NUR 210 (MED-SURG 2 - Test 4) Study Notes Questions with
Correct Answers (Grade A+)
Question 1: Cystic fibrosis (CF)
Answer: An inherited disorder that affects the secretory glands, particularly the glands that are responsible
for secreting mucus, digestive enzymes, and sweat
Question 2: Cystic fibrosis (CF) affects what organs in the body?
Answer: 1. Lungs 2. Pancreas 3. Intestines 4. Liver
Question 3: Cystic fibrosis (CF) stems from a mutation from what gene?
Answer: Cystic fibrosis transmembrane conductance regulator (CFTR)
Question 4: Cystic fibrosis (CF) causes a mutation of the CFTR gene which causes an absence or
dysfunction of the CFTR protein. What is the CFTR protein responsible for?
Answer: Movement of chloride into and out of the body cells
Question 5: Cystic fibrosis (CF) causes the production of what?
Answer: Thick, sticky mucus
Question 6: The production of thick, sticky mucus by cystic fibrosis (CF) does what?
Answer: Obstructs ducts and passageways within the body, including the airways and pancreatic ducts
Question 7: What causes cystic fibrosis (CF) to produce thick, sticky mucus?
Answer: Excess salt to enters the body cells which causes not enough water to leave the cells because of the
absence or dysfunction of the CFTR protein ***This imbalance causes the cells to secrete the abnormally
viscous sticky mucus
Question 8: What type of disorder is cystic fibrosis (CF)?
Answer: Autosomal recessive
Question 9: In order for a child to express the cystic fibrosis (CF) disease, which parent would they
have to receive the abnormal CFTR gene from?
Answer: BOTH parents
Page 1
,Question 10: If both parents pass on an abnormal CFTR gene to their offspring. What percent
possibility will it be that the child will express (have) cystic fibrosis (CF)?
Answer: 25%
Question 11: Genetic screening for cystic fibrosis (CF) is recommended to assess carrier status in what
individuals?
Answer: 1. Individuals with personal or family history of cystic fibrosis (CF) 2. Individuals who has a
partner with cystic fibrosis (CF) or family history of cystic fibrosis (CF)
Question 12: What clinical manifestation is a hallmark manifestation and is one of the basis for
diagnosis of cystic fibrosis (CF)?
Answer: Chloride in the sweat
Question 13: What are some clinical manifestations for cystic fibrosis (CF)?
Answer: 1. Chronic cough 2. Chronic sinusitis 3. Chronic diarrhea 4. Nutritional deficiencies 5. Delays in
growth/development 6. Impaired blood glucose control 7. Hepatic dysfunction 8. Gallstone formation
Question 14: Most men with cystic fibrosis (CF) have what issue?
Answer: Congenital bilateral absence of vas deferens (CBAVD) **they experience infertility
Question 15: What reproductive complications are women with cystic fibrosis (CF) subject to?
Answer: Decreased fertility
Question 16: T/F: All newborns in the United States are tested for cystic fibrosis (CF) either at birth or
before the age of 2 years old
Answer: TRUE
Question 17: If prenatal testing for cystic fibrosis (CF) is not performed prior to the birth of an infant
then the newborn undergoes newborn screening which involves what?
Answer: Blood test that determines how much immunoreactive trypsinogen (IRT) the newborn's pancreas is
releasing
Question 18: After performing blood work during newborn screening for cystic fibrosis (CF), if the
immunoreactive trypsinogen (IRT) levels are high then what diagnostic test is perform on the newborn
typically at the age of 1 month?
Answer: Sweat test
Page 2
,Question 19: Sweat test
Answer: A diagnostic test that measures the amount of salt in the baby's sweat and is most effective for the
diagnosis of cystic fibrosis (CF) **test is usually performed TWICE
Question 20: What are some examples of surgical and medical procedures used for the treatment of
cystic fibrosis (CF)?
Answer: 1. Removal of nasal polyps (improves breathing) 2. Endoscopic lavage (removes mucus=helps
breathing) 3. Oxygen therapy 4. Feeding tube insertion (administer nutrients) 5. Bowel surgery (bowel
blockage/intussusception)
Question 21: What is medication is recommended before chest physical therapy or before
administration of other inhaled medication to improve mucus clearance and medication delivery in
patients with MILD cystic fibrosis (CF)?
Answer: Bronchodilators **Ex: albuterol (Proventil, Ventolin)
Question 22: What two primary mucolytics are recommended for patients with cystic fibrosis (CF) to
help break up the thick mucus secretions in the airways?
Answer: 1. dornase alfa (Pulmozyme) 2. hypertonic saline (Mogayzel)
Question 23: T/F: Bronchodilators should be administered before hypertonic saline (Mogayzel) to limit
bronchospasms often associated with inhalation of hypertonic saline (Mogayzel)
Answer: TRUE
Question 24: Patients with cystic fibrosis (CF) have altered distribution and metabolism of drugs. If a
patient is prescribed an antibiotic the nurse knows they would be given a dose that is _____ than other
patients with the same infection?
