and symptoms should the nurse correlate with the condition? Select all that apply.
A. Irregular menstruation
B. Clubbing of fingers
C. Vesicular rash
D. Intermittent claudication
E. Steatorrhea
A. Irregular menstruation
Irregular menstruation is a clinical finding associated with cystic fibrosis caused by abnormal hormonal levels.
B. Clubbing of fingers
Clinical manifestations of cystic fibrosis primarily involve the respiratory tract, so clients typically present with cough, wheezing, and cyanosis, which over time can manifest as clubbing of the fingers.
E. Steatorrhea
Steatorrhea refers to feces with an abnormal amount of fat and is a clinical finding associated with cystic fibrosis.
The nurse is monitoring a client receiving treatment for cystic fibrosis. Which assessment finding should the nurse immediately report to the health care provider?
Oxygen saturation 96%
Fasting glucose 145 mg/dL
White blood cell count of 5500mm3
Temperature of 99.8°F (37.6C)
Fasting glucose 145 mg/dL
The nurse should report abnormal laboratory findings such as fasting glucose greater than 130 mg/dL. Elevated fasting glucose indicates that the pancreas may not be producing enough insulin because injury has occurred from the disease.
The nurse has provided education to a client with cystic fibrosis. Which statement indicates the client requires further teaching?
“I will contact my healthcare provider if I have constipation or diarrhea.”
“I am scheduled to receive my flu vaccine next week.”
“I will eat more foods high in protein, iron, and calcium.”
“When I huff cough, I will take rapid shallow breaths making a “ha” sound with each exhalation.”
“When I huff cough, I will take rapid shallow breaths making a “ha” sound with each exhalation.”
For huff coughing, the nurse should instruct clients to sit up straight and take a slow, deep breath, and hold it for 2-3 seconds; then use their diaphragm and stomach muscles to make a series of rapid exhalations, making a “ha” sound with each exhalation, as if they were trying to fog a mirror with their breath. Have them repeat this technique 2-3 times.
The nurse is caring for a client with suspected cystic fibrosis. Which type of diagnostic test might the health care provider order to confirm this suspicion?
Electrocardiogram
Sweat chloride test
Peak flow test
Scratch test
Sweat chloride test
This answer is correct because cystic fibrosis leads to a high level of chloride in the sweat, so a sweat chloride test often composes part of the diagnostic work-up for a client with suspected CF. During a sweat test, a chemical that causes sweating is applied to the client’s washed extremity on gauze or a disc, and a mild electrical current is applied for five minutes to enhance the sweating reaction. The client’s sweat is then collected for 30 minutes, after which a lab conducts an analysis to determine the level of chloride present in the sweat.
Based on an understanding of the underlying pathophysiological process, the nurse understands which area of the body is most affected in clients with cystic fibrosis? Select all that apply.
Brain
Lungs
Pancreas
Kidneys
Liver
Lungs
This answer is correct because cystic fibrosis is a genetic disorder in which a protein that normally transports sodium and chloride in sweat and saliva glands becomes defective. This abnormality leads to a change in consistency of mucus, making secretions thicker and stickier. The excessively thick and sticky mucus accumulates in the lungs, which can occlude airways and predispose clients to infections.
Pancreas
This answer is correct because cystic fibrosis is a genetic disorder in which a protein that normally transports sodium and chloride in sweat and saliva glands becomes defective. This abnormality leads to a change in consistency of mucus, making secretions thicker and stickier. Mucus build-up blocks release of digestive enzymes in the pancreas, which negatively impacts food digestion and nutrient absorption (leading to poor growth and failure to thrive).
Liver
This answer is correct because cystic fibrosis is a genetic disorder in which a protein that normally transports sodium and chloride in sweat and saliva glands becomes defective. This abnormality leads to a change in consistency of mucus, making secretions thicker and stickier. Mucus accumulation can occlude the bile duct in the liver.
The nurse is caring for a client with cystic fibrosis (CF) who asks about the possibility of passing on cystic fibrosis to future offspring. Which information is correct?
Offspring inherit cystic fibrosis only if both parents give a copy of the CF gene
Any children with cystic fibrosis decrease the likelihood of subsequent siblings with CF
Since the client has the cystic fibrosis gene, all children are guaranteed to have CF
All daughters of this client will become carriers, while all sons will have CF
Offspring inherit cystic fibrosis only if both parents give a copy of the CF gene
This answer is correct because cystic fibrosis results from an inherited disorder that causes a protein abnormality that changes the consistency of mucus. Cystic fibrosis is an autosomal recessive disorder. In order to have cystic fibrosis, individuals must inherit two copies of the faulty gene (one from each parent).
The nurse is caring for a client with cystic fibrosis. Which manifestations are consistent with cystic fibrosis (CF)?
Excessive thirst and increased urination
Frequent ovarian cysts and abdominal cramps
Developmental delays and abnormal muscle tone
Persistent productive cough and weight loss
Persistent productive cough and weight loss
This answer is correct because cystic fibrosis results from a protein abnormality that changes the consistency of mucus, making secretions thicker and stickier. The excessively thick and sticky mucus accumulates in the lungs, which can irritate and occlude airways and predispose clients to a persistent, productive cough and frequent respiratory infections. Mucus can also impair digestive enzymes in the pancreas and lead to undigested food in the small intestine, resulting in poor digestion and nutrient absorption that leads to weight loss.
The nurse is educating a client with cystic fibrosis. Which information should the nurse include?
Be sure to avoid aerobic exercise to avoid putting stress on compromised lungs
Choose low calorie, reasonably portioned meals to avoid unnecessary weight gain
Time postural drainage and positioning sessions before meals to avoid vomiting
Avoid gatherings with more than three people to prevent exposure to illnesses
Time postural drainage and positioning sessions before meals to avoid vomiting
This answer is correct because postural drainage and positioning combine specific positioning with clapping or vibration techniques to help break up the thick mucus and secretions that accumulate in clients with cystic fibrosis. Session goals involve helping the mucus migrate into the larger airways so that it can be expelled by coughing. Postural drainage and positioning sessions should be conducted before meals or at least two hours after meals to decrease the likelihood of vomiting.