Answer: HIGHER
Question 25: CFTR modulators
Answer: A medication used for the treatment of cystic fibrosis (CF) that targets the cause of the problem
rather than just the clinical manifestations of the disease
Question 26: Due to the high risk for respiratory infections in patients with cystic fibrosis (CF), most
guidelines recommend that the patient stays up to date on what?
Answer: Immunizations, including influenza vaccine **patient must receive INACTIVATED version of the
vaccine not the LIVE version
Page 3
, Question 27: Airway inflammation is a hallmark of cystic fibrosis (CF). What is the most common
anti-inflammatory medication prescribed to for patient with cystic fibrosis (CF)?
Answer: ibuprofen
Question 28: In patients with cystic fibrosis (CF), the small intestine duct is clogged by thick mucus
which prevents digestive enzymes from reaching the intestines. Due to this issue, most patients are
prescribed what oral pancreatic enzymes to help aid in digestion and absorption of nutrients?
Answer: pancrelipase (Creon, Pancreaze)
Question 29: Patients with cystic fibrosis (CF) who are prescribed pancrelipase (Creon, Pancreaze)
should be educated that this medication should be taken when?
Answer: Before every meal or snack **effective for 1 hour after taking it
Question 30: What is the most basic type of airway clearance techniques for nonpharmacological
therapy of patients with cystic fibrosis (CF) because it is an involuntary reflex?
Answer: Coughing
Question 31: Huffing (cystic fibrosis (CF))
Answer: A airway clearing technique that involves holding a breath and then actively exhaling it to move
mucus out of the lungs
Question 32: Chest physical therapy (CPT)
Answer: A airway clearing technique that involves percussion (clapping or pounding on the chest) as well
as vibration and deep breathing to loosen mucus from the lungs so that it can be expelled
Question 33: When is chest physical therapy (CPT) typically performed on cystic fibrosis (CF)
patients?
Answer: Before meals or 1.5 hours after meals
Question 34: How many times a day is chest physical therapy (CPT) typically performed on cystic
fibrosis (CF) patients?
Answer: 1 to 4 times a day
Question 35: How long is chest physical therapy (CPT) typically performed on cystic fibrosis (CF)
patients each session?
Answer: 20 to 40 minutes
Page 4
Correct Answers (Grade A+)
Question 1: Cystic fibrosis (CF)
Answer: An inherited disorder that affects the secretory glands, particularly the glands that are responsible
for secreting mucus, digestive enzymes, and sweat
Question 2: Cystic fibrosis (CF) affects what organs in the body?
Answer: 1. Lungs 2. Pancreas 3. Intestines 4. Liver
Question 3: Cystic fibrosis (CF) stems from a mutation from what gene?
Answer: Cystic fibrosis transmembrane conductance regulator (CFTR)
Question 4: Cystic fibrosis (CF) causes a mutation of the CFTR gene which causes an absence or
dysfunction of the CFTR protein. What is the CFTR protein responsible for?
Answer: Movement of chloride into and out of the body cells
Question 5: Cystic fibrosis (CF) causes the production of what?
Answer: Thick, sticky mucus
Question 6: The production of thick, sticky mucus by cystic fibrosis (CF) does what?
Answer: Obstructs ducts and passageways within the body, including the airways and pancreatic ducts
Question 7: What causes cystic fibrosis (CF) to produce thick, sticky mucus?
Answer: Excess salt to enters the body cells which causes not enough water to leave the cells because of the
absence or dysfunction of the CFTR protein ***This imbalance causes the cells to secrete the abnormally
viscous sticky mucus
Question 8: What type of disorder is cystic fibrosis (CF)?
Answer: Autosomal recessive
Question 9: In order for a child to express the cystic fibrosis (CF) disease, which parent would they
have to receive the abnormal CFTR gene from?
Answer: BOTH parents
Page 1
,Question 10: If both parents pass on an abnormal CFTR gene to their offspring. What percent
possibility will it be that the child will express (have) cystic fibrosis (CF)?
Answer: 25%
Question 11: Genetic screening for cystic fibrosis (CF) is recommended to assess carrier status in what
individuals?
Answer: 1. Individuals with personal or family history of cystic fibrosis (CF) 2. Individuals who has a
partner with cystic fibrosis (CF) or family history of cystic fibrosis (CF)
Question 12: What clinical manifestation is a hallmark manifestation and is one of the basis for
diagnosis of cystic fibrosis (CF)?
Answer: Chloride in the sweat
Question 13: What are some clinical manifestations for cystic fibrosis (CF)?
Answer: 1. Chronic cough 2. Chronic sinusitis 3. Chronic diarrhea 4. Nutritional deficiencies 5. Delays in
growth/development 6. Impaired blood glucose control 7. Hepatic dysfunction 8. Gallstone formation
Question 14: Most men with cystic fibrosis (CF) have what issue?
Answer: Congenital bilateral absence of vas deferens (CBAVD) **they experience infertility
Question 15: What reproductive complications are women with cystic fibrosis (CF) subject to?
Answer: Decreased fertility
Question 16: T/F: All newborns in the United States are tested for cystic fibrosis (CF) either at birth or
before the age of 2 years old
Answer: TRUE
Question 17: If prenatal testing for cystic fibrosis (CF) is not performed prior to the birth of an infant
then the newborn undergoes newborn screening which involves what?
Answer: Blood test that determines how much immunoreactive trypsinogen (IRT) the newborn's pancreas is
releasing
Question 18: After performing blood work during newborn screening for cystic fibrosis (CF), if the
immunoreactive trypsinogen (IRT) levels are high then what diagnostic test is perform on the newborn
typically at the age of 1 month?
Answer: Sweat test
Page 2
,Question 19: Sweat test
Answer: A diagnostic test that measures the amount of salt in the baby's sweat and is most effective for the
diagnosis of cystic fibrosis (CF) **test is usually performed TWICE
Question 20: What are some examples of surgical and medical procedures used for the treatment of
cystic fibrosis (CF)?
Answer: 1. Removal of nasal polyps (improves breathing) 2. Endoscopic lavage (removes mucus=helps
breathing) 3. Oxygen therapy 4. Feeding tube insertion (administer nutrients) 5. Bowel surgery (bowel
blockage/intussusception)
Question 21: What is medication is recommended before chest physical therapy or before
administration of other inhaled medication to improve mucus clearance and medication delivery in
patients with MILD cystic fibrosis (CF)?
Answer: Bronchodilators **Ex: albuterol (Proventil, Ventolin)
Question 22: What two primary mucolytics are recommended for patients with cystic fibrosis (CF) to
help break up the thick mucus secretions in the airways?
Answer: 1. dornase alfa (Pulmozyme) 2. hypertonic saline (Mogayzel)
Question 23: T/F: Bronchodilators should be administered before hypertonic saline (Mogayzel) to limit
bronchospasms often associated with inhalation of hypertonic saline (Mogayzel)
Answer: TRUE
Question 24: Patients with cystic fibrosis (CF) have altered distribution and metabolism of drugs. If a
patient is prescribed an antibiotic the nurse knows they would be given a dose that is _____ than other
patients with the same infection?
Answer: HIGHER
Question 25: CFTR modulators
Answer: A medication used for the treatment of cystic fibrosis (CF) that targets the cause of the problem
rather than just the clinical manifestations of the disease
Question 26: Due to the high risk for respiratory infections in patients with cystic fibrosis (CF), most
guidelines recommend that the patient stays up to date on what?
Answer: Immunizations, including influenza vaccine **patient must receive INACTIVATED version of the
vaccine not the LIVE version
Page 3
, Question 27: Airway inflammation is a hallmark of cystic fibrosis (CF). What is the most common
anti-inflammatory medication prescribed to for patient with cystic fibrosis (CF)?
Answer: ibuprofen
Question 28: In patients with cystic fibrosis (CF), the small intestine duct is clogged by thick mucus
which prevents digestive enzymes from reaching the intestines. Due to this issue, most patients are
prescribed what oral pancreatic enzymes to help aid in digestion and absorption of nutrients?
Answer: pancrelipase (Creon, Pancreaze)
Question 29: Patients with cystic fibrosis (CF) who are prescribed pancrelipase (Creon, Pancreaze)
should be educated that this medication should be taken when?
Answer: Before every meal or snack **effective for 1 hour after taking it
Question 30: What is the most basic type of airway clearance techniques for nonpharmacological
therapy of patients with cystic fibrosis (CF) because it is an involuntary reflex?
Answer: Coughing
Question 31: Huffing (cystic fibrosis (CF))
Answer: A airway clearing technique that involves holding a breath and then actively exhaling it to move
mucus out of the lungs
Question 32: Chest physical therapy (CPT)
Answer: A airway clearing technique that involves percussion (clapping or pounding on the chest) as well
as vibration and deep breathing to loosen mucus from the lungs so that it can be expelled
Question 33: When is chest physical therapy (CPT) typically performed on cystic fibrosis (CF)
patients?
Answer: Before meals or 1.5 hours after meals
Question 34: How many times a day is chest physical therapy (CPT) typically performed on cystic
fibrosis (CF) patients?
Answer: 1 to 4 times a day
Question 35: How long is chest physical therapy (CPT) typically performed on cystic fibrosis (CF)
patients each session?
Answer: 20 to 40 minutes
Page 4
